Pediatric Radiology
Pediatr. Radiol. 8, 3941 (1979)
9 bySpringer-Verlag1979
Case Reports Intracraniai Calcification in a Neonate with the Sturge Weber Syndrome and Additional Problems A. Alonso, D. Taboada, L. Ceres, J. Beltran, R. Olague, and A. Nogues Servico de Radiodiagnostico Pediatrico, Clinica Infantil "La Fe", Valencia, Spain
A b s t r a c t . The neonate in this report had severe encephalotrigeminal angiomatosis with intracranial calcification, cranial hemiatrophy, microcephaly and generalised severe cerebral atrophy. Such findings are not c o m m o n in the newborn with this syndrome. K e y w o r d s : Intracranial calcification, Sturge-Weber's
syndrome
The finding at birth of intracranial calcification in Sturge W e b e r ' s Syndrome is very uncommon. In the literature we have found only two previous reports [3, 4].
Fig. 1. Radiography of skull, first day of life. Fine curvilineal calcification in the right frontoparietal region (arrows)
Fig. 2. a and b. 50 days old. Increase in density and size of the calcification, right
cranial hemiatrophy. Diploic swelling (arrows)
0301-0449/79/0008/0039/$01.00
40
A. Alonso et al.: Intracranial Calcification in a Neonate with the Sturge Weber Syndrome
Fig. 3. Pneumoencephalography at the age of 3 months. Intense atrophy in the right hemicerebrum with displacement of the middle line structures Fig. 4. Right carotid angiography (3 months). Hypertrophy of the external carotid principally in the middle meningeal artery. Increased bilateral diploic swelling
Fig. 5. Right carotid angiography. Venous phase. Few cortical veins, with irregular filling of the longitudinal sinus
When 50 days old the infant began to have severe generalised convulsions and in the interim intracranial calcification had become more intense (Fig. 2 a and b); the calcification was also more extensive and then characteristic of the condition. The diploic widening had increased and was bilateral. Pneumoencephalography at the age of 3 months showed intense atrophy in the right hemicerebrum (Fig. 3). At angiography a large right middle meningeal artery was shown to divide into many small vessels having a telangiectatic appearance. The middle cerebral artery was elevated leaving an initially avascular space to be filled later by angiomatous vessels derived from the middle meningeal artery. During the venous phase only a few cortical veins, filling in an irregular way, emptied into the sagittal sinus (Fig. 5).
Discussion Case Report This one day old girl, weighing 3.4 Kilo, was born after a 39 week pregnancy in a 37 year old healthy mother. The father, aged 32 years, was also healthy and the parents had three healthy children, but there had been one miscarriage. There was no family history of any neuroectodermal dysplasia. At birth the infant had a flat angioma extending over the right side of the face to the mid-parietal region. Skull radiographs on the first day of life showed microcephaly (Cronquist index = 50 and a total sum of the cranial diameters of 279). There was a right parieto-temporal diploie widening of 4 mm thickness. There was also fine cerebral calcification in the right fronto-parietal region (Fig. 1).
In Sturge-Weber's Syndrome typical calcification d o e s n o t a p p e a r o r d i n a r i l y b e f o r e 2 y e a r s of a g e [4]. R a r e l y has c a l c i f i c a t i o n b e e n d e s c r i b e d in t h e v e r y y o u n g i n f a n t [3, 5]. T h e f i n d i n g s in t h e skull r a d i o g r a p h s , p n e u m o e n c e p h a l o g r a m , a n d at a n g i o g r a p h y a r e t h o s e c o m m o n l y f o u n d in t h e s y n d r o m e [2, 1, 6], b u t at a l a t e r age. C a l c i f i c a t i o n at b i r t h , t h e e a r l y o n s e t o f c o n v u l sions a n d c e r e b r a l a t r o p h y all u n d e r l i n e t h e g r a v i t y o f t h e p r o g n o s i s in this y o u n g infant.
A. Alonso et al.: Intracranial Calcification in a Neonate with the Sturge Weber Syndrome
Acknowledgement. We wish to express our appreciation to the neonatology service and the neuropediatrics service of the Clinica Infantil for their co-operation.
References 1. Bentson, J. R., Wilson, G. H., Newton, T. H.: Cerebral venous drainage pattern of the Sturge-Weber syndrome. Radiology 101, III (1971) 2. Di Chiro, G., Lindgren, E.: Radiographic findings in 14 cases of Sturge-Weber syndrome. Acta Radiol. (Stockh.) 35, 387 (1951) 3. Fanconi, G.: Sturge-Weber Syndrome beim Neugeborenen: Ausgedehnte naevi telangiectaci, partieller Riesenwuchs und intrakranielle Verkalkung der rechten Hemisphfire, die im Lauf der ersten 1 ~/2Jahre eher zur/ickgehen. Helv. Paediatr. Acta 17, 486 (1962)
41
4. Harwood Nash, D. C.: Neuroradiology in infants and children, Vol. I. St. Louis: C. V. Mosby. Co 1976 5. Nellhaus, G., Haberland, C., Hill, B.J.: Sturge-Weber disease with bilateral intracranial calcifications at birth and unusual pathologic findings. Acta Neurol. Scand. 43, 314 (1967) 6. Poser, C. M , Taveras, J. M.: Cerebral angiography in Encephalotrigeminal Angiomatosis. Radiology 68, 327 (1957) 7. Swischuk, E.L.: Radiology of the newborn and young infant. Baltimore: Williams and Wilkins 1973 Date of final acceptance: March 3, 1978
A. Alonso, M. D. Servicio de Radiodiagnostico Pediatrico Clinica Infantil "La Fe" Valencia Spain
Pediatr. Radiol. 8, 3941 (1979)
9 bySpringer-Verlag1979
Case Reports Intracraniai Calcification in a Neonate with the Sturge Weber Syndrome and Additional Problems A. Alonso, D. Taboada, L. Ceres, J. Beltran, R. Olague, and A. Nogues Servico de Radiodiagnostico Pediatrico, Clinica Infantil "La Fe", Valencia, Spain
A b s t r a c t . The neonate in this report had severe encephalotrigeminal angiomatosis with intracranial calcification, cranial hemiatrophy, microcephaly and generalised severe cerebral atrophy. Such findings are not c o m m o n in the newborn with this syndrome. K e y w o r d s : Intracranial calcification, Sturge-Weber's
syndrome
The finding at birth of intracranial calcification in Sturge W e b e r ' s Syndrome is very uncommon. In the literature we have found only two previous reports [3, 4].
Fig. 1. Radiography of skull, first day of life. Fine curvilineal calcification in the right frontoparietal region (arrows)
Fig. 2. a and b. 50 days old. Increase in density and size of the calcification, right
cranial hemiatrophy. Diploic swelling (arrows)
0301-0449/79/0008/0039/$01.00
40
A. Alonso et al.: Intracranial Calcification in a Neonate with the Sturge Weber Syndrome
Fig. 3. Pneumoencephalography at the age of 3 months. Intense atrophy in the right hemicerebrum with displacement of the middle line structures Fig. 4. Right carotid angiography (3 months). Hypertrophy of the external carotid principally in the middle meningeal artery. Increased bilateral diploic swelling
Fig. 5. Right carotid angiography. Venous phase. Few cortical veins, with irregular filling of the longitudinal sinus
When 50 days old the infant began to have severe generalised convulsions and in the interim intracranial calcification had become more intense (Fig. 2 a and b); the calcification was also more extensive and then characteristic of the condition. The diploic widening had increased and was bilateral. Pneumoencephalography at the age of 3 months showed intense atrophy in the right hemicerebrum (Fig. 3). At angiography a large right middle meningeal artery was shown to divide into many small vessels having a telangiectatic appearance. The middle cerebral artery was elevated leaving an initially avascular space to be filled later by angiomatous vessels derived from the middle meningeal artery. During the venous phase only a few cortical veins, filling in an irregular way, emptied into the sagittal sinus (Fig. 5).
Discussion Case Report This one day old girl, weighing 3.4 Kilo, was born after a 39 week pregnancy in a 37 year old healthy mother. The father, aged 32 years, was also healthy and the parents had three healthy children, but there had been one miscarriage. There was no family history of any neuroectodermal dysplasia. At birth the infant had a flat angioma extending over the right side of the face to the mid-parietal region. Skull radiographs on the first day of life showed microcephaly (Cronquist index = 50 and a total sum of the cranial diameters of 279). There was a right parieto-temporal diploie widening of 4 mm thickness. There was also fine cerebral calcification in the right fronto-parietal region (Fig. 1).
In Sturge-Weber's Syndrome typical calcification d o e s n o t a p p e a r o r d i n a r i l y b e f o r e 2 y e a r s of a g e [4]. R a r e l y has c a l c i f i c a t i o n b e e n d e s c r i b e d in t h e v e r y y o u n g i n f a n t [3, 5]. T h e f i n d i n g s in t h e skull r a d i o g r a p h s , p n e u m o e n c e p h a l o g r a m , a n d at a n g i o g r a p h y a r e t h o s e c o m m o n l y f o u n d in t h e s y n d r o m e [2, 1, 6], b u t at a l a t e r age. C a l c i f i c a t i o n at b i r t h , t h e e a r l y o n s e t o f c o n v u l sions a n d c e r e b r a l a t r o p h y all u n d e r l i n e t h e g r a v i t y o f t h e p r o g n o s i s in this y o u n g infant.
A. Alonso et al.: Intracranial Calcification in a Neonate with the Sturge Weber Syndrome
Acknowledgement. We wish to express our appreciation to the neonatology service and the neuropediatrics service of the Clinica Infantil for their co-operation.
References 1. Bentson, J. R., Wilson, G. H., Newton, T. H.: Cerebral venous drainage pattern of the Sturge-Weber syndrome. Radiology 101, III (1971) 2. Di Chiro, G., Lindgren, E.: Radiographic findings in 14 cases of Sturge-Weber syndrome. Acta Radiol. (Stockh.) 35, 387 (1951) 3. Fanconi, G.: Sturge-Weber Syndrome beim Neugeborenen: Ausgedehnte naevi telangiectaci, partieller Riesenwuchs und intrakranielle Verkalkung der rechten Hemisphfire, die im Lauf der ersten 1 ~/2Jahre eher zur/ickgehen. Helv. Paediatr. Acta 17, 486 (1962)
41
4. Harwood Nash, D. C.: Neuroradiology in infants and children, Vol. I. St. Louis: C. V. Mosby. Co 1976 5. Nellhaus, G., Haberland, C., Hill, B.J.: Sturge-Weber disease with bilateral intracranial calcifications at birth and unusual pathologic findings. Acta Neurol. Scand. 43, 314 (1967) 6. Poser, C. M , Taveras, J. M.: Cerebral angiography in Encephalotrigeminal Angiomatosis. Radiology 68, 327 (1957) 7. Swischuk, E.L.: Radiology of the newborn and young infant. Baltimore: Williams and Wilkins 1973 Date of final acceptance: March 3, 1978
A. Alonso, M. D. Servicio de Radiodiagnostico Pediatrico Clinica Infantil "La Fe" Valencia Spain
