Abstract
Objective and importance
Whereas posthemorrhagic hydrocephalus is well known after preterm birth, its association with syringomyelia has been reported only once. Here, we describe two additional patients showing this rare constellation.
Clinical presentation
The children had been born after 27 and 28 completed weeks of gestation, respectively. Both had developed neonatal cerebral hemorrhages. At the age of 4 years, patient 1 presented with progressive ataxia leading to the diagnosis of internal hydrocephalus and extensive syringomyelia. In patient 2, progressive ventriculomegaly and syringomyelia were diagnosed at the age of 4 weeks.
Intervention
In both children, ventriculoperitoneal shunting resulted in clinical improvement, decrease of the ventricular size, and regression of the syringomyelia. After surgery, patient 1 developed a subdural hematoma and patient 2 parenchymatous cerebral hemorrhages.
Conclusion
The combination of syringomyelia and posthemorrhagic hydrocephalus may be more frequent than commonly assumed. Therefore, neurological deterioration may mandate spinal MRI in addition to cerebral MRI. Syringomyelia seems to result from impaired ventricular cerebrospinal fluid drainage as it regresses after ventriculoperitoneal shunting. Bleeding may complicate the postsurgical relaxation of distended brain parenchyma.
Similar content being viewed by others
References
Becker DP, Wilson JA, Watson GW (1972) The spinal cord central canal: response to experimental hydrocephalus and canal occlusion. J Neurosurg 36:416–424
Camacho A, Simon R, Munoz A, Hinojosa J, Orbea C (2008) Syringomyelia secondary to posthemorrhagic hydrocephalus in a preterm infant. Pediatr Neurol 38:211–214
Cramer BC, Jequier S, O'Gorman AM (1986) Sonography of the neonatal craniocervical junction. AJR Am J Roentgenol 147:133–139
Eisenberg HM, McLennan JE, Welch K (1974) Ventricular perfusion in cats with kaolin-induced hydrocephalus. J Neurosurg 41:20–28
Escobar A, Vega J (1981) Syringomyelia and syringobulbia secondary to arachnoiditis and fourth ventricle blockage due to cysticercosis. A case report. Acta Neuropathol Suppl 7:389–391
Heiss JD, Patronas N, DeVroom HL, Shawker T, Ennis R, Kammerer W, Eidsath A, Talbot T, Morris J, Eskioglu E, Oldfield EH (1999) Elucidating the pathophysiology of syringomyelia. J Neurosurg 91:553–562
Kawaguchi T, Fujimura M, Tominaga T (2009) Syringomyelia with obstructive hydrocephalus at the foramens of Luschka and Magendie successfully treated by endoscopic third ventriculostomy. Surgical neurology 71:349–352, discussion 352
Klekamp J, Volkel K, Bartels CJ, Samii M (2001) Disturbances of cerebrospinal fluid flow attributable to arachnoid scarring cause interstitial edema of the cat spinal cord. Neurosurgery 48:174–185, discussion 185-176
Klekamp J, Iaconetta G, Batzdorf U, Samii M (2002) Syringomyelia associated with foramen magnum arachnoiditis. J Neurosurg 97:317–322
Krishnamoorthy KS, Shannon DC, DeLong GR, Todres ID, Davis KR (1979) Neurologic sequelae in the survivors of neonatal intraventricular hemorrhage. Pediatrics 64:233–237
Oi S, Matsumoto S (1985) Slit ventricles as a cause of isolated ventricles after shunting. Child’s Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery 1:189–193
Oldfield EH, Muraszko K, Shawker TH, Patronas NJ (1994) Pathophysiology of syringomyelia associated with Chiari I malformation of the cerebellar tonsils. Implications for diagnosis and treatment. J Neurosurg 80:3–15
Piatt JH Jr (2005) Progressive syringomyelia controlled by treatment of associated hydrocephalus in an infant with birth injury. Case report. J Neurosurg 103:198–202
Stoodley MA, Jones NR, Brown CJ (1996) Evidence for rapid fluid flow from the subarachnoid space into the spinal cord central canal in the rat. Brain Res 707:155–164
Stoodley MA, Gutschmidt B, Jones NR (1999) Cerebrospinal fluid flow in an animal model of noncommunicating syringomyelia. Neurosurgery 44:1065–1075, discussion 1075-1066
Author information
Authors and Affiliations
Corresponding author
Additional information
For preparation of this manuscript, none of the authors has received funding requiring open access.
Rights and permissions
About this article
Cite this article
Weinzierl, M., Honnef, D., Assmann, B. et al. Syringomyelia in preterm children with posthemorrhagic occlusive hydrocephalus. Childs Nerv Syst 28, 2153–2156 (2012). https://doi.org/10.1007/s00381-012-1857-y
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00381-012-1857-y