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Table of Contents    
Year : 2015  |  Volume : 63  |  Issue : 5  |  Page : 785-788

New onset syringomyelia: An uncommon presentation of ventriculo-peritoneal shunt dysfunction following treatment of hydrocephalus in the young

1 Department of Neurosurgery, Jawaharlal Institute of Medical Education and Research, Puducherry, India
2 National Neurosciences Centre, Kolkata, West Bengal, India

Date of Web Publication6-Oct-2015

Correspondence Address:
Prasad Krishnan
National Neurosciences Centre, Kolkata, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.166561

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How to cite this article:
Roopesh Kumar V R, Krishnan P. New onset syringomyelia: An uncommon presentation of ventriculo-peritoneal shunt dysfunction following treatment of hydrocephalus in the young. Neurol India 2015;63:785-8

How to cite this URL:
Roopesh Kumar V R, Krishnan P. New onset syringomyelia: An uncommon presentation of ventriculo-peritoneal shunt dysfunction following treatment of hydrocephalus in the young. Neurol India [serial online] 2015 [cited 2020 Jul 11];63:785-8. Available from:


An 11-year-old boy, who underwent a ventriculo-peritoneal shunt for postmeningitic hydrocephalus at the age of 5 years and was well after that, presented with episodes of severe low back pain, tingling in both lower limbs, and ataxia of gait of one month duration. He had no complaints of raised intracranial pressure. Magnetic resonance imaging (MRI) of the dorsolumbar spine showed holocord syringomyelia with pan ventricular dilatation without any tonsillar descent [Figure 1]. The reason for the syringomyelia was not clear and to rule out meningitis, lumbar puncture was done. The cerebrospinal fluid (CSF) analysis was normal. A syringo-subarachnoid shunt was planned but the family declined further treatment. Three weeks later, the patient presented with a sixth nerve palsy. A computed tomography (CT) scan showed gross ventriculomegaly with periventricular lucency (PVL) and absent cortical sulci [Figure 2]. The routine X-rays revealed that the entire lower end of the shunt tube had migrated into the peritoneal cavity [Figure 2]. The patient underwent a shunt revision and subsequently became asymptomatic. Postoperative MRI showed a decrease in the size of the syringomyelic cavity [Figure 1] and CT scan showed decrease in hydrocephalus as well as PVL and opening up of cortical sulci [Figure 2].
Figure 1: Preoperative sagittal T2-weighted images showing (a) pan ventricular dilatation with obliteration of cerebellar folia and brain stem compression with bowing of corpus callosum and cervical cord syringomyelia; (b) holocord syringomyelia is seen; and, (c) decrease in ventricular size with shunt seen in lateral ventricle, cerebellar folia are opened up, and brain stem compression is reduced, and (d) holocord syrinx is considerably reduced

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Figure 2: X-rays of the abdomen anterior–posterior (a) and lateral (b) views showing complete intraperitoneal migration of part of the shunt below the chamber; axial computed tomography scan images showing (c) pre-revision dilated ventricles and periventricular lucency with obliteration of cerebral sulci; and, (d) post-revision images showing decrease in ventricle size, periventricular lucency, and opening of cortical sulci

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Shunt dysfunction is a common problem in pediatric patients. Of the 234 pediatric patients who were followed up for at least 15 years, Stone et al., reported a shunt revision rate of 84.5%.[1] The common presenting symptoms in such cases include headache, vomiting, drowsiness, visual blurring, diplopia and increasing head circumference.[2] These findings are due to the accumulation of CSF in the intracranial compartment.

Rarely, the communication of the ventricular system (through the obex of the fourth ventricle) into the central canal of the spinal cord may allow egress of accumulating intracranial CSF and cause new onset progressive syringomyelia. Such a presentation of shunt dysfunction, in patients who did not have preexisting  Chiari malformation More Details, has been reported rarely in literature and we found only four reports of such cases described previously [Table 1]. The features in such cases are atypical, and the diagnosis is often delayed, as occurred in our patient also. Patients may present with quadriparesis[3],[4],[5] or paraparesis,[6] leg pain,[4] ataxia,[4] back pain,[3] wasting,[6] hypotonia,[4] as well as spasticity.[3] Our patient had back pain, ataxia, as well as dysasthesias.

He had undergone a shunt initially due to the earlier occurrence of postmeningitic hydrocephalus. The initial infection may have caused stiffening and decreased compliance in ventricular walls due to which the initial compensation after shunt malfunction was not the commonly encountered ventriculomegaly with transependymal seepage (shown as PVL on imaging) but rather progressive dilatation of the central canal of the cord. Conversely, it may well be that in cases with pliant ventricular walls and thin ependymal linings, syringomyelia may not be a presenting feature of shunt malfunction.

Another interesting factor pertaining to this case is that the central canal tends to close as the age of an individual increases (beyond the second decade of life) due to overgrowth of ependymal cells and astrocytes.[7] As a result, while almost all normal adults have a closed spinal central canal, one-third of adults who have had severe hydrocephalus in childhood, do not have an occluded spinal central canal.[7] It is unclear, though, if shunting hydrocephalic children predisposes to a normal or even early closure of the spinal central canal further making syringomyelia rare as a presenting feature of shunt dysfunction.

The duration between the initial surgery and the subsequent presentation with syringomyelia may vary. It was 6 years in our patient but has been reported to occur even after 17 years.[3],[5] Hence, a long-term follow-up both by clinical and radiological assessment, seems to be essential in these patients, as the sequelae of infection of CSF spaces in young age may persist even during adolescence and adulthood.

Aniruddha and Pruthi have emphasized that these patients may undergo needless syrinx surgeries[4] when awareness of this condition could have expedited a simple shunt revision. In our case too, we were planning a syringo-subarachnoid shunt but did not do so as the child developed a sixth nerve palsy. In our case, all symptoms regressed after shunt revision. However, in view of the likelihood of delayed occurrence and rarity of such a presentation, we would like to emphasize the need for careful long-term follow-up and also for serial imaging to confirm for a decrease in syrinx size after shunt revision. In the event of lack of clinical improvement or in imaging findings, the possibility of another cause of syringomyelia must be kept in mind and other factors must be sought for explaining its pathogenesis.

In conclusion, symptoms of syringomyelia are rarely the initial presentations of shunt dysfunction. This is more likely in those cases where hydrocephalus was a sequelae of infection and when the initial ventricular dilatation occurred prior to the closure of the central canal of the spinal cord. A high index of suspicion must be present in patients who have undergone a shunt previously and later present with atypical limb and truncal symptoms. A new onset syrinx in such individuals may herald the presence of an underlying shunt dysfunction and should prompt a corresponding cranial imaging study.

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  References Top

Stone JJ, Walker CT, Jacobson M, Phillips V, Silberstein HJ. Revision rate of pediatric ventriculoperitoneal shunts after 15 years. J Neurosurg Pediatr 2013;11:15-9.  Back to cited text no. 1
Lee TT, Uribe J, Ragheb J, Morrison G, Jagid JR. Unique clinical presentation of pediatric shunt malfunction. Pediatr Neurosurg 1999;30:122-6.  Back to cited text no. 2
Martínez-Lage JF, Alarcón F, López López-Guerrero A, Felipe-Murcia M, Ruíz-Espejo Vilar A, Almagro MJ. Syringomyelia with quadriparesis in CSF shunt malfunction: A case illustration. Childs Nerv Syst 2010;26:1229-31.  Back to cited text no. 3
Aniruddha TJ, Pruthi N. Shunt malfunction presenting with symptomatic syringomyelia: Demonstrated on contrast ventriculogram. J Pediatr Neurosci 2014;9:132-5.  Back to cited text no. 4
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Peraud A, Grau S. Decompensated hydrocephalus causing syringomyelia and tetraparesis: A case report. Childs Nerv Syst 2009;25:263-6.  Back to cited text no. 5
Beswick E, St George EJ, Solanki GA. Paraparesis secondary to expansion of cervical syrinx: An unusual presentation of ventriculo-peritoneal shunt malfunction. Pediatr Neurosurg 2005;41:334-6.  Back to cited text no. 6
Kasantikul V, Netsky MG, James AE Jr. Relation of age and cerebral ventricle size to central canal in man. Morphological analysis. Neurosurg 1979;51:85-93.  Back to cited text no. 7


  [Figure 1], [Figure 2]

  [Table 1]


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