Pediatr Radiol (1990) 20:377-424

Pediatric Radiology 9 Springer-Verlag 1990

27th Congress of the European Society of Paediatric Radiology Munich, May 16-19, 1990 Abstracts


Abstracts. 27th Congress of the European Society of Paediatric Radiology

Head and Spine Oral Presentations 1




n t r o u c t l o n : ~ ) - ~ - ~ g - t - h - e - ~ i r s t two y e a r s ~ , " g ~ ~ 1 . 0 T demonstrate characteristic changes o f g r a y and w h i t e m a t t e r s i g n a l i n t e n s i t i e s . The purpose o f t h i s s t u d y was: 1 . t o q u a n t i f y t h e s e changes f o r the sequences commonly used; 2 . t o p r o v i d e a s e t of normal v a l u e s and s t a n d a r d deviations for cerebral matter development. Method: 120 b r a i n e x a m i n a t i o n s i n i n f a n t s y o u n g e r T ah--a-n--2-4 months o f age were e v a l u a t e d . 35 e f t h e s e p a t i e n t s were diagnosed as n o r m a l . In a l l cases r e l a x a t i o n t i m e s and p r o t o n d e n s i t i e s f o r f r o n t a l and o c c i p i t a l w h i t e and gray m a t t e r as w e l l as f o r the i n t e r n a l c a p s u l e and the corpus c a l l o s u m were measured. For 4 d i f f e r e n t sequences the contrast-to-noise r a t i o between gray and w h i t e m a t t e r was c a l c u l a t e d . R e s u l t s : R e l a x a t i o n times and proton densities d e c ~ i t h i n c r e a s i n g age. This t e n d e n c y was more pronounced f o r w h i t e matt e r than f o r gray m a t t e r . Based on t h e s e d a t a , a simple algorithm provided theoretical gray/white c o n t r a s t c u r v e s as a f u n c t i o n o f age and of the v a r i o u s imaging p a r a m e t e r s . In normals t h e s e c a l c u l a t e d diagrams agreed w e l l w i t h the t i m e c o u r s e o f the c o n t r a s t measured f o r each p a t i e n t . In some i n f a n t s w i t h c o n g e n i t a l o r p e r i n a t a l d e f e c t s g r a y / w h i t e c o n t r a s t d i f f e r e d from the normal v a l u e s . In t h e s e cases a 2 - s i g m a - c u r v e p a r a l l e l l i n g t h e mean v a l u e s helped t o d i s c r i m i n a t e between normal and p a t h o l o g i c a l d e v e l o p m e n t . C o n c l u s i o n : S u c c e s s i v e s tages o f m a t u r a t i o n can be ass~ss--~-e-de x a c t l y by MR Imaging a t 1.0 T.

A. Daneman, P. Burrows An otherwise well four week old male was found to have cardiomegalyon routine postnatal checkup. Further clinical evaluation revealed a cranial brtfit but no other significant finding. Echocardiographyconfirmed the cardiomegalybut showed no cardiac defects. Cranial sonographyat a referring institution failed to document an intracranial cause for the cranial bruit. Clinical follow-up t w o weeks later again revealed cardlomegaly and the cranial bruit but cardiac failure was not present. A repeat cranial sonogram at our institution showed moderate enlargement of the superior subaracbnnld space over the cerebral hemispheres-(a little larger on the right than the left) and widening of the interhemispheric fissure. The superior sagittal sinus was enlarged. There were a few echogenlc foe[ noted in the superior subarachnoid space art the right lateral to the falx. The appearance of some of these focl suggested the presence of enlarged superficial cerebral vessels. The color Doppler study clearly showed a markedly enlarged branch of the right anterior cerebral artery, ascending along the medial surface of the right cerebral hemisphere. This communicated with a bunch of irregular vessels on the superior service of the right cerebral hemisphere and these drained into the superior sagittal sinus. These findings were confirmed on angiography. Superficial arteriovenous malformations presenting in the neonatal period are rare. This case emphasizes the need to evaluate superficial structures diligently in the search of arteriovenous malformations and illustrates how color Doppler evaluation can facilitate the documentation of the presence of abnormal vessels with their unusual communications and directions of flow. The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, Canada, M5G 1X8 (Department of Radiology)

Radiologische Klinik, U n i v e r s i t ~ t MUnchen, Marchioninistr.15, D-8OO0 MUnchen 70




N. Sellier*, C.Combes**, G.Kalifa** Opercular malformation is a rare bra'm lesion characterized by the abnormal development of the sylvian and rolandie fissures. The diagnosis may be suggested by CT but MRI offers a better demonstration of the three main features: -



verticalization of the sylvian fissure which seems prolongated by the rolandic one, abnormal cortical venous drainage, micropolygyrlc cortex surrounding these fissures.

One can find good correlation with the embryological data. In one case the postmedical history allows to date the time of this injury around 22 weeks of gestation. This entity belongs to the large spectrum of neuronal migrational disorders, is different from localized pachygyria and fused schizencephaly, and can easily be identified thanks to MRI. *Hep. Jean VERDIER, Av. du 14 jnillet, BONDY 93143. **H6p. St. Vincent-de-Paul, 74 Av. Denfert Roehereau, Paris 75014.





DEPARTMENT OF PAEDIATRIC RADIOLOGY - INSTITUTE OF RADIOLOGY; UNIVERSITY OF HEIDELBERG, B-6900 HEIDELBERG I n c o n t r a s t t o e a r l i e r papers i n which i t was presumed t h a t t h e ascendence o f t h e conus m e d u l l a r i s up t o t h e l e v e l L1/2 t o o k p l a c e t h r o u g h o u t c h i l d h o o d , MR-imaging has proved t h a t t h e conus m e d u l l a r i s o f n e w -b a rn s i s a l r e a d y t o be found a t t h e l e v e l o f L 1 / 2 . * T h e r e f o r e we examined t h e l e v e l o f t h e cenus m e d u l l a r i s o f 100 p r e m a t u r e and f u l l t e r m b a b i e s and young i n f a n t s by u sin g u l t r a s o u n d ( l i n e a r t r a n s d u c e r 5 MHz), C o n c l u s i o n : I n c h i l d r e n aged between t h e 30th and 39th week o f g e s t a t i o n t h e cenus m e d u l l a r i s m o s t l y l i e s a t L2/3 and from t h e 40th week o f g e s s a t ~he l e v e l o f L 1 / 2 . Thus t h e ascendence o f t h e conus m e d u l l a r i s i s c o m p l e t e d by the 40th week of g e s t a t i o n , The case of one three month old baby (53th week of gestation) we found the tip of the conus m e d u l l a r i s at the level of L4. This finding had not changed when e x a m i n e d 2 months later (tethered cord?). *Wilson, O.A. Prince J.R. 1989 MR Imaging D e t e r m i n a t i o n of the Location of tile Normal Conus M e d u l l a r i s T h r o u g h o u t Childhood AJR 1 5 2 : 1 0 2 9 - 1 0 3 2

Abstracts. 27th Congress of the European Society of Paediatric Radiology






V.Donoghue, J.Gillen, S.Gormally, M.King. We report 3 newborn infants with sonographic evidence of linear increased echo~enicity at the level of the lenticulostriate vessels ("Candlestick Sign", Toms et al, E.S.P.R. 1989 ) Contrary to the previous report there was no evidence of congenital infection in any of the three infants. 2 infants were born prematurely and were of low birth weight. Both were second twins and were thought to have suffered cerebral asphyxia. Both infants had negative T.O.R.C.H. titres. Cerebral ultrasonogbaphy showed linear areas of increased echogenicity in the region of the lenticulostriate vessels. One infant had a small area of adjacent peri-ventricular leucomalacia on one side. The third infant was bo~n at term and was thought clinically to have Maebius Syndrome. He was ventilated intermittently for 19 days and subsequently died. Autopsy in this infant showed (i) bilateral multifocal microscopic astroglial reaction in the thalami. (2) calcium encrustation of individual neurones. No vessel abnormality was identified histologically and there was no morphological evidence of congenital infection in the infant or its placenta. The findings were thought to be the result of intrauterine asphyxia. We conclude that the findings on sonography are nonspecific and are due to ischaemia rather than congenital infection as previously was suggested. Radiology Department, The Children's Hospital Temple St., Dublin 1 Ireland.

Cerebral infarction (CI) or focal ischemia occurs in the neonate without specific clinical symptoms. With the advent of CT and US scan, the recognition of CI become easier, We report here imaging features of MRI in five term babies suffering from CI and we compare them to US and CT scan findings in the neonatal perlod. A 0.5 T CGR GE magnet is used. T1 (600/32 msec), Gradient Echo (GE) (450/12 msec/90 ~ and T2 (2000/120 msec) are utilised. The MRI charecteristics can be divided in two overlapping stage : stage 1 or edematous phase early after birth in which CI territory demonstrates an homogenous signal intensity lower than white matter's hut higher than cerebral spinal fluids on T1 or GE. A midline shift and absence of gyral definition is also noted. US shows altered parenchymal echogenicity and CT scan hypedensity. Multiplanes images allows us to delimitate the CI with accuracy. In 4 cases whole territory of right middle cerebral artery (MCA)was involved whereas in the remainder only a distal branch of l e ~ MCA was-cg~ceFne~. F-afeac~v{~al hemorrhage was associated in 2 cases. Two weeks after : stage 2, cystic spaces were well developped on MRI, hemorrhage was still identified whereas on US and CT scan altered parenchymal echegenicity and hypodensity persisted. From these features, MRI enabled us to date the occurence of CI. In one infant prenatal occurence was evident on MRI at 3 day s old (stage 2) whereas in US and CT scan findings couldn't differ it. Furthermore, old and fresh hemorrhage were easily identified on T2 in this case. MRI seems to be more accurate in the recognition of CI in the neonatal period and may be of great interest during the evolvlng stage from infarction (stage i) to liquefication and ultimately cavitation (stage 2) which occurs sooner than presumed. * Service de N6onatologie, ** Radiop6diatrie Radiologie 2, CHU de Hautepierre, 67098 STRASBOURG Codex



Interpretation of sub-ventricular pseudo-cyst

CLINICAL RELEVANCE AND YALIDITY OF CEREBRAL DOPPLER ULTRASONOGRAPHY IN NEWBORNS BY LONG TERM REGISTRATION W. Kirsch, K. K a l j u and P. Mayer ........................................................... Analysis o f Doppler-frequency curves of big b r a i n vessels i s based on average values obtained from only few h e a r t cycles, because so f a r t e c h n i c a l problems prevented rang term measurements. Now these measurements ere p o s s i b l e by means o f g adequately adapted m i n i a t u r i z e d system which i s able to perform eontinous measurements end trend a n a l y s i s [HP 77020 AC m o d i f i e d ) . Comparison o f manual and computer supported e v a l u a t i o n methods showed the f o l l o w i n g r e s u l t s : because of the i n t e r n algorithm the automatically calculated values of v max (maximal blood flow velocity) were too low compared to values obtained by manual analysis using identic low Doppler intensity. In regard to clinical validity further s t u d i e s showed t h a t v mean (overage blood f l o w v e l o c i t y ) seems to be the best parameter. In 20 quiet and not acutely affected preterm neonates, the blood flow v e l o c i t i e s of internal carotid orterias showed distinct semi~periodicol oscillations oF the Doppler curves. These oscillations were independent of blood-pressure end breothieg-frequency. In these potients the peek flow systolic velocities showed o standard deviation of 3.3 5.2 %. Interpretation of these results, which POURECELOT else observed in adult patients, is difficult. Statistical onQlysis of individual Doppler oscillations demonstrates a intrQ-individuel stability, on the other hand inter-individual comparison showed distinct differencies. These differencies ore caused by individual mechanisms of cerebral blood flaw regulation not well understood so for. More then now, these oscillation of Doppler curves in long term meosurements must be token into consideration in the interpretation of short term registrotion of Doppler frequency curves.

J.H.Lu,D.Hanau,D.Emons,S.Kowalewski $ D.Hoffmann,H.J.F6disch $$ Department of Neonatology,University Bonn,FRG ~$Inst.of Pediatric Pathology,University Bonn,FRG Senolucent lesions are occasionally detected in the subependymal germinal matrix, at the upper external angle of lateral ventricle and anterior or at the level of the foramen of Monroi. These are most commonly believed to be the sequelae of prior subependymal germinal hemorrhage that has undergone cystic deEeneration. Central liquefaetation in an area of prior hemorrhage is believed to be the most common cause of subependymal pseudocyst formation in neonates. We examined postmortem 12 brains with subependymal germinal hemorrhage at different stages.The serial sonographic images (Acuson,5~z) are attempted to correlate with the corresponding brain sections. We have seen 5 cases with pseudocyst phenomenon in the subependymal germinal matrix in whom there was no evidence of central liquefactation. The echogenic wall of pseudocyst is not hematoma itself but the surrounding tissues. Increased periventricular eehogenicity around the hematoma is due to edematous changes of the surrounding tissues or even leukomalacia. The organized subepemdymal hematoma itself is homogeneous aneehogenic. This study emphasizes the better knowledge of pathology for interpretation of subependymal pseudocyst and its associated changes. So it is necessary to remind cautiouslybefore predicting the cystic changes of subependymal hemorrhage.

Kinderkl*nik der St6dt. Kliniken Winterberg D-6600 SaarbrOcken, Theodor-Heuss-Str.


Abstracts. 27th Congress ofthe European Societyof Paediatfic Radiology

11 MR IMAGING OF INTRACRANIALHEMORRHAGE IN ABUSEDAND OTHERWISE TRAUMATIZED CHILDREN M. Reither, E. Imschweiler, R. Lindner For biochemical and physical reasons CT is the image modality of choice in patients with acute intracranial hematomas. In subacute and chronic hemorrhages methem~]globin shortens TI- and T2-times significantly. So MRI with its advantages superior to CT in children is very helpful in detection and characterization of intracranial hematomas especially in repeatedly abused children. Moreover MR imaging best discloses small subdural hematomas often not detectable in CT scans due to beam hardening effect, small concurrent intrafalcial bleedings caused by whiplash injuries, and other concurrent small intracerebral hemorrhages. This paper focuses on our MRI experiences in 12 patients with intracranial hematomas due to: child abuse (4), road accidents (3}~ sports activities (2), and spontaneously in vascular malformations (3). We compared ~ results as far as reasonable and possible with ultrasonographic and CT findings. In some patients follow-up studies were done emphasizing the value of MRI as control examination. Radiol. Zentrum - Diagnostik / R~ntgenpraxis Tennenlohe St~/dt. Klinikum Flurstr. 17 D-8500 N~rnberg 91

MRI OF C E N T R A L D I A B E T E S I N S I P I D U S : 5 C A S E S C. C h r i s t o p h e , D. B a l 6 r i a u x , S. H a n q u i n e t , C. H e i n r i c h , E. Sariban, C. S e g e b a r t h and N. P e r l m u t t e r Five o b s e r v a t i o n s of c e n t r a l d i a b e t e s i n s i p i d u s d e m o n s t r a t e the p o t e n t i a l of MRI to e x p l o r e the h y p o t h a l a m o h y p o p h y s i a l axis. Various etiologies w e r e observed: a b n o r m a l s i g n a l of the p o s t e r i o r p i t u i t a r y g l a n d in a case of " i d i o p a t h i c " d i a b e tes insipidus, l o b a r p r o s e n c e p h a l y , h i s t i o c y t o sis X, m a l i g n a n t t e r a t o m a and c r a n i o p h a r y n g i o m a . The h y p e r i n t e n s e s i g n a l of the p o s t e r i o r lobe of the p i t u i t a r y gland, n o r m a l l y o b s e r v e d in the T l - w e i g h t e d SE sequences, w a s absent. T h r e e out o f the all cases i l l u s t r a t e t h a t the d i a g n o s i s of p r i m a r y or i d i o p a t h i c d i a b e t e s i n s i p i d u s m u s t r e m a i n u n c e r t a i n for s e v e r a l y e a r s u n t i l r e p e a ted i n v e s t i g a t i o n s fail to d e m o n s t r a t e a n y signs of a n e o p l a s m or h i s t i o c y t o s i s X. The i n o c u i t y a n d the g e a t e r s e n s i t i v i t y of MRI, c o m p a r e d to CT, w i l l p e r m i t an e a r l i e r d i a g n o s i s of p a t h o l o g i e s r e s p o n s i b l e for s e c o n d a r y d i a b e t e s insipidus. However, MRI is n o n s p e c i f i c and has to be c o r r e l a t e d to the c l i n i c a l and the b i o l o g i c a l d a t a (including t u m o r a l t r a c e r s such as B - H C G and alpha-foetoprotein). The c a s e s of l o b a r p r o s e n c e p h a l y and h i s t i o c y t o s i s X i l l u s t r a t e t h a t in a d d i t i o n to the h y p o t h a l a m o h y p o p h y s i a l axis, the w h o l e p a r e n c h y m a l b r a i n has to be explored. G d - e n h a n c e d MRI a p p e a r s u s e f u l to del i n e a t e the e x t e n s i o n o f t u m o r a l p r o c e s s or of a b n o r m a l cell i n f i l t r a t i o n . MRI is the i m a g i n g m o d a l i t y of c h o i c e for the d i a g n o s i s and followup of c e n t r a l d i a b e t e s i n s i p i d u s . H U D E R F - S e r v i c e de R a d i o l o g i e - Av. J.J. Crocq, 15 - B. 1020 B R U X E L L E S - BELGIQUE.





We p e r f o r m e d 320 c o n t r a s t - e n h a n c e d MR e x a m i n a t i o n s of 112 i n f a n t s and c h i l d r e n aged 6 d a y s to 18 y e a r s with tumors of t h e c e n t r a l n e r v o u s s y s t e m and t h e s p i n a l c a n a l . Using a s u p e r c o n d u c t i v e m a g n e t (0.35 T e s l a ) , we a c q u i r e d TIand T 2 - w e i g h t e d images, followed by T l - w e i g h t e d images obtained immediately after i.v. ir0ection of Gadolinium-DTPA (0.I mmol/kg body w e i g h t ) . In no case side e f f e c t s were e n c o u n t e r e d . 29 t u m o r s were l o c a t e d in t h e s u p r a t e n t o r i a l region, 27 in t h e p i n e a l region, 97 in t h e p o s t e r i o r f o s s a and 19 in t h e s p i n a l c a n a l . The most common t u m o r s were p r i m i t i v e n e u r o e c t o d e r m a l t u m o r s (n= 19) and pilocytic a s t r o c y t o m a s (n=12). During i n i t i a l d i a g n o s i s , t h e m a j o r i t y of t u m o r s showed i n t e n s i v e e n h a n c e m e n t with i m p r o v e d d e m a r c a t i o n from n e i g h b o u r i n g s t r u c t u r e s . The solid c o m p o n e n t of a c y s t i c tumor, e v e n when small and not v i s i b l e on t h e original scan, could be d e m o n s t r a t e d in all c a s e s by m e a n s of Gadolinium-DTPA. T h e s e n s i t i v i t y of MRI for d e m o n s t r a t i n g spinal intradural extramedullary tumors was greatly improved by Gd-DTPA. During chemotherapy or radiotherapy, contrast-enhanced MRI p r o v i d e d reliable d e m o n s t r a t i o n of t h e s u c c e s s of t r e a t m e n t a n d a c c u r a t e l y d e m o n s t r a t e d the size of t h e t u m o r and t h e p r e s e n c e of necrosis. P o s t - o p e r a t i v e l y , T2-weighted scans regularly showed a r e a s of high s i g n a l s t r e n g t h a t t h e m a r g i n of t h e r e s e c t i o n ; in t h e a b s e n c e of a m a s s or d e m o n s t r a b l e p r o g r e s s i o n , t h e s i g n i f i c a n c e of this is u n c e r t a i n . In t h e s e c a s e s Gd-DTPA b e c a u s e of t h e f u n c t i o n of t h e b l o o d - b r a i n barrier, greatly increased accuracy in demonstrating r e s i d u a l or r e c u r r e n t tumors. I n s t i t u t fiir D i a g n o e t i s c h e Radiologie der H e i n r i c h - H e i n e Universit~tt, MoorenstraBe 5 , 4 Dfisseldorf 1, FRG


M. Argyropoulou, F. Perignon, F. BruneUe, R. Brauner, R. Rappaport The etiology of GHD ist often unknown. MRI was performed on SE, T1 saglttaI and coronal scans, 3 mm thick in 31 children with GHD. The PG height (FGH) was measured and compared to a control group (1). MR anatomy discriminatecl 2 groups. Group I: 12 children had PG stalk (PGS) interruption syndrome (PSIS), caracterized by: 1) absence of PGS and posterior lobe hypersignal, 2) hyperintense nodule in the infundlbulum of the 3th ventricule. Group If: 19 children had a norma I MR anatomy. The PGH in the 2 groups was inferior to normal values (p 0.001). Clinical and MR data were compared in the 2 groups: GROUP PGH (ram) GH (ng/ml) Multiple deficits Perinatal insults Facial malformation Head trauma

I (PSIS) 2,5 + 0.9 3.7 + 2A 86 % 75 % 2 1

II 3.6 + 1.1 6.6 + 2.4 14 % 25 %

p 0.02 0.001 0.05 0,05

GHD ist associated with dimi~shed PGH. There is a good endocrhaological and MR correlation. Abnormal MR anatomy (PSIS) is associated with a m o r e severe clinical presentation. (i) M. Argyropoulou, F. Ferignon, F. Brunelle Height of normal pituitary gland as a function of age evaluated by MR in children. Pedlar Radiol (submitted for publication) Sce de Radiol P6d Hopital des Enfants Malades 149, rue de S6vres, 75730 Paris Codex 15 (France).

Abstra~s. 27th Congress of the European Society of Paediatfic Radiology

13 C O N T R I B U T I O N OF NBP TO THE D I A G N O S I S L E S I O N S OF A D R E N O L E U K O D Y S T R O P H I E . G. K A L I F A ~, P. D E M A N G E ~, A. PASCO ~, J.L. S A R R A Z I N * and P. A U B O U R G ~


15 OF C E R E B R A L

MRI examinations were performed in 31 males with bioehemieally proved adrenoleukodystrophy (ALD}. 13 p a t i e n t s had c h i l d h o o d o r juvenile c e r e b r a l ALD, 8 p a t i e n t s had a d r e n o m y e l o n e u r o pathy (AMN) and i0 were n e u r o l o g i c a l l y asympt o m a t i c boys w i t h or withoul a d r e n a l i n s u f f i c i e n cy. In c e r e b r a l ALD, MRI was r e m a r k a b l y sensitive in d e m o n s t r a t i n g lesions in the w h i t e matter l o c a t e d w i t h i n the a u d i t o r y and v i s u a l p a t h w a y s , the c o l l i c u l u s and the p y r a m i d a l tracts w i t h i n the int~nal c a p s u l e s and the pons. F u r t h e r m o r e , a two d i s t i n c t temporal phase was identified by the v a r i a t i o n in the a b n o r m a l white m a t t e r T2 signals. MRI d e t e c t e d white m a t t e r lesions in 6/10 n e u r o logically asymptomatic boys : four of these patients became neurologically symptomatic within the two following years. MRI lesions in the brain white m a t t e r therefore a p p e a r as r e l i a b l e p r e d i c t o r s of c l i n i c a l ALD. 4/8 AMN p a t i e n t s had white m a t t e r lesions in the b r a i n and 3/4 of these p a t i e n t s h a d m o d e r a te to severe n e u r o p s y n h o l o g i c a l deficits. This s u g g e s t s that c e n t r a l n e r v o u s s y s t e m i n v o l v e m e n t is more f r e q u e n t in AMN than p r e v i o u s l y t h o u g h t on the basis of c l i n i c a l and CT data.

E V O L U T I O N OF MRI FINDINGS IN A Y O U N G CHILD WITH BRAIN CYSTICERCOSIS AFTER THERAPY WITH P R A Z I Q U A N T E L A Beutler, Ch, A, Haenggeli and D, Nussl6 Cyslicercosis is a common parasitic disease of the nervous system in developping countries, and occurs also in industrialized nations with high rate of immigration. Therapy of neurocystlcercosis with antiparasitlc agents is still controversial in childhood, because the disease appears to be selflimlted in the majority of cases, and because adverse reactions after medication occur in almost all patients, due to a flare-up of inflammatory reaction. Clinical, laboratory and MRI find{ngs before and 3, 7, 10 and 16 months after Praziquaetel therapy in a 4 1/2 year-old glrl are presented. She had numerous partial and generalized seizures since the age of 12 months, after a stay in Haiti between 4 and 8 months. The diagnosis of parcnchymal brain cysticercosis was made 3 1/2 years later with CT and serological analysis of blood and CSF. CT showed multiple parenchymal calcifications without foei of enhancement. MRI showed no abnormal signal in T1 weighted images. T2 imaging demonstrated several large areas of high signal intensity in the grey and white matter, surrounding hypointense loci, suggesting active inflammatory lesions. Three months after Praziqoantcl therapy new inflammatory areas were seen al well as small rings with high intensity around calcified loci, concommitent with a clinical exacerbation and increasing serological tlters. Follow up examinations showed progressive disappearance of inflammatory lesions with persisting calcified loci and serological Improvement. These MRI studies demonstrate that calcified loci can still be active, an increase of the inflammatory process after antiparasitic treatment, followed by complete inactivation. Unit6 de Radiop6diatrie and Clinique des P6d~atrle HCUG, Ch-1211 Geneva, Switzerland

9 D e p a r t m e n t of R a d i o l o g y , 9 * I N S E R M U 188 D e p a r t m e n t of P e d i a t r i c N e u r o l o gy, Hop. Saint V i n c e n t de Paul, 74 avenue Denfert Rochereau 75674 PARIS CEDEX 14 (FRANCE)



MAVLE SYRUP URINE DISEASE. CT AND MR OF THE BRAIN. J. Brismar, G. Brismar, R. Coates, G. Gascon, P. Ozand

S E R I A L MR-STUDIES IN M U L T I P L E SCLEROSIS IN C H I L D H O O D F. Ebner, M. Millner, K.A. Hansegger, R. Fotter, G. Ranner, E. Iustich

Maple syrup urine disease (MSUD) is a rare enzymatic defect blocking the catabolism of the branched chain amino acids. The affected child is usually normal for a few days after birth, then rapidly deteriorates with failure to thrive, truncal hypotonia, convulsions and stupor progressing to cc~a. Without treatment the infant often dies within the first few weeks. An early, rigid diet may prevent this course and even preserve a normal intellect. The cranial CT and MR findings have previously only been presented in scattered case reports. We have performed totally 26 CT and 13 MR examinations in 12 patients with MSUD during different stages of the disease. As the age at diagnosis varied from 3 days to 7 months our results, for the first time, document the natural course of the disease. CT is negative during the first few days of life, then a generalized edema appears. In addition, a more severe, localized edema is seen extending from the deep cerebellar white matter up through the cerebral peduncles and through the posterior limb of the internal capsule; these changes are best appreciated from T2 weighted MR registrations. Both t~he local and the generalized edema subside during the second month of life, often leaving well defined low density scars; some loss of brain substance then also becomes obvious~ The CT and MR changes are typical enough to permit an early diagnosis of MSUD. Department of Radiology, King Faisal Specialist Hospital and Research Centre, P. O. Box 3354, Riyadh 11211, Saudi Arabia.

The aim of the study was to assess CNS focal white matter abnormalities in childhood MS, and to correlate changes in appearance over time with clinical courses of the disease. Methods: Superconductive magnet 1.5 T. Pulse sequences: IR 1 5 0 0 [ 4 5 0 / 3 0 / 4 , PSSE 450/20/4, SE 2500/30/60/2. Imaging in transverse and sagittal planes. Subjects: From 1/87 - [X/89 873 consecutive children (age 0-18 years) were referred to MR of the CNS. Within the age group 10-14 years 7 patients had clinically definite (n = 5) and laboratory supported definite (n = 2) multiple sclerosis (MS) (Ann. Neurol. 13: 227,1983). Results: The average onset of MS in the present series was 12 1/12 years. The femal male ratio was 5:2. The patients presented clinicly with motor symptoms (4/7), visual disturbances (3/7), urinary dysfunction (2/7), gate disturbance (2/7), vertigo (2/7) and nystagmus (1/7). From all paraclinlcal tests MR proved to be the most useful paraelinical test for establishing the clinical diagnosis of MS. All patients had brain white matter abnormalities which fulfilled the criteria of Paty's MR classification of MS (Neurot. 38: 180, 1988). In 4/7 patients with acute clinical attack large lesions (8-21 mm L~a~ ~ m a t a r ' i lttpro nro~ont ,tlh~oh d ~ m ~ n q t r o t ~ A o r~md,* l~rnollor structure on both T I - ~ proton density and T2-weighted images. On MR follow-up studies, performed in a series of 3-4 examinations, all children e/~hibited changing patterns of their CNS signal abnormalities. In 3/6 cases clinically siIent brain lesions were detected. The lesions exhibited the best contrast to cerebral white matter on long TR]short TE spin echo sequences. Combined sagittal and transverse series, obtained with ECG-gating and flow compensating technique were proved best for repeated MR follow-up studies. MR could possibly be used to monitor the in vivo regression of the fresh demyelinating process. Furthermore MR may be of use to contribute to more reliable epidemiologie data on prevelance and incidence of MS in childhood. MR possibly offers a tool to evaluate therapeutic response in the acute MS-plaque. Depts. of Radiology and Paediatrics, KarI-Franzens Auenbruggerplatz 9, A-g036 Graz, Austria



Abstracts. 27th Congress of the European Society of Paediatric Radiology


17 S P E C T / M R C O R R E L A T I O N S IN C H I L D H O O D O N S E T EPILEPSY L.A,O'Tuama, S.T.Treves ............................................. T h e r e l a t i o n s h i p of f u n c t i o n a l and s t r u c t u r a l a b n o r m a l i t i e s in ii p a t i e n t s w i t h c h i l d h o o d onset epilepsy was evaluated using SPECT and MRI. E l e v e n p a t i e n t s a g e d 2.2 to 27 y e a r s u n d e r w e n t S P E C T (Tc-99m-HMPAO) and MRI. In 5 p a t i e n t s w i t h n o r m a l MRI, S P E C T d e t e c t e d a s y m m e t r y of c e r e b r a l b l e e d flow. I n the o t h e r six p a t i e n t s , b o t h M R I a n d S P E C T w e r e a b n o r m a l , b u t the e x t e n t a n d d i s t r i b u t i o n of t h e a b n o r m a l i t i e s d i d not c o r r e l a t e completely. S P E C T findings: in m e s i a l t e m p o r a l s c l e r o s i s (n=2) a n d t e m p o r a l lobe d y s g e n e s i s (n=l): p e r f u s i o n asymmetry, m o r e e x t e n s i v e t h a n M R I a b n o r m a l i t y ; in i n f a n t i l e c e r e b r a l v e n o u s t h r o m b o s i s (n=l~: c o n t r a l a t e r a l h y p o p e r f u s i e n n o t s e e n on MR; c h r o n i c h y p o x i c e n c e p h a l e p a t h y (n=l): n o n - p e r f u s i o n in m i d d l e c e r e b r a l a r t e r i a l d i s t r i b u t i o n , n o t s e e n on MR. In c o n c l u s i o n , this s t u d y d e m o n s t r a t e s t h a t in p a t i e n t s w i t h c h i l d h o o d o n s e t e p i l e p s y ; i) S P E C T d e t e c t s a b n o r m a l i t i e s n o t s h o w n b y MRI, a n d 2) w h e n b o t h S P E C T and M R I are abnormal on the same patient, SPECT appears to detect more extensive involvement, and gives more specific functional information t h a n MRI. Therefore, both modalities should b e u t i l i z e d to u n d e r s t a n d b e t t e r f u n c t i o n a l e f f e c t s of s t r u c t u r a l l e s i o n s of the b r a i n in p a t i e n t s w i t h c h i l d h o o d - o n s e t epilepsy. C h i l d r e n ' s Hospital, H a r v a r d M e d i c a l School, B o s t o n , M A 02115, U.S.A.


P. B a i e r l

H.P. N i e n d o r f

40 c h i l d r e n a n d a d o l e s c e n t s (aged 1 - 16 y e a r s ) w e r e e x a m i n e d b y M R I m a g i n g a t 1.0 T. G d - D T P A was g i v e n i n t r a v e n o u s l y at a d o s e Of 0.i m m o l / k g b o d y weight. Zn all c a s e s TIweighted SE sequences were used to demonstrate contrast enhancement. No adverse e f f e c t s w e r e seen. 30 p a t i e n t s h a d one or more lesions; in 20 patients contrast e n h a n c e m e n t w a s seen. In 4 c a s e s l e s i o n s w e r e not o b s e r v e d b y p l a i n M R and c o u l d o n l y be d e t e c t e d a f t e ~ Gd-DTFA. In addition, c o n t r a s t enhancement provided additional information about the differentiation of lesion from edema or n e c r o s i s in 13 p a t i e n t s . Normal b r a i n m a t t e r d i d not s h o w a n y c h a n g e s in signal intensity- However: an a g e - d e p e n d e n t signal increase was found in the normal v e r t e b r a l b o n e m a r r o w in all children. GdDTPA should be used as a supplementary e x a m i n a t i o n w h e n e v e r a t u m o r or an i n f e c t i o u s d i s e a s e of the CNS is s u s p e c t e d and p l a i n MRI is normal, or w h e n o r i g i n and e x t e n t of a lesion cannot be adequately defined with p l a i n MRI. J. H a u s t e i n MD, S c h e r i n g AG, C l i n Res M u e l l e r s t r . 170-178, D-1000 B e r l i n 65


NEURAL CREST DEFECTS J. M c C r e d i e R e d u c t i o n d e f o r m i t i e s of the limbs caused by contergan (thalidomide, distaval) can be interpreted r a d i o l o g i c a l l y by reference to maps of the segmental sensory nerve supply of the skeleton (sclerotome maps)C o n t e r g a n was a sensory n e u r o t o x i n with a short p h a r m a c o k i n e t i cally active period, but it caused an intractable sensory p e r i p h e r a l a e u r o p a t h y in adults. C o n g e n i t a l reduction deformity is seen as the embryonic equivalent o~ eontergan neuropathy, in which toxic damage to segments of the d e v e l o p i n g neural crest result in aplasia or h y p o p l a s i a w i t h i n the fields dependant upon the injured levels. A s s o c i a t i o n s of limb r e d u c t i o n deformities and m a l f p r m a ~ i o n s in internal organs can be a t t r i b h t e d to toxic autonomic n e n r o p a t h y related t o the damaged segment of neural crest . Thi 8 toncept of "neural crest defects" can be expanded beyond the c o n t e r g a n model to provide a logical iihterpretation for many congenital m a l f o r m a t i o n s of skeletal and visceral tissues. As a group in the h i e r a r c h y of birth defects, neural crest defects are separate from, but adjacent to, the w e l l e s t a b l i s h e d neural tube defects. D e p a r t m e n t of Surgery, Sydney, NSW 2006

U n i v e r s i t y of Sydney, Australia

Abstracts. 27th Congress of the European Society of Paediatric Radiology


Poster Presentations





The sonografic manifestations of periventrieular and intrsventricular hemorrhage in neonates have been well described by many investigators. In this study we examined pediatric patients with other cerebral pathologies excluding those with neonatal hemorrhage. 35 neonates and infants underwent both US and CT. The ages of the patients ranged between newborn and 12 months of age. All ultrasound examinations were performed with a real-time sector scanner using a 5-MHz transducer. No sedation was employed. The patients were scanned on Siemens Somatom 2 and CGR CE 12000 scanners. The indications for examination were enlarging head size, inflammatory disease, trauma, seizures, neural canal defects and eranio-facial anomalies. All the ultrasonic diagnosis were compared with CT findings. Overall sensitivity of US in detecting the intracranial lesions was 80 % (28/35). US showed the similar accuracy with CT in determining the abnormalities concerning about the cerebral ventricles (hydrocephalus, abnormalities of shape, ventriculitis) 100 % (22/22). US was insensitive in recognizing the brain lesions of 7 patients. These were 3 diffuse parenchymal disease, I lacunar infarct, I small cortical abscessand hematoma, I subarachnoid hemorrhage.

Neonatal vascular thrombosis not r e l a t e d to umbilical c a t h e t e r i z a t i o n is a r a r e e n t i t y with v a r i a b l e c l i n i c a l p r e s e n t a t i o n . We report the case of a f u l l term male neonate whose g e s t a t i o n was uneventful. Absence of spontaneous movement of the r i g h t arm at b i r t h oriented the f i r s t c l i n i c a l diagnosis to a r i g h t b r a c h i a l plexus injury. Additional findings such as cyanosis and absence of palpable pulse of the r i g h t armmotivated a d i g i t a l i z e d arcography and thoracic color duplex sonography. These revealed an o b s t r u e t i n g throrrbus in the r i g h t innominate a r t e r y causing a r i g h t subclavian steel with reversed flow through the r i g h t c a r o t i d and v e r t e b r a l a r t e r i e s . Color Doppler ultrasound demonstrated f u r t h e r thrcrnbi at the emergence of the l e f t corrrnon c a r o t i d a r t e r y and in the r e t r o h e p a t i c i n f e r i o r vena eava. No ombilical c a t h e t e r i z a t i o n had been performed p r i o r to angiography. A c o n t r a s t enhanced CT revealed a

right t~rporo pnrietal infarction. This status was

In conclusion US offers an accurate, non-ionizing diagnostic modality for early recognition of suspected hydrocephalus and their long term follow-up. However CT should be the initial screening procedure in defining the diffuse parenchymal lesions, tumors and subarachnoid hemorrhage.

confirmed by a t r a n s f o n t a n e l l a r Doppler US. The absence of neonatal symptomatology and the presence of a c a l c i f i e d , atrophic i n f a r c t were highly suggestive of a prenatal thr~-nbo-embolic event. Under conservative anticoagulant therapy, the follow-up showed the resolution of the l e f t c a r o t i d a r t e r y thrcrnbus, a rapid recuperation of the r i g h t arm function and delayed apparition of a l e f t hemiparesis at 3 months of age. The e t i o l o g y of t h i s presumed antenatal thraTbotic disease remained unknown : no coagulation ancmalies, e s p e c i a l l y no Antithrombin I l l and P r o t e i n C d e f i c i t in the patient and his parents has been detected. Suspected vascular throrrbosis of the neonate should benefit from color duplex sonography and DSA since c l i n i c a l l y unexpected extension may occur.

Department of Diagnostic Radiology, Cerrahpa~a Medical Faculty, University of Istanbul. 34300 - Istanbul, Turkey

Departenents of Radiology and Pediatrics, University Hospital, 1011 Lausanne, Switzerland.



G Y R I F O R M E N H A N C E M E N T ON CT BRAIN IN S E V E N INFANTS P. Close~ H. Carty G F r i f o r m e n h a n c e m e n t seen on CT s c a n is an u n u s u a l f i n d i n g unless a s s o c i a t e d w i t h a r t e r i o v e n o u s m a l f o r m a t i o n s (AVM). T h e r e are s p o r a d i c c a s e r e p o r t s in the l i t e r a t u r e of its o c c u r r e n c e in a s s o c i a t i o n w i t h h e r p e s s i m p l e x virus e n c e p h a l i t i s (HSVE), p u r u l e n t m e n i n g i t i s , f o l l o w i n g c h e m o t h e r a p y for l e u k a e m i a , in a c h i l d w i t h c h r o n i c r e n a l f a i l u r e , and in a c h i l d w i t h folic a c i d d e f i c i e n c y . We present a series of seven cases e x h i b i t i n g this p h e n o m e n o n , n o n e of w h o m h a v e AVMs, w h o h a v e b e e n s c a n n e d at this h o s p i t a l in the first 2 89 years f o l l o w i n g the i n s t a l l a t i o n of a CT scanner. F o u r of the c a s e s h a d c o n g e n i t a l h e a r t d i s e a s e r e q u i r i n g c o r r e c t i v e s u r g e r y or cardiac catheterisation. T h e o t h e r three h a d probable meningo-encephalitis. In all cases the g y r i f o r m e n h a n c e m e n t f o l l o w e d an i s c h a e m i c i n s u l t to the c h i l d ' s brain. We h y p o t h e s i s e that this p h e n o m e n o n is an i s c h a e m i c r e s p o n s e in the i m m a t u r e b r a i n and that its o c c u r r e n c e is not so r a r e as the l i t e r a t u r e may suggest.

S P I N A L C O R D A R T E R I O V E N O U S M A L F O R M A T I O N IN A CHILD. EMBOLIZA~ION UNDER SPINAL EVOKED POTENTIAL MONITORING. J. D u b o i s , F. B r u n e l l e , F. S a l f r a n q u e , P. L a c o m b e .






A five y e a r old c h i l d is r e f e r e d to our i n s t i t u t i o n for the t r e a t m e n t of a s p i n a l c o r d A V m a l f o r m a t i o n , r e v e a l e d by a p a r a p l e g i a a f t e r a fall. Pre e m b o l i z a t i o n w o r k up i n c l u d e d MRI and a n g i o g r a p h y a n d s h o w e d an a r t e r i o v e n o u s f i s t u l a l o c a t e d in the r e g i o n of the c o n u s and d r a i n i n g t h r o u g h a s i n g l e v e i n toward the p o s t e r i o r fossa. The a n t e r i o r s p i n a l a r t e r y c o n t r i b u t e d to the fistula, and e m b o l i z a t i o n was c o n s i d e r e d to be dangerous. Monitoring of the s p i n a l c o r d vaecularization was p e r f o r m e d thanks to e v o k e d p o t e n t i a l c o n t r o l of the s p i n a l c o r d d u r i n g e m b o l i z a t i o n . A b a l l o o n was i n f l a t e d and left in the f i s t u l a without neuro-physiological modifications. C o n t r o l MRI shows t h r o m b o s i s of the AVM. The p a t i e n t is w e l l w i t h o u t a n y c l i n i c a l d e f i c i t s . S e r v i c e de R a d i o l o g i e P @ d i a t r i q u e H o p i t a l des E n f a n t s M a l a d e s 149, rue de S~vres 75730 PARIS C ~ d e x France



Abstracts. 27th Congress of the European Society of Paediatric Radiology




INTRACRANIAL ABNORMALITIES OF CHILDREN WITH OPTIC NERVE HYPOPLASIA A.S. Hollman, J.S. Straiton and S.M. Zeki Optic nerve hypoplasia is an uncommon congenital abnormality which may range in severity from being asymptomatic to giving rise to blindness. The condition is associated with a range of CNS malformations, most notably, absence of the septum pellueidum (eepto-optie dysplasia ). We report the neuro-radiological features of a series of 20 children with optic nerve hypoplasia. The findings are related to the underlying neurological and ophthalmic status of the patient. Sixteen patients had undergone cranial CT and i0 underwent cranial ultrasound examinations. For the whole group the following features were identified; absent septum pellucidum (60~), hydrocephalus (45Z) , porencephaly (30~), absent corpus callosum (15Z), cerebral atrophy (SZ), and suprasellar epidermoid cyst (not previously reported) (SZ). In 5 cases the examination was normal (i CT and $ U/S). From the neurological stand point, the majority (17 cases) had manifest pathology (eg. cerebral palsy) but 5 patients were blind without additional manifest neurological disturbance. These patients were found to have dilated suprasellar and shiasmatie cisterns, septo-optic dysplasia, and mild hydrocephalus with poreneephalic cysts. Sixteen patients underwent detailed ophthalmic and endocrine assessment (I had died and ] failed to attend for follow-up). Eight patients had severe bilateral visual dysfunction with nystagmus, but the remainder demonstrated less severe visual defects. These patients all demonstrated neurological disturbance and/or hypopituitarism. Astigmatism was present in all cases and was more severe in cases with severe brain disorders. Patients with bilateral clinically manifest optic nerve hypoplasia merit neurological assessment and imaging, and warrant investigation for endocrine dysfunction.

Neurological outcome os PVb is well established. Cerebral ultrasound (US) in the neonatal period seems to be of a prognostic value but is limited in the follow up. The purpose of this study is to aware whether MRI can be as useful as US in predicting outcome and to measure the effect of PVL on brain maturation. Nine infants whose gestational age ranged from 27 to 32 weeks and one infant at term who experienced bilateral PVL are enrolled in this study. All underwent US and MRI scan. Neonatal MRI findings allowed us to classify PVL as following : parieto-occipital (PO) (9 cases) and mixed PVL (i case} (P0 + subcortical ) ; 8 PVL ; 2 hemorrhagic PVL (one mixed}. Neurological and electrophysiological (visual and auditory evoked responses) assessment was performed in all in the first year of life. MRI (CGR GE 0.5 T ; T2 2000/120 msec) was performed once or twice during the same period. All infants with PVL exhibited spastic diplegia.whereas hemorrhagic PV5 were associated with quadriplegla, hearing loss and blindaess. MRI fin&ings~uring the first year of life were as following : delay in myelination most marked in centrum semi-ovals, internal capsule and occipito-thalamic radiation. Reduction of white matter volume, irregulary dilated occipital horn and reduction of thikness of corpus callosum. On the contrary, white to grey matter dlfferenciation was not affected. From these preliminary results, we could emphasize that MRI in the neonatal period seems to constitute at short term a prognostic value especially when hemorrhagic PVL is detected. Functional abilities correlate well with MRI findings in the first year of life. Studies at long term are needed to confirm those data. Service de ntonatologie, ** Radiopddiatrie Radiologie 2, CHH de Hautepierre, 67098 STRASBOURG Cedex

Department of Radiology, Royal Hospital for Sick Children, Yorkhill, Glasgow G3 8SJ

25 PRE- AND POSTNATAL DIAGNOSIS OF ENCEPHALOCELES D.HSrmann, K.Meinel, E.Herrmann and W.Tischer The prenatal diagnosis of encephaloceles by means of sonography is possible frora about the 18. ~ . The evidence of a big cele containing cerebral parts up to the 24. GW. has nearly always the consequence of an interruption of the gravidity. Postnatally the clinical, sonographic and radiographic investigation must inform preoperatively on size and form of the ventricles, additional malformations and alterations of the skull. - From 1983 to 1989 4 frontal and 8 occipital encephaloceles were found prenatally by sonography in three confessional hospitals. In 8 cases the gravidity was interrupted, 4 children were born due to the delayed diagnosis; 3 of them died after short time. In the sccm~e period ii newborns with occipital encephaloeeles were hospitalized in the Clinic of Pediatric Surgery of the [q~U Leipzig - as a consequence of the improved prenatal investigation only one in the last three years. None of them had been recognized prenatally. 9 celes contained predominantely parts of the cerebrum with regressive alterations. Only in 3 patients a primary hydrocephalus was found, in 2 caused by Cniari-IIsyndroms and additional lumbal myelomeningoceles. 4 infants developed a hydrocephalus after operation. The hydrocephalus - %o he expected in about 2/3 of the children - is caused either by an additional cerebral malformation or by the postoperative blockage of the liquor circulation. The cele was removed in 9 infants, in 6 the hydrocephalus was shunted. 9 of the ii children survived up to now. - Because of the considerable mental retardation o{ nearly all children with encephalomyelomeningoceles an early prenatal diagnosis is essential to have the possibility of the decision whether or not to interrupt the gravidity. Klinik fur Kinderchirurgie der Karl-Marx-Universit~b OststraBe 21, Leipzig DDR 7o50

27 M A G N E T I C R E S O N A N C E I M A G I N G IN F E T A L A L C O H O L SYNDROME C. V a n de M e r c k t , C. C h r i s t o p h e , E.F. A v n i , H. S z l i w o w s k y , D. B a l ~ r i a u x , N. P e r l m u t t e r D i s o r d e r s of g y r a t i o n - a r e a related group of a n o m a l i e s b e l i e v e d to r e s u l t f r o m a l t e r e d m i g r a tion of neuroblast. T h e r e is n o w i n c r e a s i n g e v i d e n c e of g e n e t i c transmission o r at l e a s t a g e n e t i c s u s c e p t i b i l i t y o f t h e f e t u s to d i f f e r e n t insults (vascular, i n f e c t i o n , alcohol). We report two cases of f e t a l a l c o h o l s y n d r o m e (FAS) w i t h s o m e o f the classical characteristic anomalies : craniofacial disorders, cardiac, vertebral and costal anomalies, limb malformation and vesical disorder. In o n e o f t h e m , MRI o f t h e b r a i n s h o w e d a b n o r m a l thickness and folding of the cortex, typical of disorder of gyration. In the s e c o n d c a s e , MRI o f t h e s p i n a l c o r d w a s performed and showed a globulous appearance of the conus medullaris t h a t c o u l d b e r e l a t e d to the aspect of the bladder (neurogenic or nonneurogenio bladder?). Therefore we conclude that cerebro-spinal MR i m a g i n g is u s e f u l in FAS e s p e c i a l l y to d e t e c t cerebral gyration anomalies.

H U D E R F - S e r v i c e de R a d i o l o g i e - Av. 15 - 1 0 2 0 B r u x e l l e s - Belgique.



Abstracts. 27th Congress of the European Society of Paediatric Radiology



30 (1): ANATOMY,



I. Pastor, C. Prieto, P. Cortes and ML. Hoyo A) By studying the eyes in the form of echography one can observe its interior without any preparation (anesthesia), or radiation, seeing the normal anatomy: anterior chamber, lens, vitreous humour, retina, optic nerve, rectus muscles and retro-ocular fat. B) The congenital abnormalities correspond to the following echngraphy: 1) Congenital mlcrophtalmia, small but porportioned ocular globe 2) Congenital cataract, hyperechogenlc lense 3) Persistent primarious vitreous, hyperechogenlc band from papilla to the lense, caused by mesodermic remains 4) Persistent hyaloid artery, similar to above, with pulse 5) Coloboma, lack of fusion of the sphenoid gap, with small ocular balloon, with retro-ocular cystic mass 6) Coats disease, is a retinal angioma with retinal detachment and subretinal haemorrhage (2) The traumas and foreign bodies are re|ativeIy frequent in children, existing: 1) Vitreous haemorrhage and membranes, echoes and lineal bands in vitreous without anchored points, mobile 2) Vitreous with air, by irtclsion and lead pellets, hyperecbngenlc area with posterior shadowing artefacted, with mobility. Servicio de Radiologfa Pedi,'itrica, Hospital Infantil "La Paz", Paseo de Ia Castellana 261, 28046 Madrid, Espafia




Isolated vestibular aqueduct dilatation is a rare cause of fluctuant hearing loss and vertigo. We report 16 cases of such anomaly. The diagnosis is based upon computed tomography an axial viewj the normal aqueduct has a shape of an inverted "J". In one case~ MRI discloses the presence of lymphatic fluid in an enlarged vestibular aqueduct and endolymphatic sac. According to the different segments of the vestibular aqueduct on CT, one can differentiate several types of aqueduct dilatation : dilatation of the long often inilateral (3 cases), -




dilatation of the short and long limbs the "J" (5 eases),

"J" of

dilatation of the two limbs of the "J" with posterior petrous bulging corresponding to a large endolymphatic sac C8 cases) . These two last types are most often bilateral and the value of MRI must be pointed out. * Hop. Jean VERDIER, ,Av. du 14 juillet, BONDY 93i43 Hop. St VINCENT-de-PAUL, 74 Av. Denfert Rochereau PARIS 75014.





I. Pastor, C. Prieto, J. Gutierrez and L. Pablo D) Vitreous retinal disease: 1) Retinal detachment and its treatment, linealhyperechogenicband with anchoring stitches in the papilla area; in the stitched area we can see hyperechogenie nodals in the ora serrata 2) Corold detachment, bands from the ora serrata with anchoring at the sides of the papilla 3) Edema of the papilla, is the protrusion and increase in size of the papilla E) Masses. Retro-ocular, all can produce exophthalmos 1) Cellulitis, retro-ocular fat is infiltrated by diffusion of low echogenily, which could affect the muscles and the optic nerve 2) Dermoid cyst, a round mass with a good delimitation with irregular echogenity depending on its contents 3) Angioma, mass of mixed echogenity with small aueehoic areas ins{de 4) Neurofibroma, well defined mass near the papilla 5) Rhabdomyosarcoma,badly defined mass with rapid development near the muscles and of variable echogenity 6) Glioma, a well delimited mass of mixed echogenlty in the optic nerve 7) Neuroblastoma, with good delimitation in the mass of mixed echogenity 8) Histiocytosis, with increased rectal muscles 9) Cyst of the lacrimal duct, with well delimited cystic mass, middle ocular balloon Intra-ocular, not causing exophtalmus. 1) Retinoblastoma, simple or multiple hyperechogenlcmass with calcium inside, associated to the detachment of the retina; hereditary and bilateral (66%) z) Angioma, subretinal mass with detachment and haemorrhage of the retina, in Sturge Weber disease. Servicio de Radiodian6stico, Hospital lnfantil "La Paz", Paseo de la Castellana 261, 28046 Madrid, Espafia

Involvement of i n t r a c r a n i a l vessels is responsible for epilepsy, paresis and dementia in the Sturge-Weber syndrome. Neurological symptoms and outcome depend on the extent and d i s t r i b u t i o n of leptomeningeal angiomatosis, which can be visualized for the f i r s t time by MRI using Gadolinium-DTPA enhancement (Gd-DTPA). This report is based on the study of four children, aged 7 to 19 months, who presented with cutaneous, neurologic and ocular symptoms at the time of MRI examination. With T2-weighted images, angiomatous a l t e r a t i o n s of the skull, a t y p i c a l l y located and congested intracerebral veins as well as changes secondary to the leptomeningeal angiomatosis (brain atrophy, accelerated myelination) are demonstrated. Gd-DTPA enhanced Tl-weighted images e x h i b i t c l e a r l y the regional d i s t r i b u t i o n of angiomatosis in the skull, meninges and in brain parenchyma. Prior to possible recognition of c a l c i f i c a t i o n s by CT, MRI is the method of choice to discover i n t r a c r a n i a l involvement. Enhancement with Gd-DTPA improves the diagnostic value of MRI before neurological symptoms appear. Follow-up studies with Gd-DTPA enhanced MRI demonstrate also thrombotic changes of leptomeningeal angiomatosis as well as subsequent cerebral impairment, whereas the CT is more sensitive for incipient c a l c i f i c a t i o n s in this disease. Kinderklinik (KAVH) des Universit~tsklinikum Rudolf Virchow der Freien Universit~t Berlin, Heubnerweg 6, 0-1000 Berlin 19

Abstracts. 27th Congress of the European Society of Paediatric Radiology




CT DEMONST~IATION OF DIFFUSE INFARCTION IN UNEXPLAINED CHILDHOOD ENCEPHALOPATHY J.S. Straiton, A . S , H o l l m a n , E.M. T e a s d a l e and J . B . P . Stephenscn Diffuse low attenutation in both hemispheres with sparing of central structures on CT has been labelled the "Pattern Reversal" sign, cortical gray matter showing apparent reduced attenuation in comparison with white matter. Most reported examples are cases of non-accidental injury in infancy, the reversal sign being accompanied by other stigmata of trauma. In cases where there is no history or evidence of trauma, the significance of this sign is less clear, although the prognosis is uniformly bad.


In 3.5 years, 6 examples of pattern reversal and a further 6 with more widespread low attenuation have been identified in patients aged between 5 weeks and 2 y e a r s referred for CT. Patients bad been previously well, with illness rapidly progressing to coma. A history of traLLma was forthcoming in only one instance, with respiratory distress OF hypotension a feature common to all cases. EEG when performed showed extensive abnormality with low amplitude or featureless recordings in extreme cases. It is concluded that the reversal sign is a non-specific manifestation of a severe ischaemie insult to the brain, with co-existing systemic disease in many cases. Careful scrutiny for evidence of trauma is justified where details of the preceding history are vague, although there will remain a sub-group in which no distinction can be made between "near-miss" sudden infant death and non-accidental asphyxia. Department of Radiology, Royal Hospital for Sick Children, Yorkhill, Glasgow G3 8SJ






CEPHALY ~.B. Towbin and K. Hacker

Lissencephaly is a migrational anomaly, presumably due to arrest of neonatal migration between the llth & 13th week of gestation. It has been recently associated with chromosomal deletion and may be genitically inherited. At times it is difficult to distinquish lissencephaly from immature brain, however, we believe that there is a characteristic appearance of the periventricular white matter, and since this reflects pathogeaesis of the disease, is pathognemosic. 9 children with lissencephaly, ranging in age from 3 mos to 9 yrs were evaluated using CT or MRI. CT was performed on 6 cases; 5 of 6 had non-enhanced and 3 of 6 had enhanced examinations. 3 were evaluated with MRI using both T1 and T2 weighted sequences. Ventricular enlargement was presented in all cases. There was triangular or rectangular shaped enlargement of the sylvian fissures in all cases. All showed a smooth brain surface with some pashygyria noted i 5 cases. In all instances the grey matter to white matter ratio was increased, the white matter was angularly distributed around the ventricles, was hypodense on non-enhanced CT and hyperintense on T2 weighted MRI, and did not interdigitate with the grey matter at the level of the centrum semiovale. In addition, a hypodense TI hyperintense subcortical band was noted in all 9 patients. From our experience and reviews of the literature, we believe that the angular appearance of the white matter on CT and MRI is diagnostic, and permits differentiation of normal immature brain from those with lissencephaly. (hildren'sHospitalof Michigan, Dept. of Radiology, Wayne State University, 3901BeaubienBo~levard, Detroit, Michigan 48201 U.S.A.

33 MAGNETIC RESONANCE IMAGING OF THE FETAL DISEASES (14 CASES) P.Toma', M.A. Lituania 12 fetuses (21 to 37 weeks of pregnancy), with an anomaly detected by sonography (US), were studied in utero by Magnetic Resonance Imaging (MRI) using a 0.5 Tesla Magnet. A fetal ne~romuscolam blockade, during MRI, was obtained by intravenous curarization using Pancuronium Bromide (0.08 mg/kg) injected into the umbilical vein under US guidance. 2 fetuses were studied only by post mortem MRI. Our series consists of: - Schizencephaly (1 case) - Holo prosencephaly (semilobar: i case; lobar: i case) - P o r e n c e phaly (l case) - Dandy Walker syndrome (1 case) - A r a c h n o id cyst (i case) Hemorrhagic cyst in posterior fossa (l case) - Spina Bifida (3 cases; 2/3 studied only postmortem) - Osteogenesis Imperfecta (i case) - Hydrothorax (2 cases) - Wilms tumor (I case). All the cases were studied after the abortion (6) or at birth ( 8 : 4 / 8 in vivo, 4/8 post mortem) by Rx and/or US and/or CT and/or MRI. Fetal move_ ments are the main drawback to prenatal use of MRI. I n t r a vascular fetal curarization results in adeguate suppres_ sion of fetal activity (we did not observe damages for the fetus). US remains the modality of choice in fetal imaging. Fetal MRI, on the contrary of US, allows a multiplanar s t u dy, but, sometimes, the position in utero does not permit a correct study of principal planes. The MRI images quali_ ty of the fetus is often worse than the US one, particula_ fly under 30th week of pregnancy. We obtained a good visi_ bility only of the cerebral and abdominal lesions. Post mortem MRI is very useful: it may bring essential informs_ tions in order to obtain a specific diagnosis. -

G. Gaslini Institute, Largo G. Gaslini, 16148 Genova-Italy

Abstracts. 27th Congress of the European Society of Paediatric Radiology


Skeletal System Oral Presentations 35. CONTRAST CHARACTERISTICS OF PEDIATRIC BONE MARROW ON GRADIENT RECALLED ECHO IMAGING G . H . S e b a g 1-2, S.G. M o o r e 2 G r a d i e n t R e c a l l e d E c h o (GRE) i m a g i n g is b e c o m i n g increasingly u s e d in p e d i a t r i c musculoskeletal imaging since this technique allows fast MR Imaging with the a d v a n t a g e of a 3D acquisition. However b e c a u s e of t h e l a c k of 180 ~ refocusing pulse GRE is not d e p e n d e n t o n T2 b u t on T2* which is the e f f e c t i v e transverse relaxation time. To e v a l u a t e t h e i m p a c t of t h i s T 2 * d e p e n dence on bone marrow contrast, 17 n o r m a l e x t r e mities ( 6F, 6M, 5 m - 2 5 years ) and i0 patients (5F,5M, 3-25 years) with traumatic, neoplastic, vascular and esteopenic lesions (n=14) were e x a m i n e d on a 1,5 T m a g n e t . S p i n e c h o (TI 6 0 0 / 3 0 a n d T2 2 0 0 0 / 2 0 , 8 0 ) a n d G R E i m a g e s (TR 60, flipa n g l e 30 ~ , T E = 1 0 , 1 2 , 1 6 , 2 0 , 2 4 , 3 0 , 4 0 msec) were compared. The s t u d y s h o w s t h a t m a r r o w in c o n t a c t with trabecular bone exhibits a shortened T2* relaxation time and resultant signal loss in healthy subjects. This is d u e to l o c a l field gradients resulting in i n h o m e g e n e o u s suspectibilily where the m i n e r a l i z e d matrix interfaces with marrow. T h i s T 2 * e f f e c t is increased in t h o s e r e g i o n w i t h a g r e a t e r a m o u n t of t r a b e c u l a r bone (epiphysis) as c o m p a r e d to t h o s e regions w i t h l e s s o r no t r a b e c u l a r bone (diaphysis) and by increasing TE. Knowledge of t h e e f f e c t of trabecular b o n e on G R E is i m p o r t a n t in the analysis of MR studies for three reasons : low signal intensity on GRE may represent fatty marrow w i t h a h i g h c o n t e n t of t r a b e c u l a r bone, and s h o u l d n o t be i n t e r p r e t e d o n l y as r e d marrow. Secondly the d e t e c t i o n of epiphyseal lesions is improved by decreasing TE a n d pixelsize. Finally, GRE may have a potential r o l e in the e v a l u a t i o n of o s t e o p e n i a . 1-Hop.Enfants-malades,Paris, France.2-Stanford USA











AND 24

CASES. Kozlowski K,Campbell J,McAlister W,Masel J,Taccone A. Primary tumours of the cranial vault and base of the skull with exeption of Eosinophilic Granuloma and Epidermoid/Dermoid tumours are rare in childhood. Twenty four cases with such rare lesions are reported. These include 4 children with Haemangioma, 30steoma, i 0steochondroma, 2 0steoblastoma, 2 Progonoma, 3 post radiation 0steosarcoma, 1 E w i n g Sarcoma, 4 Chordoma and 4 not well classified~ most likely fibrous benign lesions. The differential diagnosis of the cranial vault and base of skull tumours and tumorous lesions is discussed. The difficulties of both the X-ray and pathological diagnoses are stressed. The value of CT and NR imaging in delineating the exact location of the tumour and its

relationship to adjacent structures is illustrated by some representative cases.

Royal Alexandra Hospital for Children,Sydney,Australia The Children's Hospital, Denver, Colorado, USA Mallinckrodt

Institute of Radiology,

St.Louis, Miss, USA

Children's Hospital, Brisbane, Qnsl, Australia. Istituto Giannina Gaslini, Geneva, Italy.


Severs combined immunodeficisncy (SCIO) with absent or reduced activity of adenosine deaminase (ADA-) in red blood cells and other tissues is now a well-established clinical entity within the defects of the immune system. We report on two affected out of three children from healthy, unrelated parents. The first male young infant died at the age of nine weeks from bacterial pneumonia, septicemia, thrush and ultimately electrolyte imbalance. SCID was suspected but could not be confirmed during lifetime. the second female child is by now three years old and healthy.The third infant presented as s ten week old girl with failure to thrive and BCG-lymphadenifis. Her total white count was below 2000/ul with lymphocyte count below 150/ul. Chest X-ray revealed absence of thymus as well as splayed and cupped costochondral junctions. Further radiologic abnormalities included thick growth arrest lines, a broad pelvis with squared-off ilia, horizontal and long ecetabular roofs, and spiculse formation. Similar alterations could be detected in the first child by post-mortem reevaluation of his radiographs. In the patient, diagnosis of SCID ADA- was confirmed by gel electrophoresis of lysed erythrocytes showing no enzymatic activity while her parents and sister were heterozygotes. Without prior conditioning the patient was grafted with unmanipulated bone marrow from her HLA-identical sister. Post transplant course was uneventful except of severe generalized BCG-infection which was successfully treated by tuberculostatic drugs. Eight months post transplantation radiographs showed regular pelvic structure and impressive normalisation of costochsndral junctions. Thus the developing immune system as well as the developing skeletal system share a requirement for ADA activity and impaired functions in both systems are restored by bona marrow transplantation. Dpt of Pediatrics; Univ of Kiel, Schwanenweg 20, 2300 Kiel and (*) Dpt of Pediatric II, Univ of Ulm, West-Germany

BONE MARROW METASTASES IN NEUROBLASTOMA: MRI IN CORRELATION TO BONE MARROW CYTOLOGY AND MIBGSCINTIGRApHy G. Benz-Bohm, W. Gross-Fengels, B. Widemann, A. Linden Thirteen MRT-investigations of the knee region of nine children with neuroblastoma stage IV/III were performed in order to demonstrate bone marrow metastases. The MRT-findings were correlated with bone marrow cytology, immunofluorescence and mIBG-scintigraphy. MRT showed a high sensivity in demonstrating bone marrow abnormalities. Bone marrow aspiration and mIBGscintigraphy will still be needed to make a specific diagnosis. Evidence of hone marrow infiltration only by MRT does not exclude bone marrow metastases. Institnt und Poliklinik f~r Radiologische Diagnostik Joseph-Stelzmann-Str. 9, D-5ooo K~ln 41

Abstracts. 27th Congress of the European Society of Paediatric Radiology




C o n g e n i t a l hip d i s l o c a t i o n is the result of i n t e r a c t i o n of m u l t i p l e factors: a c e t a b u l a r m o r p h o m e t r y , soft t i s s u e e l a s t i c i t y and e x t r i n s i c m e c h a n i c a l factors. The i m p o r t a n c e of the r a c i a l i n f l u e n c e is r e f l e c t e d in the v a r y i n g i n c i d e n c e of c o n g e n i t a l hip d i s l o c a t i o n in d i f f e r e n t races. The i n c i d e n c e is r e l a t i v e l y low in C h i n e s e infants. To e x p l o r e the p o s s i b i l i t y of i n t r i n s i c d i f f e r e n c e s in the a n a t o m i c or the d e v e l o p m e n t a l p a t t e r n of the a c e t a b u l u m , an u l t r a s o u n d study of 240 n o r m a l Hong K o n g C h i n e s e infants aged 2 days to 7 m o n t h s w i t h e q u a l sex d i s t r i b u t i o n was done u s i n g G r a f ' s technique. Ultrasound imaging with 7.5 MHz or 5 MHz l i n e a r t r a n s d u c e r s b y a s t a n d a r d c o r o n a l a p p r o a c h w i t h the b a b i e s in the d e c u b i t u s p o s i t i o n was used. The a l p h a and b e t a angles w e r e m e a s u r e d as a f t e r Graf. 1050 hip e x a m i n a t i o n s w e r e performed. The f i n d i n g s w e r e c o m p a r e d w i t h f i g u r e s o b t a i n e d in W e s t e r n p o p u l a t i o n .



We report 5 newborns with a contracted pelvic outlet, imperforate an~m, absent or rudimentary urinary tract and g e n t l y defective or absent ~ a a l genitalia, v~gina and uterus but normal gonads. The first 2 patients had small pelvic outlet syndrome (SPOS) (A/R 109:399, 1970 - Radiol 101:629, 1971 Pediatr 53:270, 1974). The findings in the 3rd patient were more severe, probably midway between SPOS and sirenomelia. The 4th and 5th patients had sirenomelia with the characteristic fusion of the lower limbs (somewhat atypical in Case 4). The similarity of many abnormalities observed in these cases and related material in the literature suggests that SPOS and sirenomelia form a family or a continuum of malformations, probably the result of an insult to a developmental field at the caudal end of the embryo very early in gestation.

Departments of Radiology and Pathology, C~aildren's Medical Center of Dallas and The University of Texas Southwestern Medical Center at Dallas, 1935 Motor Street, Dallas, Texas 75235, USA.

A d i f f e r e n c e was n o t e d in the v a l u e s of the acetabular morphometric parameters. The t r e n d of a c e t a b u l a r d e v e l o p m e n t was similar. D e p a r t m e n t of D i a g n o s t i c R a d i o l o g y and O r g a n Imaging, Prince of Wales Hospital, Shatin, New Territories, Hong Kong



THE BONY PELVIS AS D I A G N O S T I C INDICATOR: A) THE S C H N E C K E N B E C K E N - D Y S P L A S I A (SBD) , B) THE T R I S O M Y 8 M O S A I C I S M (T8M), C) THE S M A L L P A T E L L A S Y N D R O M E (SPS), D) A N E W S Y N D R O M E S I M I L A R TO SPS, BUT W I T H I M M U N O D E F I C I E N C Y AND H Y P O P L A S T I C KIDNEYS A~ G i e d i o n r Ch. B e y n o n r G. E i c h and A. S c h i n z e l _


A) The p a t h o g n o m o n i c c o n f i g u r a t i o n of the iliac w i n g in the SBD, a AR lethal type of n e o n a t a l short limbed p l a t y s p o n d y l i c d w a r f i s m r e s e m b l e s the s i l h o u e t t e of a snail s t i c k i n g its n e c k of the shell medially. The r a d i o g r a p h i c and h i s t o logic f i n d i n g s o b s e r v e d in a f a m i l y w i t h 3 affected s i b l i n g s are d e m o n s t r a t e d . B) The "typical iliac horns" (IH) of 3 cases w i t h T8M w e r e e x a m i n e d by CT: T h e y are c a u s e d by an e x c e s s i v e l y large s p i n a iliaca a n t e r i o r superior, w h i c h may be f o l d e d in m e d i a l l y or may p o i n t c a u d a l l y or laterally, thus p r o d u c i n g q u i t e d i f f e r e n t a p p e a r a n c e s in the ap-view. A second "horn" m a y be o b s e r v e d , due to a b o n y p r o t u b e r a n c e s t i c k i n g out d o r s o - l a t e r a l l y f r o m the iliac crest. C) The A R SPS (Scott and Taor) m a y show a striking h y p o p l a s i a of the i s c h i u m t o g e t h e r w i t h h y p o p l a s t i c or a b s e n t patellae. Such a case, and D) a s i m i l a r one, but t o g e t h e r w i t h p o l y d a c t y l y , i m m u n o d e f i c i e n c y and c o m p l e x renal d y s f u n c t i o n , a new syndrome, are p r e s e n t e d .

D e p a r t m e n t of R a d i o l o g y , U n i v e r s i t ~ t s - K i n d e r klinik, S t e i n w i e s s t r . 75, C H - 8 0 3 2 ZUrich

J. Buhr By the example of WeiU-Marchesani-Syndrome which goes on with abnormalities in eyes (small spherical lens, ectopia lentis in half of cases, myopia with or without glaucoma, blindness in one third), in limbs (brachydactyly with broad metacarpals and phalanges, with or without late ossification of epiphyses), in growth (small stature), in crauiofacial dysmorphia (broad skull, small shallow orbits, mild maxillary hypoplasla with narrow palate) and in teeth (malformed and malaligned) the importance of a subtile assessment in native radiologlcal diagnosis is emphasized. Beside a lot of syndromes in case of which the diagnosis can be made by one look on the fdm there are not few which demand the physician to act - as the German philosopher Friedrich Nietzsche said . "with the subtlety of a policeman and advocate". Kinderabteilung des Kreiskrankenhauses Greiz, Irchwitzer Str. 18, DDR-6600 GrelZ

Abstracts. 27th Congress of the European Society of Paediatric Radiology




NEUROFIBROMATOSIS BONES J.C. H o e f f e l , B. C. S c h m i t t

G. Eich, T. Costa, MJ. Phillips and A. Daneman, (Departments of Radiology, Genetics and Pathology from the Hospital for Sick Children, Toronto, Canada.) This paper presents previously unreported clinical and radiographic observations in 3 neonates who had profoundly advanced bone age. The two M and one F were born at term. Polyhydramnlos had been present in 2. Family history, available biochemical and chromosomal studies were unremarkabIe. Maior Clinical F i n d ~ 1.Long Extremities with big hands and feet 2.CraniofaciaI abnormafities with large fontanelles, prominent eyes, dowaslanting palpebral fissures, hypertelorism, small nose, mleroretrognathia and dysplastie auricles. 3.Urogenital anomalies. 4.Hypotonla, feeding problems, developmental delay. Death occurred at 25 hours, 3 months and 3 years. Radio~aphlc Findings: Identical in all 3: the most striking features are at the craniocervicaljunction and the hands: 1.Marked ilattening of the occiput with downward projection of the occipital rim posterior to the foramen magnum and incongruityof the skull base and C1 with narrowing of the spinal canal. Small mandible with obtuse angle, wide sutures and fontanelles, hypertelorlsm. 2.The hands are large with a bone age of 2 to 3 years at birth and widened proximal and middle phalanges. 4.Sharp angles of vertebral bodies with mild anterior wedging and decreased AP diameter. Anterior scalloping on follownp examination in one patient. Premature ossification of coccyx with three to four centers. 5.Steep ifiac angles, somewhat triangular chest, excessive curvature of clavicles, mild diaphyseal stenosis of the long bones. Marshal and Smith (1971) described a syndrome with similar clinical features and typical x-ray findings of the hand. In our three infants the presence of auricular findings and the distinctive skeletal radiographic features mainly of the crauiofacial and spinal region raise the question whether our patients represent a new syndrome, which would have to be considered a bone dysplasia.

44 MINOR TIBIAL DUPLICATION: ANOTHER CAUSE OF PRENATAL BOWING. REPORT OF FOUR CASES C. Adamsbaurn*, G. Kalifa*, R. Seringe**, J. C. Bonnet** Four infants presented with posterior and medial unilateral concavity of the tibia. The fibula was normal but a homolateral great toe duplication was seen as well as a hand malformation. Plain films and C.T. showed no evidence of tibia pseudarthrosis but disclosed a true tlbial duplication with distinct cortieals. We do think that this abnormality represents a true entity. The embryology and surgical treatment will be discussed. Other causes of such tibia bowing can be ruled out, such as congenital pseudarthrosis (with or without neurofibromatosis) or some skeletal dysplasias. The main problem of these children is a marked leg discrepancy. No visceral malformation has been noted in association. * Department of Radiology, ** Department of Orthopedics, H6p. Saint Vincent-de-Paul, 74 Avenue Denfert Rochereau, 75674 Paris Cedex 14 (France)






OF Galley

LONG and

A 18 years old girl was admitted because of painful swelling of t h e t i b i a s . T h e r e w a s no history of i n f e c t i o n nor of t r a u m a . X-Rays showed appositional new bone on the antero-lateral aspects of both tibial diaphyses at t h e level of the interosseous membrane and a l s o on t h e left f i b u l a . Radionuclide bone imaging showed a mild cortical uptake. Biopsy was negative. At physical examination there was some cafe-au-lait spots and molluscum on the thorax. Two y e a r s later a huge bilateral acoustic neuroma developped rapidly. Surgery was performed but the girl died post-operatively. We believe this hyperostosis o f long d u r a t i o n may be associated with neurofibromatosis. Up to now only subperiosteal hematoma has been described in association with neurofibromatosis.

Department of R a d i o l o g y ~ 5, A l l ~ e d u M o r v a n 5 4 5 1 1

Children's Vandoeuvre

Hospital C@dex


Abstracts. 27th Congress of the European Society of Paediatric Radiology

Poster Presentations





pathologic fractures in juvenile bone cysts (JBC) are related with spontaneous healing. Currently, the trea~nent of choice is the instillation of steroids. Retrospectively, all files of patients with cystic bone lesions were analysed resulting in 50 patients with JBC and doc~nented follow-up since 1980. Characteristic radiographic patterns for JBC were found in 79 %. Accurracy of radiography canpared to pathology was 85 %. Most patients had ccsNoined instillation of steroids, spongioplastic surgery or pathologic fractures. Overall success of trea~nent was 70 % with 20 % of relapses. The average n~nber of required steroid instillations was 4.2. The chosen irodalityof treahnent had no influence on the results nor did a pathological fracture or the n~nber of instillations improve the outcotne. The radiologic follow-up of typical cases is illustrated, criteria for relapse and definite healing are discussed. Because of the limited accurracy of radiography, the first instillation of steroids should be used to gain material for pathology.

G. Etch l, T. Costa i A. Giedion 2, A. Daneman 1 1 Departments of Radiology and Genetics from the Hospital for Sick Children, Toronto, Canada. 2 Department of Radiology, Childrens' Hospital, Zurich, Switzerland. This paper presents a female neonate with a short limb and short trunk dwarfism that had been diagnosed on prenatal ultrasound in the third trimester. The family hlstory was unremarkable. Apart from short stature no other significant clinical finding was noted. Radio~;raphlc findings: 1. The extremities showed shortening of all three segments with irregularities and slight flaring of the metaphyses and premature osslficatlon of several epiphyses of the proximal phalanges of the hands. The fibulae were long in comparison to the tlbae. 2. The most striking radiographic finding was that of abscence of ossification of several cervical vertebral bodies and lower sacral segments. The remainder of the vertebral bodies were oval shaped and the interpedicular distance was diminished in the lumbo-sacral area. 3. The pelvis showed a typical trident appearance of the acetabula with flaring of the lilac wings. The ischial bones were comma shaped and the pubic bones were delayed in ossification. 4. The head and the clavicles were normal. The rib ends were slightly irregular and flared. The combination of the above mentioned radiographic findings has not been described before. The differential diagnosis includes the achondrogenesis hypochondrogenesis - spondyloepiphyseal dysplasia congenita - spectrum. None of these entities, however, have all the features of our patient, suggesting that the present case could represent a new skeletal dysplasia.

Radiolog. Institut und Orthop~dische Klinik, Olgahospital - P6diatrisches Zentr~, Bismarckstr. 8, D-7000 Stuttgart ]

The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, M5G 1X8





R. Dominguez, and L. Robinson A case report is presented o f a boy with l o c a l i z e d congenital hypertrophy of his lower extremity. Conventional radiographs revealed o s t e o i y t i c lesions of the long bones in the affected extremity as an expression of the rare lymphangiematosis o f bone. An H~r multiplannar supplemental study revealed the extent, nature and l i m i t s of the subcutaneous lymphangiomatosis and was very helpful in planning the surgical resection. Department of Radiology, U n i v e r s i t y of Texas Medical School #2,132, 6431Fannin, Houston, Texas 77030


The purpose of this study was to determine whether either a low or high calcium diet maintained throughout the growing period, would affect peak vertebral bone density (BD). The study was carried out using the New Zealand white rabbit as an animal model. Twenty.four rabbits had BD measured by QCT at 6 week intervals until their growth plates closed. Eight received a standard calcium diet for rabbits (0.45 gm calcium per 100 gm of chow); eight were fed a low calcium diet (0.15%), and eight a high calcium diet (1.35%). There was a statistically significant difference among the three groups: F(2,101) = 25.94 (p< .0001). Multiple comparison tests showed that animals on a medium or high calcium diet did not differ in BD from each other but were associated with larger mean BD values than the animals on a low calcium diet (p< .05). Throughout the study, there were no significant differences in weight, length, or age at time of epiphyseal closure between the rabbits fed low, medium, or high calcium diets. Our results do not support the hypothesis that increased calcium intake during growth affects peak bone mass. A high calcium diet did not increase vertebral BD o r peak BD over that of a standard calcium diet in our study. Moreover, moderately severe calcium restriction had a relatively small affect on vertebral BD. A diet threefold lower in calcium than a standard diet resulted in a 4% lower peak vertebral BD. Departments of Radiology and Pediatrics, Childrens Hospital Los Angeles, USC School of Medicine, Los Angeles, CA 90054-0700

Abstracts. 27th Congress of the European Society of Paediatric Radiology




1-123-/I-131-MIBG IN DIAGNOSIS AND FOLLOW-UP OF NEUROBLASTOMA A. Hach, H. Bihl, P. Gutjahr, B. Kimmig, g. Hahn


Neuroblastoma is the most common extracranial solid tumor of childhood, in most cases showing specific enrichment of meta-iodo-benzylgnanidine (MIBG). The aim of our study was to determine the reliability of HIBG in primary tumor diagnosis and evaluetion of metastatic and recurrent disease. From 1982 to now, we performed 137 MIBG-sciatigraphies in 97 children, either with iodine-123 (n=71) or iodine-131 (n=66). Indications for scintigraphy were the differential diagnosis of abdominal masses (n=ll), staging of neuroblastomas before and after surgery (n=68), monitoring disease status after chemotherapy (n=51), or the evaluation of clinical symptoms (n=7). 79 patients had neuroblastomas, 56 of them suffering from stage III or IV disease. Specificity in primary tumor diagnosis was 98 %, sensitivity = 9 1 % . For bone marrow involvement and recurrent disease, sensitivity was 75 % each. It decreases in cases of very large tumors or weak bone marrow involvement. In 30 Cases, an additional bone scintigraphy was performed. 22 children had bone lesions with corresponding findings of both examinations in 20 cases. MIBG scintigraphies and bone marrow biopsies after chemotherapy showed correlating results. 43 MIBG scans were true negative, ii scans showed false negative results, 6 of them being performed under chemotherapy. We conclude tbat MIBG scintigraphy is a sensitive and highly specific method in routine monitoring of nenroblastoma. Abt. fur Nuklearmedizin der Universit~tskliniken Langenbeckstr. i, D-6500 Mainz;

V. Jovanovic, Z. Vuskasinovic, B. Tanaskovic One hundred cases of Ewlng sarcoma was classified in "Banjica" register of bone tumors. Among them, 11 were located on several parts of vertebral column. There were six males and five females. Mean age was 13.9 years (ranged from 7 to 22). Some of them were misdiagnosed at the beginning of the treatment and, consequently, they were treated as having osteomyelitis of vertebrae, tuberculosis of the spine, juvenile discus etc. This caused prolonged period between initial complaints and proper treatment (7.3 months) compared with average period for this group of tumors. Authors present initial radiograms which caused mistakes in diagnosis. Special orthopaedic hospital "Banjica", Takovska 16, YU-11000 Belgrade


Abt. fur Nuklearmedizin der Universit~tskliniken Heidelberg, Neuklinikum, INF 400, D-6900 Heidelberg



USE OF DYNAMIC HIP SONOGRAPHY IN THE TREATMENT OF CONGENITAL HIP DISLOCATION H.T. Harcke, L.E. Grissom, M.E. Boulden, and J.R. Bowen The Pavlik harness and similar devices are used to treat infants with congenital hip abnormality. Radiographs are of limited use because they can fail to detect posterior dislocations that occur when the hips are positioned in flexion and abduction.


The dynamic, real-time method of sonography permits the hip to be evaluated, not only at rest, but while the hip is moved thru the range allowed by the harness. Position and stability is assessed in transverse and coronal planes. The technique has been used in over 500 examinations performed on infants wearing a Pavlik harness. Sonography is useful for checking position. Clinically undetected posterior dislocations have been identified. The harness can be adjusted with the assistance of ultrasound in order to obtain the most beneficial position. With dislocations detected within four weeks of birth, placement in the harness can result in gradual reduction over time. Serial sonograms are critical in deciding if the dislocated hips are reducing and if treatment should be discontinued. Department of Medical Imaging, Alfred stitute, Wilmington, Delaware 19899 USA











Kozlowski K.

Three cases of unclassified bone dysplasia with distinctive radiographic features are presented. CASE I. Boy 5 year-old with sacral hypoplasia, bifid distal femora and mild generalised bone changes. CASE II. Girl 3 year-old with mesomelic shortening of the upper extremities, unusual deformity of the femora and minor generalised bone changes. CASE Ill. Girl 17 year-old with acroosteolysis, lar hypeplasia and cervical ribs.


No similar cases have been reported to the best of our knowledge in the literature.

Royal Alexandra Hospital for Children, Sydney, Australia.


Abstracts. 2 7 t h C o n g r e s s o f the E u r o p e a n Society o f P a e d i a t r i c R a d i o l o g y



ACROOSTEOLYSIS SECONDARY TO BENIGN PHEOCHRO~OCYTOMA F. Mar~on, J.C. H o e f f e l , A.M. Worms, M.A. G a l l e y and M. S o h m i t t


A boy ii years of age suffer from h y p e r t e n s i o n and acrocyanosis with puffy and cyanotic hands. The distal metaphyses of several phalanges look irregular and tapered. At surgery bilateral benign adrenal pheochromocytoma is removed. Hypertension r e g r e s s e d and follow up of the b o n e e of the hands shows disappearance of the a o r o o s t e o l y s i s of the p h a l a n g e s . Metaphyseal bone changes associated with benign pheochromocytoma are very rare. U s u a l l y the knees, ankles, e l b o w s and w r i s t s are involved. 0steolysis of the phalanges h a v e net b e e n r e p o r t e d up to now. The bone lesions can be explained by microcirculatory changes. Adrenaline cause hypovolemia and hemoconcentration with t e n d e n c y toward h e m o c o n o e n t r a t i o n r e s p o n s i b l e for aggregation of red blood cells and f o r m a t i o n of m i c r o t h r o m b i r e s u l t i n g in stasis. Since the vascularity is growing bone, bone changes r a p i d l y in the child.

Rohden, L. yon, Krebs, P, & Steinbieker, V. Klinik fiir Kinderheilkunde, Medizinische Akademle, DDR-3014 Magdeburg, E.-Larischweg 17 Clinical myology and genetics are often facing the problem of early detecting carriers of x-linked recessive muscular dystrophies. The differential diagnosis of patients with neuromuscular diseases (ND) is an unsolved problem hi many cases, but a precise diagnosis is required for every genetic counselling. To solve these problems myosonography as introduced by Dr. Heckmatt was used. A total number of 874 persons was investigated: healthy controls 218, patients with known or suspected ND 351, screening of carriers 305. Myosonography was able to detect abnormalities before the onset of dinieal symptoms. The following muscular diseases can be differentiated by ultrasound: Duchenne, Becker and 13mery-Dreifuss-dystrophy, Facioscapulohumeral and Limb Girdle dystrophy, Congenital and Fukuyama dystrophy as well as Welander and Myotonic dystrophy, Hnallyspinal atrophies. In screening of carriers myosonography had a sensitivity of 92%, a specificity of 93%, and a positive predictive value of 92,5%. In neuropathles, however, this methode seems not to be promising. Myosouography is recommended as easiest way to detect carriers and to differentiate dystrophies. Additionally, patients at risk for malignant hyperthermia can be identified preoperatively by ultrasound.

greater in the take p l a c e more

D e p a r t m e n t of C a r d i o l o g y , C h i l d r e n ' s Hospital 5, A l l ~ e du M o r v a n 54511 V A N D O E U V R E C ~ d e x



S O N O G R A P H I C D I A G N O S I S OF O S G O O D - S C H L A T T E R DISEASE: A C O M P A R A T I V E S T U D Y B E T W E E N S O N O G R A P H I C A N D C O N V E N T I O N A L X - R A Y TECHNIQUES. M. M e r a d j i , S.G.F. R o b b e n


High r e s o l u t i o n s o n o g r a p h y was p e r f o r m e d in 30 c h i l d r e n (21 boys and 9 girls) w i t h O s g o o d S c h l a t t e r disease. In 27 cases the knee was a l t e r e d u n i l a t e r a l l y and in the r e m a i n i n g 3 cases b i l a t e r a l l y (33 knees). C o m p a r a t i v e x - r a y films w e r e t a k e n r o u t i n e l y in all p a t i e n t s of this group. Soft t i s s u e s w e l l i n g in front of the t u b e r o s i t y as a c h a r a c t e r i s t i c feature of a l t e r e d knee was seen and m e a s u r e d in all 33 k n e e s (30 cases), w h i l e r a d i o l o g i c a l l y the s w e l l i n g was v i s i b l e in 31 knees. A v u l s i o n of tibial t u b e r o s i t y , as m a y be seen in the second stage of this d i s e a s e , was s o n o g r a p h i c a l l y and r a d i o l c g i c a l l y r e c o g n i z e d in 17 of 33 a l t e r e d knees. In a d d i t i o n s o n o g r a p h y showed a v u l s i o n in one knee, that was m i s s e d b y r a d i o g r a p h y . In two o t h e r cases (two knees) the f r a g m e n t a t i o n was c l a e r l y seen on u l t r a s o u n d , but not on the r a d i o g r a p h s . F r a g m e n t a t i o n of t u b e r o s i t y u s u a l l y a p p e a r s late stages of O s g o o d - S c h l a t t e r disease. This sign was s e e n b o t h s o n o g r a p h i c a l l y and r a d i o g r a p h i r a l l y in 21 of 33 knees. In two knees s o n o g r a p h y d e t e c t e d f r a g m e n t a t i o n , m i s s e d by x-rays. The r e s u l t s of this c o m p a r a t i v e study show the s u p e r i o r i t y of s o n o g r a p h y in the d i a g n o s i s of Osgood--Schlatter d i s e a s e e s p e c i a l l y in e a r l y stages, not o n l y for the r e c o g n i t i o n of soft t i s s u e s w e l l i n g but also to d e t e c t a v u l s i o n of the t i b i a l t u b e r o s i t y and c h a n g e s of the o s s i f i c a t i o n centre and can be u s e d f o r the e v a l u a t i o n of O s g o o d - S c h l a t t e r disease. S o p h i a C h i l d r e n ' s Hospital, Dep. of Paed. Ead., G o r d e l w e g 160,3038 GE R o t t e r d a m r T h e N e t h e r l a n d s .

Two patients showing campomelic syndrome are compared with four others showing but partial symptoms of the syndrome, thus allowing no definite classification. The partial symptoms found in the latter patients are considered specific to the campomelic syndrome. The authors discuss the question whether the letter cases, which lack the most striking feature of the syndrome (to which the latter owes its name) - the bowing of the tibiae -, might be classified as an oligosymptomatic form of the syndrome. Kinderklinik und Abteilung for Klinische Genetik der Hedizinischen Akademie Dresden, Fetscherstr. 74, ODR-8019 Dresden

Abstracts. 27th Congress of the European Society of Paediatric Radiology




SIBS WITH A DISORDER RESEMBLING BLOUNT DISEASE H. Schmidt, S. Mundlos, R. Schumacher, J.W. Spranger

GORHAM'S DISEASE IN CHILDHOOD (A case report and review o f the l i t t e r a t u r e )

Blount disease (tibia vara, osteochondrosis deformans tibiae) is a local disturbance of growth of the medial aspect of the proximal end of tibia, affecting the metaphysis, epiphyseal cartilage and epiphysis.Histological changes are principally localized to epiphyseal cartilage. No findings suggest the occurrence of avascular necrosis of bone.The irregularity of the metaphysis and medial part of epiphysis is a consequence of irregular advance of ossification.The entity was classified into the infantile and adolescent type (Blount, 1937).The radiographic abnormalities of the infantile type can be divided into 6 stages depending on the maturity of the skeleton and the degree of growth disturbance (Langenski61d, 1952, 1964). 3 Turkish sibs presented at the age of 7 (~), 26 (0~ and 45 (~) months with bowing of legs.An elder brother and further members of the family were healthy.Their parents are first cousins. Diet had always been adequate and the bowing was not preceded by any localized or systemic illness.Radiographic investigation showed physiologic bowing of tibia in the 7 month old girl, Stage I Blount disease in the 26 month old boy and Stage II Blount disease in the 45 month old girl. The reported cases provide evidence for autosomal recessive inheritance. Blount disease has been reported to be an autosomal dominant (McKusick, 18870) and recessive (McKusick, 25920) disorder.This observation points to genetic heterogenity, i.e. the existence of two genetically different, but radiologically indistinguishable disorders.Germinal mosaicism is less likely, multifactorial inheritance has been suggested by Bathfield and Beighton, 1978.

P.THIESSE P.KAEMMERLEN E,BOUFFET C.BAILLY M.FAVROT P.JONAS T.PHILIP M.BRUNAT-MENTIGNY Lymphangiomatosis o f bone is an uncommon disease. I t appears as a massive p r i m a r i l y avascular o s t e o l y s i s a f f e c t i n g both the c o r t e x and spongiosa r a p i d l y progressive , extending t o the adjacent bones w i t h no respect f o r J o i n t boundaries. A painless s o f t s w e l l i n g is u s u a l l y present and the v i t a l prognosis depends on the chest involvement. We r e p o r t one case o f an 11 y e a r - o l d g i r l w i t h p l e u r a l involvement which lead t o death w i t h i n 4 months a f t e r the appearence o f a cutaneous lymphangioma. The d i f f i c u l t i e s o f the diagnosis on h i s t o p a t h o l o g i c a l samples and the v a r i a b i l i t y o f the c l i n i c a l progression give a preeminent place t o the r a d i o l o g i c a l i d e n t i f i c a t i o n o f these disease. The r a d i o l o g i c a l appearence is the key t o the separation of t h i s e n t i t y from c y s t i c lymphangioma or o r d i n a r y hemangioma o f bones,

CENTRE LEON BERARD D i r e c t e u r Pr.T.PHILIP 28, Rue La~nnec,F 69373 LYON FRANCE

Dr.Helga Schmidt, Kinderradiologie der UniversitfitsKinderklinik, Langenbeckstrage, D-6500 Mainz





Few authors report a coincidence of minor congenital anomalies and embryonic tumors of infancy. Wellknown are those combined with chromosomal anomalies (hemihypertrophy, aniridia, 13q-syndrome, trisomy-21...). The incidence of rib anomalies in children without malignancies, congenital heart disease or spinal dysraphism was 5.5% (200 children). We reviewed thorax X-rays of 1000 children with malignancies for any kind of rib anomaly. We found 242 rib anomalies in 218 patients (21,8%); a significant (p < O.O01) accumulation considering an incidence of 5.5% rib anomalies in a "healthy" population. All kinds of malignancies showed a high incidence of cervical Fibs: neuroblasioma 35,2%, cerebral tumors 28,4%, Wilms' tumor 26,5% and leukemia 25,7%. The increased incidence of these mesenchymal "defects" in malignancies of infancy and childhood may be another clue for an alterated morphogenesis in tumor origination. On the other hand in nenroblastoma the rib anomaly may be another expression of neurocristopathy as proposed for the association of congenital heart disease and neuroblastoma*. *Bellah,R, Andrea,AD, Darillis,E,Fellows,KE (1989) The association of congenital neuroblastoma and congenital heart disease. Pediatr.Radiol.19:ll9-121 Universit~ts-Kinderklinik, D-6500 Mainz

Langenbecksirasse i,

K. Urbanek, K. Bitvai, R. Kecsk6s, Z. Harkfinyi, Prof. L. SzI~ivy Conventional radiography and MRI were used in twenty children (age range 4 15 years) to determine the cause of joint pain. 13 boys, 7 girls were examined. MRI studies were performed with a 1,5-T system (Siemens, Magnetom). SE, T1 and T2 weighted images were obtained through each lesions in a variety of planes. In these cases conventional X-Ray revealed intracapsular or extracapsular focal lesions. MR imaging proved to be accurate in estimating the size and localisation of the lesions, soft tissue and bone marrow involvement. Different types of lesions were detected (osteoid osteoma, chronic osteomyelitis, enehondroma etc.). MRI provided helpful information in planning the surgical intervention. Department of Diagnostic Radiology, National Institute for Vascular Surgery Department of Pediatric Radiology and Department of Orthopedic Surgery, Budapest, Hungary


Abstracts. 27th Congress of the European Societyof Paediatric Radiology

62 CEPHALO-SKELETAL DYSPLASIA (Taybi-Linder syndrome): report of a new case with unusual natural history. G.F. Vichi, P.L. Duvina, F. Bettini, T. Bougleux, N.L. Giovannucci Uzielli Cephalo-skeletal dysplasia (CSD) is a very rare syndrome, characterized by g e n e r a l i z e d ~ severe enchondral, d e l a y e d growth d e v e l o p m e n t , and brain dysplasia. The clinical spectrum includes: low weight birth dwarfism, microcephalia, unusual facies with bulging eyes, flat nasal bridge, high-arched and narrow palate, spade-liKe hands and feet, mental retardation. The p r o g n o s i s for life is fatal in early infancy. Radiological patterns [nclude severe microcrania, small anterior fontanel, i n c o m p l e t e l y closed s u t u r e s , more or less expressed platyspondyly, variable shortness of long bones, c u p - s h a p e d ends of short tubular bones o f hands, f e e t and r i b s , squared i l i a c bones~ narrow i s c h i a t i c notches, horizontal acetabular roof (quasi-zero acetabular angle), f l a t t e n e d ilia, incomplete or absent o s s i f i c a t i o n of pubic bones, slight widening and irregularity of metaphyses of long bones. We d e s c r i b e a new p a t i e n t , s t i l l alive at 12 m o n t h s age, we studied from several points of view (clinical, radiological, cytogenetic and genetic), showing all the m a j o r a s p e c t s of CSD syndrome. We emphasize the complex skeletal a n o m a l i e s and the severe brain dysplasia r e v e a l e d by HRI, c o n s i s t i n g in c o r p u s c a l l o s u m and frontal lobes agenesia, temporal lobes d y s g e n e s i a , lateral ventricles d i l a t a t i o n . The imaging of other organs excluded major abnomalities. Karyotype was normal in p r o p o s i t u s and in parents. Ospedale Pediatrico "A. Heyer" - i3 Via Luca Giordano - 50132 Firenze (Italy)

63 DIFFERENCES OF RADIOLOGICAL MANIFESTATIONS OF EWING SARCOMA. D U E TO TOPOGRAPHICAL DISTRIBUTION Z. Vukasinovlc, V. Jovanovic, B. Tanaskovic In "Banjica" register of bone tumors there were 100 cases of histologically verificated Ewing sarcoma. In analysis of these cases authors found great differences in radiologlcal signs of tumors of long tubular bones comparing them with the tumors of the other localization (11% on vertebrae, 8% on sacroiliac joint etc.). Authors present classical radiogram, arteriography, scintigraphy and computerized tomography of some illustrative cases. Special orthopaedic hospital "Banjica", Ivana Milutinovica 9/14, YU-11000 Belgrade

Abstracts. 27th Congress of the European Society of Paediatric Radiology


Upper Airways and Chest Oral Presentations 64


CT EVAf~UATION OF NASAL A B N O R ~ L I T I E S IN C~IILDREN C.J. Becker, R.B. Towbin and T.L. Slovis The purpose of this paper is to examine a spectrum of nasal pathology and assess its evaluation by CT.


51 cases of nasal abnormality were reviewed, consisting of 25 females and 27 males. The age group was i day to 17.5 years, with a mean of 5 years. Sedation was utilized frequently in obtaining the scans. 16 children had congenital structural abnormalities, including 6 choanal atresias (I multiple craniosynostosis), 9 ehoanal stenoses (including 2 Pfeiffer's syndrome, i Crouzon's, and I Apert's), and 1 cleft palate with nasal involvement. Benign masses were found in 23 with 4 dermolds, 5 polyps (i with cystic fibrosis end polyposis), i papilloma, 2 lipmoas, 4 eneephaloceles, 1 nasal glioma, I keloid, I foreign body granuloma, i foreign body, 1 benign fibrous osseous lesion, i lymphangioma, and 1 ease of hypertrophied adenoids. Three lacrimal duct abnormalities were seen as well as 6 patients with vascular abnormalities (2 hemangiomas and i hemangioendothelioma, and 3 angiofibromas). Fibrous dysplasia was present in i case. There were 3 malignant masses including 1 case each of carcinoma, chlorma, and transitional cell carcinoma. CT was able to delineate the extent of the abnormality and could frequently suggest the diagnosis or limit differential considerations. CT is an excellent means of accurately imaging a wide variety of nasal abnormalities in children.

Children's Hospital of Michigan, Dept. of Radiology Wayne State University, 3901 Beaubien Boulevard, Detroit, Michigan 48201 U.S.A.

23 premature neonates with severe r e s p i r a t o r y di st r es s were studied following the appl i cat i on of 200 mg/kg/b.w. "Curosurf" into the bronchial system according to the rules of the C o l l a b o r a t i v e European Multicenter Study Group. Curosurf is a natural porcine preparation of phospholipids and 1% apoproteins. The c l i n i c a l data derived from the study, f i ni shed in 1988, demonstrate within minutes signs of improvement of the status and biochemical parameters of the newborns. The radiographic changes needed four (in 4 patients) to 24 hours to improve from grade 3 to 4 of the hyaline membrane disease pattern, in 12 patients to grade 1 and in 11 patients to grade 2. In one p a t i e n t grade 3 persisted t i l l the 6th day, he needed ductus l i g a t i o n . Only one patient had i n t e r s t i t i a l emphysema and another a small pneumothorax without drainage, 6 patients needed up to 4 doses of surfactant. More than 24 hours a f t e r treatment 7 newborns had grade 2 HMD pattern, 4 of them needed a ductus l i gation. No adverse e f f e c t s of the treatment were observed. None of the treated patients died of RDS problems during the f i r s t week. While 25 % of the matched controls from the year before died of r e s p i r a t o r y i n s u f f i c i e n c y . Cont r o l s had a longer period t i l l t h e i r lungs cleared, 4 had tension pneumothorax, nine needed pneumothorax drainage, one resolved without a s p i r a t i o n . 4 controls develloped lobar a t e l e c t a s i s . No d i f f e r e n c e between the treated and the control group in complication rates as i n t r a c r a n i a l hemorrhage, r e t i n o p a t h i a , bronchopulmonary dysplasia and frequency of s i g n i f i c a n t ductus arteriosus apertus. Surfactant treatment is an evolution even in p e d i a t r i c radiography. Zentrum Kinderheilkunde der U n i v e r s i t ~ t , Robert-KochStrafe 40, 3400 GSttingen





Normal patterns of laryngeal sonography (LS) in children have already been presented during the last ESPR meeting in Dublin (Reference 64, to be published in Pediatric Radiology). We now report the findings in 22 children with endoscopically and/or surgically proved laryngeal pathology who underwent LS. This is not a double-blind study. Our series included 4 vocal cords palsies, 8 acquired subgtottic stenosis, 7 spaceoccupying lesions (cystic 134nphangioma (1), sub-glottic hemangiomas {4), heterotopic salivary tissue (1), neurofibromatosis - related mass (1)), 2 congenital sub-glottic stenosis related to an anatomic abnormal i t y of the anterior arch of the cricoid and 1 laryngeal diastema. The sonographic and endoscopic findings have been well correlated in 19 cases (86 %). The missed cases included : a laryngeal diastema in which the posterior cricoid silt could not be imaged because i t was hidden by the acoustic shadow of the subgiottic air, one out of the 4 subglottic hemangiomas because of a suboptimal study (restless child) and one out of the 4 laryngeal palsies, which was probably ascribed to the fact that the infant had been examined during deep sleep, that led to too small glottic movements. We conclude that L.S yields an overall good accuracy as a coalplementary way of investigating laryngeal pathology during childhood. LS is quite useful for studying dynamic abnormalities (provided that glottic movements can be obtained), in the work-up of space-occupying lesions and acquired and congenital laryngeal stenosis. At times, some kind of sedation may be necessary. L.S wil] not be contributive in postero-midline defects (laryngea] diastemas).

During a 20 months period 5 full-term newborns were observed, who had the classical criteria of ARDS. Four of them presented with pre- or intrapartum asphyxia; two had proven group-B streptococcal sepsis. All of them suffered from severe shock and there was obvious pulmonary hypertension. In order to achieve adequate gasexchange all five newborns were ventilated hy high frequency. Despite a high mean air-way pressure typical radiological findings of "white" lungs developed; in four patients extraalveolar air as well due to acute barotrauma was present. Only two patients survived, requiring 17 and 46 days of artificial ventilation. In three newborns, who died due to septic shock and hypoxemic hypoxia after 17 to 173 hours autopsy demonstrated the classical histological elements of ARDS.

From Department oF Radiology (*) and otolaryngology { * * ) . C. GareI, Department of Radiology, Bopital ROBERT DEBRE, 48 Boulevard Serurier 75019 PARIS. FRANCE.

R~ntgenabteilung Universit~tskinderkliniken, Inselspital CH-30 I0 Bern


Abstracts. 27th Congress of the European Society of Paediatric Radiology




D I A G N O S I S OF C H R O N I C I N T E R S T I T I A L LUNG D I S E A S E BY C O M B I N E D CT S C A N N I N G AND P E R C U T A N E O U S LUNG BIOPSY. H. Carty. D P Heaf, and D. van V e l z e n C h r o n z c i n t e r s t z t i a l lung d i s e a s e is u n c o m m o n in c h i l d h o o d and p r e s e n t s d i a g n o s t i c difficulties. C l i n i c a l and c h e s t xray a b n o r m a l i t i e s are n o n - s p e c i f i c and a e t i o l o g i c a l d i a g n o s i s is d e p e n d e n t on open lung b i o p s y that is i n v a s i v e and u s u a l l y r e s e r v e d for severe p r o g r e s s i v e disease. B e t w e e n Oct 1988 and Nov 1988~ 6 infants~ m e a n age i0 mths (Range i to 36 mths) presented with chronic respiratory distress, f a i l u r e to thrive and b a s a l crackles. Chest Xrays showed d i f f u s e i n f i l t r a t e s , CT s c a n n i n g of the chest showed e x t e n s i v e p u l m o n a r y i n f i l t r a t e s w i t h cysts (3/6). P e r c u t a n e o u s lung b i o p s y was p e r f o r m e d u n d e r g e n e r a l a n a e s t h e t i c u s i n g e i t h e r an a s p i r a t i o n n e e d l e or R a d i p l a s t b i o p t y gun. Complications incl0ded a small pneumothorax (4/6) and p u l m o n a r y h a e m o r r h a g e (i/6). None r e q u i r i n g treatment or c a u s i n g c l i n i c a l deterioration. Satisfactory biopsy specimens were obtained in all patients. Diagnoses included Fibrosing A l v e o l i t i s (4/6), D e s q u a m a t i v e I n t e r s t i t i a l P n e u m o n i a (1/6), C h r o n i c I n t e r s t i t i a l P n e u m o n i t i s (1/6). C o m b i n e d CT scanning and p e r c u t a n e o u s lung b i o p s y is less i n v a s i v e than open lung biopsy and allows early a e t i o l o g i c a l d i a g n o s i s .

L. Rausin*, J. Khamis*, M. Spehi**, JM Bertrand*, C. Piette*, E Bauduin* The adult respiratory distress syndrome is relatively rare in children. This morbid entity needs an agresslve supportive treatment and still remains a terrible challenge for the intensive care team. The authors reviewed 8 eases (6 recoveries, 2 deceased) and try to def'me the part of the X-Ray examination in the dlagnos{s and the follow up of such a syndrome. They compared the radiologle and clinica~ datas with those of 2 cases of pure pulmonary edema that oecured under ambiguous drcumstances: 1. 2. 3.

X-Ray fledings are not specific. The delay between the critical event and the onset of the X-Ray smptoms is important for the diagnosis. X-Ray examination allows the detection of the complications.

* Clinique de l'Esp6rance, 84320 St. Nicolas/Li6ge ** H6pltal Unlversitalre St. Pierre, B-1000 Brussels

A l d e r Hey C h i l d r e n ' s






9 9 m - T C - D T P A A E R O S O L L U N G C L E A R A N C E IN T H E E A R L Y D E T E C T I O N O F INTERSTITIAL P N E U M O N I T I S IN B O N E M A R R O W T R A N S P L A N T PATIENTS. H. Van der Wall, I.P.C. Murray, S. Russell and M. Vowels.

Twenty-six survivors of Stage IV BPD treated in our Intensive Gare Nursery between 1964 and 1973 were studied at ages 14-25 for late outcome and sequelae. Control groups consisted of 26 age-matched ex-prematures without lung disease and 53 healthy age-matched nonsmoking volunteers. All subjects were assessed by history, physical examination, chest radiography, EKG, doppler echocardiography, blood gas studies, and pulmonary function studies. Chest radiographs were scored normal (0), mild (i), or moderate/severe (2) for eight variables in a blinded fashion. Eight (31%) ex-BPD subjects had a total score of 4 or greater while no control subject had a score that high (P

27th Congress of the European Society of Paediatric Radiology. Munich, May 16-19, 1990. Abstracts.

Pediatr Radiol (1990) 20:377-424 Pediatric Radiology 9 Springer-Verlag 1990 27th Congress of the European Society of Paediatric Radiology Munich, Ma...
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