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Spinal Neurenteric Cyst in Association with Syringomyelia : Case Report Alfredo Puca, Beatrice Cioni, Cesare Colosimo, Guido Fadda, Mario Meglio, Ugo Salvolini, and Massimiliano Visocchi Istituto di Neurochirurgia, Radiologia e Anatomia ed Istologia Patologica, Universita Cattolica, Rome, and Servizio di Neuroradiologia, Ospedale Le Torrette, Ancona, Italy
Puca A, Cioni B, Colosimo C, Fadda G, Meglio M, Salvolini U, Visocchi M . Spinal neurenteric cyst in association with syringomyelia : case report . Surg Neurol I992 ;3T202-7 . A case of spinal neurenteric cyst in association with Klippel-Feil deformity, spinal lipoma, and syringomyelia is presented. Neuroradiological evaluation was performed by means of magnetic resonance imaging of the spine and a computed tomography myelograph . The diagnosis of neurenteric cyst was established by histological and immunohistochemical studies . The pathogenesis of syringomyelia associated with an extramedullary mass lesion is discussed . Spinal neurenteric cyst ; Syringomyelia ; Spinal lipoma ; Klippel-Feil deformity KEY WORDS :
Spinal neurenteric cysts result from the persistence of an abnormal communication between entodermal and neuroectodermal tissues, through a mesodermal layer defect . Such lesions have been observed in few cases {1,7,13,16,18,20-23,311, following the first report by Pousepp in 1934 [261 . They are frequently associated with other spinal abnormalities in relation to a common maldevelopmental insult occurring during the first weeks of gestation [5,7,32) . A patient with spinal neurenteric cyst in association with Klippel-Feil deformity, spinal lipoma, and syringomyelia came under our observation . The association between neurenteric cyst and syringomyelia has never been reported before . Case Report A 34-year-old man complained in 1982 of burning dysesthetic pain in the left scapular and pectoral area and in Addren reprint requests u .' Alfledo Puca, M .D ., Istituto di Neurochirurgia, University Cattolica, Largo A . Gemelli 8, 00168 Rome, Italy . Received August 5, 1991 ; accepted August 20, 1991 . ©
1992
by
Elsevier Science
Publishing
Co., In,,
the ulnar side of the left arm . He noted also some weakness of the left hand . In January 1989, owing to the worsening of his disturbances, the patient underwent electromyography that yielded scans that were positive for left C7, C8, and TI radiculopathy. Magnetic resonance imaging (MRI) of the spine showed a right-convex cervicothoracic scoliosis, a complete fusion of TI, T2, and T3 vertebral bodies, and a widening of the spinal canal between C7 and T3 . A complex intraspinal malformation consisting of a premedullary cystlike mass at the TI level, a small intradural lipoma, and an intramedullary syrinx at the C6-C7 level was also evident . The spinal cord was displaced posteriorly and to the left (Figure 1) . In May 1989, the patient was admitted to our department . The neurological examination revealed a left arm weakness and distal amyotrophy . A dissociated sensory loss was present in the same area as that affected by dysesthetic pain ; the pain was exacerbated by coughing and straining . A left Horner syndrome was also evident . Left tricipital and cubical deep tendon reflexes were decreased. A cerebrospinal fluid examination showed aglucose value of 59 mg/ 100 mL and a protein content of 47 mg/100 mL ; no cells were present . A computed tomography (CT) myelograph (after intrathecal injection of lopamidol) confirmed the MRI findings, precisely depicting the intradural cystic premedullary mass lesion and its relationship with the spinal cord . Serial scans after 6 and 12 hours failed to demonstrate any opacification of the premedullary cyst . The extension of the syringomyelic cavity above the intradural mass was better defined . Late CT scans underlined the lateral extension of the syrinx to the left side of the spinal cord, reaching the origin of the C7 and C8 roots (Figure 2) . A laminectomy from C7 to T2 was performed : The dural sac was tense and not pulsating . The spinal cord was markedly displaced posteriorly and to the left by agrayish lesion at the T I -T2 level . The lesion was punctured, showing a milky fluid content . A complete removal of the li0090-3019/92/55 .00
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Figure 1 . Preoperative MRI of the neurenteric
cyst and associated lapoma and syringomyelia . (A) Midline sagittal TI-weighted image shows fused TI -T2-T3 vertebral bodies and ill-defined cystlike mass (Cy) displacing the cord posteriorly . Note also the thin centromedullary syrinx (arrows) above the level of the cyst. (B) Left parasagietal TI-weighted image demonstrates, at the same level of the cyst, a small hyperintense lipoma in the spinal cord (arrows) . (C) Midline sagittal T2-weighted image confirms the relationship between the cyst (Cy) and the ventral surface of the cord as well as the cerebrospinal fluidlike intensity . (D) Coronal TIweighted slice depicts the right paramedian cyst (Cy) . the small lapoma (black arrows), and the thin syrinx (white arrows) .
poma, as well as a partial removal of the cyst wall, was performed . The spinal cord was no longer tense ; therefore the syringomyelic cavity was not drained . Monitoring of spinal cord function was performed intraoperatively_ Spinal cord cervical (C7-T1) potentials evoked by stimulation of the right and left median nerves at the wrist were recorded according to the technique previously described [6] . Before laminectomy the segmental evoked potentials were normal for amplitude and waveform ; the N 13 appeared to be slightly prolonged .
During removal of the more rostral part of the lipoma the N13 potentials showed a decrease in amplitude and an increase in latency, which were more evident on the left. At the end of surgery the potentials recovered completely on the right and partially on the left . A histological study was performed on 5-µm sections, fixed in 10% buffered formaldehyde, embedded in paraffin, and stained with hematoxylin-eosin, periodic acid-Schiff (PAS), and alcian blue . Two different epithelial structures were evident : a cyliated, mono stratified,
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cuboidal epithelium of respiratory type and a monostratifled cylindric gastroenteric-like layer (Figure 3) . The mucin stain was negative . Immunohistochemical testing with avidin-biotin peroxidase complex (ABC) was performed . A cytoplasmic positivity for cytokeratins was shown; on the other hand, test results for carcinoembryonic antigen (CEA), somatostatin, and gastrin were negative (Figure 4) . The diagnosis was neurenteric cyst . We performed the determination of sex chromatin using the guard's method with biebrich scarlet solution and fast green solution : sex chromatin was absent. The cyst is of the same sex as the patient . Postoperatively a reduction of dysesthetic pain was observed . A slight superficial sensory loss in the right axillar region was also evident . Seven months later the patient was stable . The hypoesthetic area in the right axillar region was no longer evident . MRI (Figure 5) showed the persistence of the neurenteric cyst and of the syringomyelia. The lipomaappeared completely excised . A second operation was performed in February 1990 ;
Puca et al
Figure 2 . Preoperative portmyelography CT . (A,B,C) C7 1 boar after
myelography confirm, the large ventral nearenteric cyst (Cy) . the displaced and flattened cord (black arrons), and the smail lipoma (white arrow) . In the (E) sagittal and (C) coronal reformated slices the margins of the cyst as well as the relationship with the lipoma are clearly defined . (D,E,F) CT 6 hours after myelograpby demonstrates the persistently hypodense neurenteric cyst 1C)) . while there is an evident opacification of the syrinx (black arrows) . Note that the syrinxreaches the left czepect of the cord . (F) Coronal reformtated image confirm, the extension of the syrinx within the fpinal cord at the level of the cyst and cranial!y .
the neurenteric cyst was drained and its wall partially removed . A complete removal of the cyst was once again not feasible owing to the tight adherence to the spinal cord . The postoperative course was uneventful . The last MRI did not demonstrate any morphological modification compared with the previous one . The last neurological evaluation in January 1991 showed a slight improvement in the strength in the left hand . Painful dysesthesia was still present, but it was less severe .
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1992 ;37. 2 0 2 -7
Figure 3 . Photomicrograph of the cyst showing a
cuboidal monostratified epithelium ; the stroma h loose and vascu/arized (hematoxylin and eosin stain x 40) .
Discussion Spinal neurenteric cysts have been observed in children and young adults [8,13], more frequently in males [1,8,13,321, and were situated in the cervicothoracic region [1,8,32] and of intradural or intramedullary location . They can be associated with mediastinal enteric cysts with or without direct communication . Frequently other vertebral abnormalities (Klippel-Feil, vertebral defects, spondylolisthesis) [1,18,241 or spinal cord malformations (myelomeningocele, diastematomyelia, dermal sinus) [5,18,20,23] are present . The association with a spinal lipoma is less frequent [18,32] . The clinical picture is usually compatible with the presence of an intraspinal mass lesion ; frequently, pyra-
Figure 4 . Immunohistochemical study of the cyst
showing a marked e-ytoplasmicpositivity for cytokeratinr (avidin-biotin peroxidase complex x 250) .
midal signs are dominant [8]. In a few patients, apreliminary diagnosis of syringomyelia was made [21] . In the last few years, the use of MRI has allowed a more frequent observation of spinal neurenteric cysts [2,4,15,21,22,25,29] . The histological study is essential for the diagnosis . In our case, the positivity for cytokeratins shown by the immunohistochemical research demonstrated the origin of the lesion from heterotopic entodermal tissue . This finding is consonant with a recent report [17] . On the other hand, we cannot confirm the positive reaction to CEA immunohistochemical staining that has been reported by others [12,21] . The anterior location and the tight adherence to the
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Puca cc al
Figure 5 . Postoperative MRI showing residual intramedulleery neurenteric cyst and syringonryeGe . (A) Midline sagittal TI -weighted image shores a smaller residual cyst iC:y) . Thesyrinx i.r unchanged . (B) Midline .cagittad T2-weighted image confirms the cerebrospinal fuidlike signal intensity of the cyst and the syrinx . (C) TI-weighted axial image depicts the intramedullary extension of the cyst (Cy) with a thin rim of spinal cord tissue posieriorly (arrows) . The lipoma has been completely excised. (D) More cranial T I -weighted axial image demonstrates the small eccentric syrinx without spinal cord expansion .
spinal cord usually do not permit a safe total removal of neurenteric cysts [4,8,12,21,25,32] . In our patient a total removal of the lesion was not feasible because of the excessive risk associated with the separation of the cyst wall from the nerve tissue . Even though the patient's clinical picture was consistent with the presence of syringomyelia, the intraoperative aspect led us to avoid any maneuver on the cavity itselfThe association of syringomyelia and extramedullary spinal cord tumors is very unusual [3,10,11,27,28,30]The etiopathogenesis of syringomyclia in such conditions is unknown . Pathogenetic theories include "static" and "dynamic"
factors [11,30] . Static factors are due to the chronic compression of the tumor, which produces ischemia, edema, microhemorrhages, autolysis, and finally cavitation in the spinal cord [3,10,11,14,33] . A dynamic factor is the cerebrospinal fluid transmedullary flow from the subarachnoid space to the intramedullary cavity through the Virchow-Robin spaces [ 1 11 . Such flux is augmented by any acute increase in intraspinal venous pressure {10,14,27,33] . The relevance of dynamic factors is confirmed by the reduction of the syringomyelic cavity following tumor removal and resolution of the spinal block [10,27] . The aspect of the syringomyelic cavity in our patient
Surg Neurol 1992 ;37 :202-7
Neurenrcric Cyst and Syringomyelia
seems to exclude the role
of
dynamic factors, but it
is
compatible with compressive factors on the spinal cord . However, the maldevelopmental nature teric cyst, along with the presence
of
of
the neuren-
other anomalies,
does not exclude a pure dysontogenetic origin
of
the
syringomyelia . Some authors [9,19} have found histological and embryological similarities
of
different maldevelopmental
midline lesions (neurenteric cysts, colloid cysts, Rathke cleft cysts) . The association of syringomyelia with other spinal maldevelopmental lesions will probably be more easily
of spinal cord will improve our pathophysiology of syringomyelia,
detected in the future with the diffusion MRI . Such observations hopefully knowledge about the which
is
still not well defined .
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