Cavernous malformations of central nervous system in pediatric patients: our single-centered experience in 50 patients and review of literature.

PURPOSE: Cavernous malformations (CMs) are rare developmental cerebrovascular malformations of the central nervous system with a childhood prevalence of 0.3 to 0.53%. Our purpose was to assess the clinical features and microsurgical outcome in pediatric central nervous system (CNS) CMs. MATERIAL AND METHODS: We retrospectively enrolled all the CM patients admitted to our institute from 1 January 2001 to 31 December 2014. Data was analyzed for their clinical features and surgical outcome. RESULTS: A total of 50 patients with CMs (30 supratentorial, 14 infratentorial, and 6 spinal) with a mean age of 14 years (3-18 years, SD ±4.64) were enrolled into the study. Most of these patients (78%) were male. Size varied from 1.2 to 6 cm. Three patients had multiple CMs. Symptoms of CMs were site specific. Seizure was the most common symptom (63.3%) of CMs at supratentorial location followed by headache (46%) and neurodeficiency (26%), while all brainstem and spinal CMs presented with neurodeficiencies. History of clinically significant acute hemorrhage was present in 19.2% of supratentorial (ST) superficial CMs, 50% of ST deep CMs, 25% of cerebellar CMs, 44.4% of brainstem CMs, and 50% of spinal CMs. Forty-five CMs in 44 patients were surgically excised. Their follow-up ranged from 6 to 162 months (mean 47.2 months, SD ±53). All supratentorial CM patients showed improvement in their symptoms. Patients with preoperative seizure showed good seizure control with Engel scale I in 16 (94.1%) and Engel scale II in 1 (5.9%). In infratentorial (IT) and spinal CM patients, 92.3 and 66.7% had improvement in their neurodeficiencies, respectively. There was no mortality in our series. CONCLUSION: Microsurgical excision of CNS CM results in excellent neurological outcome in pediatric patients. Early intervention is necessary in spinal CMs for better outcome.

Endoscopic pituitary surgery: Techniques, tips and tricks, nuances, and complication avoidance.

Endoscopic pituitary surgery is useful in all micro- and macro-pituitary adenomas including those with suprasellar and cavernous sinus extension. The endoscope provides a panoramic close-up, a multi-angled view with excellent illumination and magnification, permitting complete excision of the tumor with preservation of normal pituitary. However, surgeons need to learn altogether different skills unique to endoscopy and the learning curve is steep. The learning curve can be shortened by proper selection of cases, gradual transition from the microscopic to the endoscopic approach, adequate sphenoethmoidal recess widening, identification of important landmarks during each stage of surgery, and use of neuronavigation. Results and long term outcomes can be improved with bimanual dynamic dissection and sequential tumor excision, preservation of normal pituitary, avoidance of arachnoidal tear and use of extended approach for tumors with large suprasellar extension. The gradual transition from microscopic to endoscopic approach, adherence to step by step technique and learning 'tips and tricks' of the endoscopic pituitary surgery reduce complications.

Surgical management of odontoid fractures at level one trauma center: a single-center series of 142 cases.

INTRODUCTION: Odontoid fractures constitute 9-20% of all adult cervical spine fractures. The present study was carried out to focus on the nuances involved in the surgical management of odontoid fractures. MATERIALS AND METHODS: Patients with an odontoid fracture, admitted for surgical stabilization, between January 2008 and March 2014, were included in the study. RESULTS: Among 142 patients [127 male and 15 female patients; median age: 28 years range 4-75 years], type II odontoid fractures were present in 111, type IIA fractures in 8, and type III fractures in 23 patients. 58.5% patients had been involved in a motor vehicular accident while 38.7% had sustained a fall. Eighty-five patients (59.9%) with a well-reduced fracture and an intact transverse ligament underwent anterior odontoid screw (OS) placement; the other 57 patients (40.1%) underwent posterior fixation (PF). The mean follow-up duration was 22 months (range: 6 months-5.4 years). OS placement was successful in 82 patients (96.5%) with a fusion rate of 95% (95.8% in type II, 100% in type III, and 75% in type IIA odontoid fractures). The procedure-related morbidity was 11.7%. One patient died of sub-arachnoid hemorrhage (SAH) that occurred during OS placement. The PF procedures had a better fusion rate (96.5%). The latter patients, however, had significant restriction of their neck movements and an overall morbidity of 8.7%. The revision surgery rates after OS placement and PF fixation were 7% and 3.5%, respectively. CONCLUSIONS: Anterior OS fixation shows excellent fusion rates and should be the first-line management in reduced/non-displaced acute type II (including type IIA) and high type III odontoid fractures as it preserves cervical motion. PF, that has also been associated with an excellent fusion rate, should be reserved for patients where OS fixation has either failed or has not been feasible.

Retrospective analysis of the outcomes of endoscopic transsphenoidal surgery for Cushing's disease.

Background: The first-line surgical management of an adrenocorticotropic hormone (ACTH)--secreting pituitary adenoma causing Cushing's disease (CD) is endoscopic transsphenoidal resection of the tumor. This study was performed to assess postoperative (postop) complications and remission in endoscopic surgically resected cases of CD. Methods: Data of patients who underwent endoscopic transsphenoidal surgery (ETSS) for CD were collected from the neurosurgery department at a tertiary care center in a retrospective manner from January 2015 to February 2022 and analyzed. Postoperative remission was categorized as - early morning serum cortisol <138 nmol/L within 7 days of the surgery, as per the Endocrine Society Guidelines, with significant clinical improvement in features of hypercortisolism in the operated patient and strict cutoff rate of <50 nmol/L at postop day 3 was also utilized, to look for the early identification of remission. Results: A total of 41 patients were identified who underwent 44 ETSS during the same timeframe. Preoperative magnetic resonance imaging localized an adenoma in all 41 patients, out of which 32 were microadenoma, and nine were macroadenoma (2 with cavernous sinus invasion). Intrapetrosal sinus sampling was performed in 35 (85%) patients. The rate of remission for the initial surgery was 85.4% using the standard criteria and 68.3% using strict criteria. Three patients underwent early repeat surgery for the persistent disease as the day 3 cortisol was high (306-555 nmol/L). Once the outcome of this surgery was also included, the overall rate of remission was 90.2% (37/41). None of the patients had meningitis, cerebrospinal fluid leakage, visual deterioration, or vascular injury. Permanent and transient diabetes insipidus (DI) occurred in 9.75% and 26.8% following the first ETSS, respectively. We also noted a single case of CD recurrence in 9 months during the total follow-up period of 84 months. Conclusion: ETSS has satisfactory rates of remission for the primary treatment of CD, with rates being higher for microadenomas. A long follow-up period is needed to assess the rates of recurrence. Patients must be counseled regarding the risk of postop DI, whether transient or permanent, as a possible complication.

"Rabbit ear" scalp deformity caused by massive subdural effusion in infant following bilateral bur-hole drainage failure: successfully managed with subduro-peritoneal shunt.

INTRODUCTION: Subdural effusion in an infant is a rare clinical scenario which may be secondary to a variety of etiologies. Massive subdural effusion is an extremely rare complication of head injury. CASE REPORT: Authors report a rare case of progressive massive subdural effusion which, despite bilateral bur-hole placement and drainage, failed and presented with visual deterioration and massive bulge of the scalp at bur-hole sites, producing a rabbit ear appearance in a 10-month-old infant. Ultimately, cystoperitoneal shunt was carried out in a desperate attempt to prevent impending rupture of scalp sutures at sites of previous bur-hole placement. This was followed by not only complete resolution of hygroma but also visual recovery. The patient is doing well at 6 months following shunt, regaining normal vision and appropriate developmental milestones. A MRI scan of the brain was carried out at last follow-up, which revealed mild ventriculomegaly with the rest of the brain being unremarkable, and subduro-peritoneal shunt in situ. Such a case has not been reported in the literature till date. DISCUSSION: Subdural effusion usually runs a self-limiting course. Though neurosurgical intervention is occasionally needed, different methods of surgical procedure for management include bur hole alone, bur holes with subdural drain placement, twist drill craniotomy with drain, and even craniotomy. Various methods of management are discussed along with a review of pertinent literature.

Complication Avoidance in Endonasal Endoscopic Pituitary Surgery.

Endoscopic endonasal surgery for pituitary tumors can be performed safely with a good outcome. Complications are uncommon and relate most often to nearby structures at risk. The authors detail the nuances, tips and tricks useful in avoiding complications and their early detection and management.

Outcome after hemorrhage following Gamma Knife surgery for cerebral arteriovenous malformations.

OBJECT: Although the effects of Gamma Knife surgery (GKS) on the risk of hemorrhage are poorly understood, a certain subset of patients does suffer bleeding after GKS. This study was undertaken to analyze the outcome of patients sustaining hemorrhage after GKS; it is the most feared complication of radiosurgical management of cerebral arteriovenous malformations (AVMs). METHODS: Between May 1997 and June 2006, 494 cerebral AVMs in 489 patients were treated using a Leksell Gamma Knife Model B, and follow-up evaluations were conducted until June 2007 at the All India Institute of Medical Sciences in New Delhi. Fourteen patients who sustained a hemorrhage after GKS formed the study group. In most of these patients conservative management was chosen. RESULTS: The mortality rate was 0% and there was a 7% risk of sustaining a severe deficit following rebleeding after GKS. None of the patients sustained rebleeding after complete obliteration. Patients with Spetzler-Martin Grade III or less had increased chances of hemorrhage after GKS (p < 0.002). The presence of deep venous drainage, aneurysm, venous hypertension, or periventricular location on angiography was common in patients with hemorrhage after GKS. CONCLUSIONS: The risk of hemorrhage that remains following GKS for cerebral AVMs is highest in the 1st year after treatment. The present study showed a relatively good outcome even in cases with hemorrhage following GKS, with no deaths and minimal morbidity, further substantiating the safety and efficacy of the procedure.

Endoscopic Management of Fourth Ventricle Neurocysticercosis: Description of the New Technique in a Case Series of 5 Cases and Review of the Literature.

BACKGROUND: Around 7%-33% of cases of neurocysticercosis (NCC) have intraventricular involvement, and the fourth ventricle is the most frequent site. Medical management and various surgical approaches have been described for treating this disease. The objective of this study was to describe technical modification for endoscopic fourth ventricular NCC removal in a series of 5 cases. METHODS: In this study (January 1, 2016, to December 31, 2017), all cases of fourth ventricular NCC which were treated with a special technique (endoscopic transcortical transforaminal transaqueductal approach) using a rigid endoscope system and 6-French infant feeding tube (IFT) were included in the study. The IFT was passed through the main channel, the cyst was engaged at the tip by applying gentle suction with a 20 cm3 syringe, and the cyst was removed along with the whole endoscopic assembly. Patient's clinical, radiologic, and follow-up data were retrieved from the department database records. RESULTS: Five patients (3 men, 2 women; mean age, 20 years; range, 11-27 years) were enrolled. All patients had features of raised intracranial pressure. Two patients also had drop attacks, and one presented with altered sensorium and one had upgaze palsy. Duration of symptoms ranged from 3 months to 3 years. All patients had isolated fourth ventricular NCC with obstructive hydrocephalus. Complete removal of the neurocysticercal cyst could be performed in all patients without any injury to the periaqueductal region or fornix. There was no intraoperative rupture of the neurocysticercal cyst. On follow-up (range, 12-28 months; mean, 19.4 months), all patients had relief of symptoms and imaging showed no cyst and hydrocephalus. CONCLUSIONS: We conclude that our endoscopic approach is safe, simple, cost-effective, and allows minimally invasive removal of the fourth ventricle cyst and treatment of hydrocephalus without any morbidity.

Does hemorrhagic presentation in cerebral arteriovenous malformations affect obliteration rate after gamma knife radiosurgery?

OBJECTIVES: Radiosurgery has been widely adopted for the treatment of cerebral AVMs. However radiosurgical treatment of patients with hemorrhagic presentation is fraught with risk of rebleed during latency period. The present study intends to analyze the obliteration rate, time to obliteration and chances of rebleed in patients with hemorrhagic versus non-hemorrhagic clinical presentation in cerebral arteriovenous malformations (AVMs) treated with gamma knife radiosurgery (GKS). PATIENTS AND METHODS: Of all the patients with cerebral AVMs treated from May 1997 to June 2006, 157 patients with neuroimaging follow up with digital subtraction angiography harboring 160 AVM nidii formed the study group. The mean age of presentation was 28 years (range, 6-58 years); mean nidus volume being 3.64 cm3 (range, 0.011-36.6 cm3). The mean follow up period was 70 months (range, 13-121 months). All the patients were treated predominantly by primary GKS with use of adjunctive pre-GKS embolization in selected patients. RESULTS: A total of 103 (64%) patients presented with hemorrhage. There was no difference in the obliteration rate (69% versus 67%, p=0.672), mean latency period to obliteration (30 months versus 32 months, p=0.1989) and chances of hemorrhage (4.8% versus 3.5%, p=0.690) in patients with hemorrhagic as compared to non-hemorrhagic presentation. CONCLUSION: Prior hemorrhage does not affect the outcome after GKS in terms of obliteration rate, latency to obliteration as well as chances of hemorrhage during latency period. Gamma knife appears equally efficacious irrespective of the mode of clinical presentation in the management of cerebral AVMs; a concomitant use of pre-GKS embolization/surgery may be needed in patients with hemorrhagic presentation in selected cases, however.

Spontaneous thrombosis of giant cavernous internal carotid artery aneurysm in a neonate. Case report and review of the literature.

Neonatal intracranial aneurysms are rare, with only 20 cases being reported in the literature. No case of spontaneous resolution of a giant intracranial cavernous segment aneurysm in a neonate is reported till date. The authors describe a 3-week-old male child who presented with a left-sided tonic seizure episode and was diagnosed as having a right-sided giant cavernous internal carotid artery aneurysm. The aneurysm was found to be totally thrombosed on angiography done before planning definitive treatment. The child is doing well at 2 years of follow-up. Spontaneous thrombosis, an exceptionally rare but fortunate outcome in a rare case of giant neonatal internal carotid artery aneurysm, has led the authors to report this case. The need of repeat imaging or angiography should be considered to rule out this rare outcome, more so if there is any delay between the ictus and treatment.

Totally cystic intradural extramedullary schwannoma.

Totally cystic intradural extramedullary lesions are uncommon and form a different spectrum of conditions as compared to the commonly seen intradural extramedullary tumors. Spinal schwannomas are benign tumors arising from spinal nerve root sheaths and are the most common intradural extramedullary spinal tumors. Though cystic changes in schwannomas are well described, most spinal schwannomas are solid or heterogeneous solid tumors and a totally cystic schwannoma is uncommon. The authors describes a case of totally cystic intradural extramedullary schwannoma and stress the importance of complete contrast imaging to differentiate this rare but almost completely curable tumor from other common intradural cystic lesions of the spine.

MR imaging in aneurysmal bone cyst of the orbit.

Aneurysmal bone cyst is a benign fibro-osseous lesion usually described in the long bones. Intraorbital aneurysmal bone cyst is a rare clinical entity with an entirely different outcome and prognosis as compared to the usual pediatric orbital tumors. The authors report magnetic resonance imaging (MRI) features of an orbital mass lesion in a 4-year-old child who presented with painless proptosis of right eye. Characteristic MRI findings led to a suspicion of an aneurysmal bone cyst that was totally excised via a right frontotemporal craniotomy. Appreciation of characteristic MRI features can be of extreme help for appropriate diagnosis and management of this rare entity.

Eumycetoma presenting as a cerebellopontine angle mass lesion.

Eumycetoma in the cerebellopontine angle region is extremely uncommon with no case being reported as per an extensive review of the literature by the authors. The authors report a case of cerebellopontine angle eumycetoma in a young female managed by subtotal decompression and antifungal treatment. The pre-operative diagnosis of eumycetoma in this location is extremely difficult and the role of histopathology is very important to characterize this uncommon lesion. The prognosis of this bizarre pathology is dismal despite all treatment modalities as compared to the usual tumors of the cerebellopontine angle that generally have a favorable outcome.

Unusual cause of lumbar canal stenosis in 8th decade of life - Spinal epidural lipomatosis.

Spinal Epidural lipomatosis (SEL) is an uncommon condition, usually presents in young and middle aged patients, with male preponderance. Idiopathic SEL is rare, particularly in 8th decade of life. SEL should also be considered as a differential diagnosis in approach of elderly patient presenting with lumbar canal stenosis. Such a case of 77 year old man is presented here.

Fatal Superior Sagittal Sinus and Torcular Thrombosis After Vestibular Schwannoma Surgery: Report of a Rare Complication and Review of the Literature.

BACKGROUND: Cerebral venous sinus thrombosis (CVST) is a rare condition with the potential to cause severe morbidity and mortality. CVST can also occur after vestibular schwannoma (VS) surgery with the thrombosis of transverse and sigmoid sinus. However, there is not a single report of superior sagittal sinus (SSS) thrombosis after VS surgery reported in the literature. CASE DESCRIPTION: A 45-year-old woman presented to our center with large left-sided solid cystic VS. On admission she was dehydrated, and after clinical stabilization, she underwent gross total excision of tumor through left retromastoid suboccipital craniotomy after cerebrospinal fluid drainage through an external ventricular drain. Surgery was uneventful, but postoperatively she had an episode of seizure. Immediate postoperative computed tomography (CT) brain scan was normal with good operative cavity. However, 24 hours later, she developed left-sided motor deficit, and a repeat CT scan showed right frontal parenchymal hemorrhage with intraventricular extension. On further evaluation, magnetic resonance venography showed entire SSS thrombosis, with patent bilateral transverse and sigmoid sinuses. She was not started on the anticoagulants in view of intracranial hemorrhage. Subsequently, she underwent right-sided decompressive craniectomy because there was progressive deterioration in her Glasgow Coma Scale, and she succumbed despite all efforts. Retrospectively, dehydration and intracranial hypotension could be likened to her sinus thrombosis. CONCLUSIONS: This case underscores the significance of adequate optimization of the patients prior to surgery, besides adequate operative skills to avoid this rare but serious complication of SSS and torcular thrombosis after VS surgery.

Acute Foramen Magnum Syndrome Following Single Diagnostic Lumbar Puncture: Consequence of a Small Posterior Fossa?

BACKGROUND: Type I Chiari malformation (CMI) is a rare complication of lumbar cerebrospinal fluid (CSF) drainage that is usually reported after lumbar drain or lumboperitoneal shunt placement. It usually remains asymptomatic; however, even if it becomes symptomatic, symptoms are usually mild. There are only a few reports of acute foramen magnum syndrome following continuous lumbar CSF drainage, and acute foramen magnum syndrome after a single diagnostic lumbar puncture (LP) has not been previously reported. We encountered this catastrophic complication in one of our patient. CASE DESCRIPTION: A 30-year-old woman with a large supratentorial meningioma and associated asymptomatic CMI presented with holocranial headache. She underwent successful and uneventful excision of the tumor. However, she developed quadriplegia and respiratory arrest 48 hours following a diagnostic LP performed on postoperative day 9. She underwent urgent posterior fossa decompression after magnetic resonance imaging showed increased tonsillar impaction and swelling along with cervicomedullary compression. Postoperatively, she steadily improved and regained normal power after 3 months. Retrospective quantitative analysis of magnetic resonance imaging (MRI) revealed a small posterior fossa. CONCLUSIONS: The association of intracranial tumors and lumbar CSF drainage with CMI is uncommon. The documentation of a small posterior fossa signifies the importance of both developmental (small posterior fossa) and acquired (intracranial tumor/lumbar CSF drainage) factors in pathogenesis of CMI. Although the extreme rarity of acute deterioration following a single LP does not warrant LP to be contraindicated in such patients, documentation of resolution of CMI with postoperative MRI before performing lumbar CSF drainage (whether therapeutic or diagnostic), might be helpful in avoiding this rare complication.

Quantitative analysis of variable extent of anterior clinoidectomy with intradural and extradural approaches: 3-dimensional analysis and cadaver dissection.

BACKGROUND: Drilling of the anterior clinoid process (ACP) is an integral component of surgical approaches for central and paracentral skull base lesions. The technique to drill ACP has evolved from pure intradural to extradural and combined techniques. OBJECTIVE: To describe the computerized morphometric evaluation of exposure of optic nerve and internal carotid artery with proposed tailored intradural (IDAC) and complete extradural (EDAC) anterior clinoidectomy. METHODS: We describe a morphometric subdivision of ACP into 4 quadrangles and 1 triangle on the basis of fixed bony landmarks. Computerized volumetric analysis with 3-dimensional laser scanning of dry-drilled bones for respective tailored IDAC and EDAC was performed. Both approaches were compared for the area and length of the optic nerve and internal carotid artery. Five cadaver heads were dissected on alternate sides with intradural and extradural techniques to evaluate exposure, surgical freedom, and angulation of approach. RESULTS: Complete anterior clinoidectomy provides a 2.5-times larger area and 2.7-times larger volume of ACP. Complete clinoidectomy deroofed the optic nerve to an equal extent as by proposed the partial tailored clinoidectomy approach. Tailored IDAC exposes only the distal dural ring, whereas complete EDAC exposes both the proximal and distal dural rings with complete exposure of the carotid cave. CONCLUSION: Quantitative comparative evaluation provides details of exposure and surgical ease with both techniques. We promote hybrid/EDAC technique for vascular pathologies because of better anatomic orientation. Extradural clinoidectomy is the preferred technique for midline cranial neoplasia. An awareness of different variations of clinoidectomy can prevent dependency on any particular approach and facilitate flexibility.