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Background: Hypothalamic hamartomas (HH) are malformations originating from the hypothalamus and are associated with seizures, hormonal and behavioral abnormalities., Method: Most patients, especially those with a typical syndrome characterized by gelastic seizures, precocious puberty, cognitive decline, and behavior problems, are diagnosed in childhood. Pedunculated and parahypothalamic types of hamartomas are attached to the floor by a narrow or wide peduncle in the absence of distortion of the overlying hypothalamus. This location is most commonly associated with a clinical presentation of precocious puberty, and surgical removal has proved curative in small case series. Enthusiastic resection of hypothalamic lesions are known to produce severe hypothalamic disturbance while under resection might mean inadequate response to surgery., Conclusions: In this article, the authors describe the use of extradural temporopolar approach to hypothalamic hamartoma as an improvisation to improve access with reduced morbidity and describe a surgical nuance of using posterior communicating artery to determine a safe but maximal resection margin.

Background: Aplasia cutis is a rare developmental anomaly usually involving the calvarium, associated with a variable extent of defective formation of the scalp. Adams-Oliver syndrome is a condition mainly characterized by the congenital absence of skin, known as "aplasia cutis" which is usually limited to the vertex scalp and transverse limb defects., Case Report: A 17-day-old term female neonate was referred to us with an infected scalp lesion of the vertex. The lesion which is about 10 × 9 cm had signs of infection with necrotic eschar. We started the neonate on systemic parenteral antibiotics with local dressings. On day 3 of conservative management, the neonate had exsanguination due to bleeding from the midline with severe hemodynamic compromise requiring cardiopulmonary resuscitation. After controlling the bleeding with local tamponade and resuscitating the child, she was taken for early surgery. Debridement and bipedicled rotation flap of the scalp to cover the raw area was performed. On day 18, the flap started showing signs of necrosis. The neonate was taken up for debridement, and subsequently, maternal allograft of split-thickness skin was placed as a temporary wound cover. Meanwhile, the wound showed progressive epithelialization. At 1 year, the patient continued to have a non-healing area, which was later successfully covered with a split-thickness skin graft. We plan to revaluate the need for cranioplasty at around 3-4 years of age., Discussion: We discuss the dilemmas and challenges involved in the successful management of a neonate with Adams-Oliver syndrome with infected aplasia cutis and an episode of life-threatening exsanguination., Conclusion: Aplasia cutis is a rare developmental anomaly usually involving the calvarium, associated with defective formation of the scalp to a varying extent and severity, requiring various timely strategies.

Background: A trapped fourth ventricle (TFV) is diagnosed when the inlet and outlets of the fourth ventricle are obstructed with a resultant dilated fourth ventricle., Case Report: A 3-month-old male child had a ventriculoperitoneal shunt for post-infectious hydrocephalus. At the age of 9 years, he became symptomatic with altered sensorium. He had a CT head which showed enlarged ventricles with a large fourth ventricle. A subsequent ventriculogram revealed dilated lateral ventricles with an aqueductal obstruction in association with an enlarged fourth ventricle. Although diagnosed as a TFV radiologically, his lateral ventricle shunt was revised with a diagnosis of shunt malfunction as there was a simultaneous dilatation of the lateral ventricle. He was never treated for the TFV. At his latest follow-up at 25 years of age, his MRI brain showed well-decompressed lateral ventricles with a large fourth ventricle. On clinical examination, he had truncal ataxia noticed only during turning which, according to the mother, "was always there". His MR flow study showed obstruction at the level of the aqueduct with some evidence of bidirectional flow at the foramen of Magendie., Discussion: The concept of compensated hydrocephalus represents a stage where a previously active hydrocephalus attains a nonprogressive state clinically and radiologically. We postulate the concept of a "compensated" TFV with a long-term clinical and radiological follow-up., Conclusion: We demonstrate and describe a compensated TFV and reiterate that a TFV is a functional concept with imaging being at most only corroboratory.

Introduction: Temporary diversion of cerebrospinal fluid (CSF) is often required due to patient and CSF related factors, of which infection is a significant one. The various methods available have significant disadvantages. Ventriculosubgaleal shunts (VSG) have been earlier demonstrated to be useful in a variety of circumstances., Materials and Methods: Hospital charts of 21 consecutive children during a 4-year period were analyzed retrospectively. Infection was defined based on a positive CSF culture or a history of recently treated meningitis with abnormal CSF findings. The conversion to a permanent shunt was based on normalization of CSF values in a functioning VSG shunt or when the VSG shunt is ineffective. The end point was control of raised intracranial pressure (ICP) features and infection., Results: The ages ranged from 1 month to 7 years with a median age of 2 months. Five (23.8%) were born premature. Twelve children (57.1%) had a previously untreated hydrocephalus, whereas nine (42.8%) had undergone some procedure. A positive CSF culture was obtained in ten (47.6%). Repeat VSG shunts were required in five children (23.8%). Seventeen (80.9%) of these children underwent conversion to a ventriculoperitoneal (VP) shunt. Of the remaining four, one did well without any further procedure, two died due to their primary problems, and one refused any further procedure due to poor neurological status. There were two wound complications-one CSF leak and one shunt migration., Conclusion: VSG shunts are a simple and efficient way of managing infective hydrocephalus.

Introduction: Cystic lesions in and around the third ventricle have been known to cause intermittent and acute obstruction of the foramen of Monroe with rapid onset symptoms. Most of these lesions are seen on routine investigations, whereas some are not so obvious resulting in mistaken diagnosis. Symptomatic choroids plexus cysts have only been infrequently reported, predominantly in the lateral ventricle., Case Report: A 3-year-old female child presented with rapid loss of consciousness for the first time. Computed tomography and magnetic resonance imaging scans only showed triventriculomegaly. Endoscopy revealed a cyst of the third ventricle, which was excised, leading to good recovery. Biopsy of the wall revealed a choroid plexus cyst., Conclusion: Choroid plexus cysts are rare causes for symptomatic obstruction of the third ventricle and may be difficult to detect on routine investigations and may lead to the wrong choice of treatment. Endoscopic fenestration or excision is a good option to manage such lesions.