Hydrocephalus Associated with Posterior Fossa Tumors: How to Manage Effectively?

BACKGROUND: Hydrocephalus associated with posterior fossa tumor (PFT) is a common neurosurgical problem, the management of which is still controversial. AIM: To provide an overview of the advantages and limitations of different management strategies for hydrocephalus associated with PFT both before and after resection of these tumors. METHODS: Structured review of the literature on the management of hydrocephalus in PFT both in children and adults. RESULTS: The incidence of hydrocephalus associated with PFT at the time of presentation is more in children (70-90%) than adults (10-21%). This difference is maintained for hydrocephalus after the resection of PFT (~30% for children and 1.2-6.9% for adults). Preresection hydrocephalus is obstructive while emerging evidence in the literature suggests that postresection hydrocephalus may have a communicating component. The treatment of preresection hydrocephalus associated with PFT has undergone a paradigm shift in the past two decades. Preoperative Cerebrospinal Fluid (CSF) diversion is less commonly used except when required by the clinical condition of the patient. Preresection hydrocephalus may be treated by steroid use and early tumor removal, perioperative use of external ventricular drainage, or endoscopic third ventriculostomy in selected patients. Various prediction scales are available to assess the risk of postresection hydrocephalus in PFT. Certain histological tumor types and molecular phenotypes of PFT are more commonly associated with hydrocephalus. CSF diversion through endoscopic third ventriculostomy or ventriculoperitoneal shunts remains the management strategies for postresection hydrocephalus. The failure rates and the time-to-failure of both endoscopic third ventriculostomy and CSF shunts in PFT are variable and surgeons should be aware of these while taking management decisions. CONCLUSIONS: Hydrocephalus associated with PFT affects the quality of life of patients with such lesions. Routine preoperative CSF diversion is not necessary for the vast majority of patients with posterior fossa tumor-related hydrocephalus. A high index of suspicion and aggressive surveillance is required for the early identification and appropriate management of postresection hydrocephalus. Future studies are needed to address several unanswered questions pertaining to the management of this condition.

Pituitary Apoplexy: A Comprehensive Review.

Pituitary apoplexy (PA) is a rare, usually vision-threatening and occasionally life-threatening entity. The exact incidence of PA in large series of pituitary adenomas is variable; however, it is more common in macroadenomas and nonfunctioning adenomas though it has been reported rarely in microadenomas. There are several well-known precipitating factors for PA including the use of anticoagulants, surgery, head trauma, pregnancy, etc. The typical clinical presentation is characterized by the near universal presence of headache with or without the following: visual disturbances, extraocular palsies, altered sensorium. MRI is the imaging modality of choice. Most patients have hormonal and/or electrolyte disturbances at the time of presentation which needs to be quickly corrected. Both conservative and surgical treatment modalities have been advised for the management of this condition. However, on the basis of the evidence available in the literature, the treatment should be individualized for each patient with PA. If conservative management is chosen, close clinical monitoring is necessary for early identification of deterioration. Surgery generally is through the trans-sphenoidal route. Most patients have a good recovery in visual function and extraocular palsy. There is some evidence in the literature that surgical intervention, when necessary, should be undertaken early as it is associated with better visual outcome. The majority of the patients will have residual hormonal deficits which will require prolonged hormone replacement therapy. There is a small but significant risk of recurrent PA in patients with residual tumors, especially, in those with large tumor residues. There is also a small risk of tumor recurrence following PA and hence all patients should undergo surveillance imaging periodically to detect the possible recurrence of tumor. In recent years, the mortality from PA has decreased significantly. A high index of suspicion and prompt multidisciplinary management will often lead to an overall good outcome.

Problems in Instrumentation of Syndromic Craniovertebral Junction Anomalies - Case Reports.

The aim of this study is to highlight the complications of instrumentation in the setting of syndromic craniovertebral junction (CVJ) anomalies. The records of patients with syndromic CVJ anomalies treated by this author during the period of 2012-2017 were retrospectively reviewed. Patients in whom intraoperative difficulties and complications were encountered were culled out from the database. Complications were divided into (1) technique related, (2) neural injury, (3) vascular injury, (4) instrumentation pull out/breakage, (5) inaccurate screw placement and, (6) where postoperatively, the surgeon felt an alternate surgical technique could have yielded better results. Four patients with either unexpected intraoperative difficulties or complications or in whom the technique could have been refined were identified. There were 2 patients with proatlas segmentation anomalies and 2 with Morquio's-Brailsford disease. The first patient had cage migration which necessitated a second procedure during craniovertebral realignment, the second had partial penetration of the screw into the transverse foramen, the third with bipartite atlas underwent a C1-2 fixation without a horizontal cross-connector and, the fourth had screw pull outs from the subaxial cervical spine intraoperatively during an attempted occipitocervical fusion. In children with syndromic CVJ anomalies, the surgeon should be aware of the high risk of intraoperative difficulties and complications. Potential pitfalls and the ways to avoid these complications are discussed.

Department of Neurosurgery, Madurai Medical College and the development of neurosurgery in South Tamil Nadu.

The development of neurosurgery in South Tamil Nadu can be traced to the Department of Neurosurgery, Madurai Medical College and Government Rajaji Hospital, Madurai, Tamil Nadu, India. The hospital was established in the year 1940 and Madurai Medical College was started in 1954. Prof. M. Natarajan founded this department in September, 1963. This department has a Neurosurgery Residency Program that is 50 years old. The establishment of this department and its growth to its present stature is documented here.

Proatlas segmentation anomalies: Surgical management of five cases and review of the literature.

OBJECTIVE: Proatlas segementation anomalies are due to defective re-segmentation of the proatlas sclerotome. These anomalies of the craniovertebral junction are rare and have multiple presentations. The aim of this study is to report this author's personal experience in managing five of these patients with different radiological findings necessitating different surgical strategies and to provide a brief review of the relevant literature. MATERIALS AND METHODS: Five patients, all in the second decade of life were treated between 2010 and 2013. There were three males and two females. All the patients presented with spastic quadriparesis and/or cerebellar signs. Patients underwent plain radiographs, MRI and CT of the craniovertebral junction. CT of the cranioveretebral junction was the key to the diagnosis of this anomaly. Postoperatively, patients were assessed with plain radiographs and CT in all patients and MRI in one. RESULTS: Two patients underwent craniovertebral realignment with occipitocervical fixation, two patients underwent C1-C2 fixation using Goel-Harms technique and one patient underwent craniovertebral realignment with C1-C2 fixation using spacers in the atlanatoaxial joint and foramen magnum decompression. All patients improved during follow up. CONCLUSIONS: Proatlas segmentation defects are rare anomalies of the craniovertebral junction. Routine use of thin section CT of the craniovertebral junction and an awareness of this entity and its multivarious presentations are necessary for clinicians dealing with abnormalities of the craniovertebral junction.

Spondylocostal dysostosis (Jarcho-Levine syndrome) associated with occult spinal dysraphism: Report of two cases.

Spondylocostal dysostosis, also known as Jarcho-Levine syndrome, is a rare disorder characterized by multiple vertebral and rib anomalies at birth. The association of occult spinal dysraphic lesions with this entity is rare. Two patients with spondylocostal dysostosis and occult spinal dysraphic lesions, one with type I split cord malformation and another with spinal dermal sinus are being reported. A 7-month-old female child who was operated at birth for imperforate anus was noted to have a dimple at the low back with altered skin color around the dimple. Examination revealed the right lower extremity was slightly thinner than the left. Plain radiographs showed features of spondylocostal dysostosis with scoliosis. Magnetic resonance imaging (MRI) showed a type I split cord malformation at the lumbosacral junction with low-lying conus and terminal syringomyelia. Patient underwent excision of the bony spur uneventfully. A 14-month-old male child was noted to have a small swelling in the low back along with deformity of the right lower chest since birth. Plain radiographs revealed features of spondylocostal dysostosis. MRI showed a spinal dermal sinus at the lumbosacral junction with a low-lying conus. The patient underwent excision of the spinal dermal sinus and untethering of the cord uneventfully. Although rare, spondylocostal dysostosis can be associated with occult spinal dysraphic lesions like type I split cord malformations or spinal dermal sinus. Physicians should be aware about the possibility of children with spondylocostal dysostosis harboring occult spinal dysraphic lesions so that these patients receive appropriate treatment.

Odontoid synchondrosis fracture treated by c1-2 polyaxial screw-rod fixation.

The synchondrosis between the dens and the body of axis normally fuses between 5 and 7 years of age. Until this age, synchondrosis fractures can occur in children. Most synchondrosis fractures are conventionally treated by external immobilization alone. We report a 10-year-old child with odontoid synchondrosis fracture who was treated by C1 lateral mass and C2 pars screw rod fixation with a successful outcome and discuss the possible reasons for occurrence of odontoid synchondrosis fracture in this older child as well as the indications for surgery in this condition.

Growing skull fracture: two rare causes.

INTRODUCTION: Growing skull fractures are rare complications of head injury in young children. Till date, growing skull fractures due to an underlying arrested hydrocephalus or subdural hygroma have not been reported. We are reporting two such rare cases. DISCUSSION: A 12-year-old male who was a known case of arrested hydrocephalus sustained a mild head injury and was found to have a linear fracture. One month after the initial injury, a soft swelling was noted in the parietal region. Investigations revealed the dilated ventricular system communicating through a growing skull fracture with a subgaleal CSF collection. The patient underwent a ventriculoperitoneal shunt using a high-pressure shunt system. The patient died suddenly 48 h after the surgery. An 8-month-old female child sustained a mild head injury with a linear fracture in the parieto-occipital region. Two months later, the child presented with seizures and a soft, fluctuant swelling in the parieto-occipital region. Imaging revealed a frontoparietal subdural hygroma with mass effect that was communicating through a growing skull fracture with a subgaleal CSF collection. The patient underwent a subduroperitoneal shunt. The shunt tube was removed 3 months later as it protruded through the abdominal wound. Follow-up imaging studies revealed complete resolution of the subdural hygroma with healing of the growing skull fracture. CONCLUSIONS: Growing skull fractures can occur as complications of mild head injury sustained in the setting of either arrested hydrocephalus or subdural hygroma. Hence, close follow-up of patients with skull fracture and arrested hydrocephalus/subdural hygroma is necessary for early diagnosis of growing skull fractures.

A bony human tail causing tethered cord syndrome: case report.

INTRODUCTION: Dorsal cutaneous appendages, sometimes referred to as "human tails," are considered to be markers of underlying occult spinal dysraphism. Rarely, these tail-like structures can themselves be the cause of tethered cord syndrome. However, to date, a "bony human tail" causing tethered cord has not been reported in the literature. One such rare lesion is being reported. DISCUSSION: A 2-days-old female child was brought for neurosurgical consultation with a skin-covered bony protuberance in the lower back. Examination of the child did not reveal any neurological deficits. Plain radiographic and CT evaluation showed a dorsal bony protuberance arising from the posterior elements of L1 vertebra. MRI showed the cord to be displaced posteriorly and adherent to the undersurface of the bony tail through a lipoma. During surgery, the bony "tail" was excised, and the cord was untethered with excision of the lipoma, which was tethering the cord to the bony "tail." When examined 1 year later, the child was developing normally without any focal neurological deficits. CONCLUSIONS: This case is being reported for its rarity and to highlight the hitherto unreported occurrence of "bony human tail" causing tethered cord syndrome.

Transfacet screw fixation of the subaxial cervical spine--how I do it?

BACKGROUND: Transfacet screw fixation is a biomechanically effective way of fusing the subaxial cervical spine. The technique used by this author is described. METHODS: With the patient in prone position, a conventional posterior exposure of the cervical spine is done. The entry point used by this author is 2 mm above the middle of the lateral mass without any lateral angulation. Under fluoroscopic guidance the facet is drilled until all the four cortical surfaces are purchased. Then the depth is measured to assess the length of the screw required. This is followed by tapping and screw insertion both of which are done under fluoroscopic control. All screws are placed prior to laminectomy to decompress the cervical cord. CONCLUSIONS: This is a simple, inexpensive and biomechanically effective way of stabilizing the subaxial cervical spine.

Arachnoid cyst masquerading as obstetric brachial plexus palsy.

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.

C1-C3 lateral mass fusion for type IIa and type III Hangman's fracture.

Hangman's fractures, also known as traumatic spondylolisthesis of axis, can be managed either conservatively with immobilization or by surgery. Surgery is usually indicated in cases with instability or failure of conservative treatment. Different surgical approaches, both anterior and posterior, have been described for treating Hangman's fracture. We report two patients, one with type IIa and another with type III Hangman's fracture treated with C1-C3 lateral mass fusion and discuss the advantages and limitations of this technique when compared to other techniques for fusion in patients with Hangman's fracture.

Syringomyelia as a presenting feature of shunt dysfunction: Implications for the pathogenesis of syringomyelia.

The pathogenesis of syringomyelia continues to be an enigma. The patency of the central canal and its role in the pathogenesis of communicating syringomyelia continues to elicit controversy. The case reported here provides an opportunity to retest some of the hypotheses of syringomyelia. A 33 year old female presented with sensory disturbances over the left upper extremity and trunk and was diagnosed to have panventriculomegaly with communicating syringomyelia. She was initially treated with ventriculoperitoneal shunting. As there was no change in her neurological status following shunt, this was followed by foramen magnum decompression with excision of an arachnoid veil covering the fourth ventricular outlet. She had clinical and radiological improvement after foramen magnum decompression. Five months later she had reappearance of the symptoms of syringomyelia and was found to have shunt dysfunction and holocord syrinx. She improved following shunt revision. This case is being reported to highlight the following points: 1. In patients with communicating syringomyelia and hydrocephalus, shunt dysfunction can present with symptoms of syringomyelia without the classical clinical features of shunt dysfunction, 2. In patients with communicating syringomyelia, the central canal of the spinal cord acts as an "exhaust valve" for the ventricular system, and, 3. studies about the patency of the central canal are reviewed in the context of this case and the role of the central canal in the pathogenesis of communicating syringomyelia is reviewed.

Preysyrinx state and shunt dysfunction: an underrecognized entity?

Presyrinx state is a relatively recently recognized condition. Prompt identification of this condition and appropriate treatment leads to reversal of the radiological and clinical findings with a good prognosis. Failure to identify this condition in a timely fashion leads to fully established syringomyelia. To date, presyrinx state has not been described as a feature of shunt dysfunction. One such rare case is being reported. A 14-year-old boy who was shunted for postmeningitic hydrocephalus at the age of 2 years and subsequently underwent shunt revision presented for routine follow-up and was found to have shunt dysfunction. CT scan showed panventriculomegaly and MRI studies showed panventriculomegaly with a "presyrinx" state extending from the fourth ventricle up to the thoracic region. Shunt revision led to prompt radiological reversal of the presyrinx state. This case is being reported to highlight the hitherto unreported association of shunt dysfunction and presyrinx state. The literature on presyrinx state is being reviewed with reference to the 20 cases reported earlier.