✓ Spinal segmental neurofibromatosis (NF) is a rare entity. To date, patients in reported cases of segmental NF (or NF5) have harbored neurofibromas involving the peripheral nerves only. The author reports a rare case of segmental NF that caused spinal cord compression in a 40-year-old woman who presented with a 6-month history of intercostal neuralgia. Examination revealed mild lower-extremity weakness and dysesthesia in the right-sided T-9 dermatome. Magnetic resonance imaging revealed three neurofibromas involving the T-9 region, which were excised, and the patient's neuralgic pain was resolved postoperatively. Traditionally, it has been believed that segmental NF involved only the peripheral nerves. The present case illustrates that although rare, spinal cord compression can also occur in patients with segmental NF.
The purpose of this study was to report the author's experience with 14 cases of terminal and nine cases of nonterminal myelocystoceles and to highlight the differences between these two groups in regard to the embryological origins, clinical presentation, operative findings, results on neuroimaging studies, and prognosis for these lesions.
This is a retrospective analysis of 14 cases of terminal and nine of nonterminal myelocystoceles treated between January 1998 and January 2006. All patients underwent neurological examination, plain x-ray films of the spine, computed tomography scans of the brain, and magnetic resonance (MR) imaging of the spine. In seven of these cases, MR imaging included three-dimensional constructive interference in steady-state sequences, and in four cases MR myelography was also done. Follow-up duration ranged from 3 months to 4 years.
All of the patients with terminal myelocystocele presented with swelling in the low back and varying degrees of neurological deficits, except four who had normal results on neurological tests. The MR images revealed classic features of terminal myelocystoceles in all patients. In each case, patients underwent excision of the meningocele sac and drainage of the syringocele with untethering of the spinal cord. During the last follow-up visit, there was no change in the neurological status of these children. In the nonterminal myelocystocele group, one lesion was cervical, six were thoracic, and two were lumbar lesions. All except one patient presented without neurological deficits; that patient had paraplegia with incontinence. Admission MR images revealed Rossi Type I nonterminal myelocystocele in six and Rossi Type II nonterminal myelocystocele in three patients. Children with Type I lesions underwent excision of the fibroneurovascular stalk and excision of the meningocele sac, whereas those with Type II lesions underwent drainage of the syringocele, untethering of the cord, and excision of the meningocele sac. There was no change in the neurological status postoperatively. During the follow-up period no patient in either group presented with retethering.
Myelocystoceles, both terminal and nonterminal, are different from other skin-covered masses in the back. A proper imaging evaluation is required to differentiate myelocystoceles from other skin-covered masses in this area, because the surgical treatment and prognosis are different for this subset of patients with occult spinal dysraphism. Terminal myelocystoceles are different from nonterminal ones embryologically, clinically, radiologically, surgically, and prognostically. These differences are discussed.
David G. McLone
Natarajan Muthukumar, Govindaraju Venkatesh and Subbiah Thiruppathy
✓Hydrocephalus is known to be associated with syringomyelia; however, the association between arrested hydrocephalus and syringomyelia has been reported only once before. This presyrinx state is a recently recognized condition that is known to resolve with proper treatment. This case report highlights a rare association between arrested hydrocephalus and the presyrinx state and outlines the implications for treatment and etiopathogenesis.
A 6-year-old girl with arrested hydrocephalus presented with a recent history of difficulty in walking despite no antecedent trauma or infection. An examination revealed macrocrania and features of cervical myelopathy, and magnetic resonance (MR) imaging revealed panventriculomegaly with evidence of the presyrinx state on both T1- and T2-weighted images of the cervical spine. The patient underwent a procedure to insert a ventriculoperitoneal shunt that used a medium-pressure system. After the shunt was inserted, the patient's myelopathic symptoms gradually improved over a period of 6 months. Postoperative MR images obtained 1 year later revealed decompression of the ventricular system and complete resolution of the presyrinx state in the cervical spinal cord. Decompensation in arrested hydrocephalus is a well-known phenomenon. This case illustrates the concept that the presyrinx state may be one of the manifestations of decompensated arrested hydrocephalus. The importance of early recognition of this condition and its implications for the pathogenesis of syringomyelia are discussed.
Natarajan Muthukumar, Alwar Govindan Santhanakrishnan and Krishnaswamy Sivakumar
Obstetric brachial plexus palsy is not uncommon. However, lesions masquerading as obstetric brachial plexus palsy are rare. A child with a cervicothoracic arachnoid cyst masquerading as obstetric brachial plexus palsy is presented, and the relevant literature is reviewed. A girl born by vaginal delivery at full term without any antecedent risk factors for obstetric brachial plexus palsy was noted to have decreased movements of the right upper extremity. After 7 months, there was no improvement. An MRI scan was obtained, which revealed a cervicothoracic spinal extradural arachnoid cyst. During surgery, the cyst was found to communicate with the dura at the axilla of the C-7 nerve root. The cyst was excised in toto. Six months later, there was improvement in the infant's neurological status. This case illustrates that spinal arachnoid cysts should be entertained in the differential diagnosis when a child presents with obstetric brachial plexus palsy without known antecedent risk factors for obstetric palsy.
Natarajan Muthukumar, Venkatachalam Sureshkumar and Vengalathur Ganesan Ramesh
✓Spinal intradural extramedullary tuberculoma is a rare entity. Rarer still are extensive en plaque intradural extramedullary tuberculomas occurring concurrently with multiple intracranial tuberculomas as a paradoxical response to chemotherapy for tuberculosis (TB). The authors describe the case of a 21-year-old man who was treated for tuberculous meningitis. Three months after the episode of meningitis, while undergoing chemotherapy for TB, he developed features of thoracic myelopathy. Investigations revealed an extensive en plaque intradural extramedullary lesion spanning seven segments in the lower thoracic spine. Magnetic resonance imaging of the brain revealed multiple asymptomatic intracranial tuberculomas. Even after further treatment with antituberculous chemotherapy was initiated, the lesion failed to respond. The authors performed a laminectomy and excised the en plaque intradural extramedullary lesion. The patient’s condition responded well to this treatment.
Although the appearance of intracranial tuberculoma as a paradoxical response to chemotherapy has been previously reported, no authors have reported on the development of an extensive en plaque intradural extramedullary tuberculoma in conjunction with asymptomatic multiple intracranial tuberculomas as a paradoxical response.
In cases in which patients present with compressive myelopathy following therapy for tuberculous meningitis, it is important to consider in the differential diagnosis that intradural extramedullary tuberculoma may be a paradoxical response to chemotherapy. The authors’ experience and their review of the literature indicate that surgery has a definitive role to play in the management of spinal intradural extramedullary tuberculoma.
Yee Chiung Gan and Douglas Cochrane
Natarajan Muthukumar, Bhuvaneswari Subramaniam, Thangaraj Gnanaseelan, Ramesh Rathinam and Appaswamy Thiruthavadoss
Object. Anorectal malformations are known to be associated with neurological deficits, which may contribute to the disability suffered by patients with these malformations. This study was undertaken to determine the incidence and pattern of sacral abnormalities in children with anorectal malformations, the incidence and nature of the neurological deficits, and the incidence and nature of operable intraspinal abnormalities in patients with this condition.
Methods. Neurological evaluation was performed in 81 children with anorectal malformations. Plain x-ray films were obtained to identify the presence of sacral abnormalities. The patients with neurological deficits were evaluated for the presence of operable intraspinal anomalies, and when such anomalies were identified, correction of the same was undertaken. In 21% of these children radiographic evidence of sacral abnormalities was shown. Fifteen percent of patients harbored neurological deficits, and 10% harbored operable intraspinal anomalies. In addition, one patient had split notochord syndrome. Patients with operable intraspinal anomalies underwent surgical correction, with resultant neurological improvement.
Conclusions. Bone abnormalities of the sacrum, neurological deficits, and operable intraspinal lesions are not uncommon in children with anorectal malformations. Because the neurological deficits can contribute to the disability suffered by these individuals, we recommend routine screening of patients with anorectal malformations and neurological deficits and/or sacral abnormalities for the early identification and treatment of potentially correctable intraspinal lesions.