A case of von Hippel-Lindau disease with multi-organ involvement: a rare case report

Von Hippel-Lindau (VHL) disease is a rare autosomal dominant syndrome manifested by a spectrum of tumours in the central nervous system (CNS) and other visceral organs. We herein report a case of 35 years aged newly diagnosed diabetic female patient presented with headache, gait instability, loss of vision in both eyes, left sided hearing impairment and subsequently diagnosed to have VHL disease. The pathophysiology involves the inactivation of the VHL tumour suppressor gene. Early recognition and treatment remains the mainstay of management. Even many years after the complete tumour excision, newer neoplasms may develop. Increasing knowledge about the molecular enabled us to investigate the role of anti-angiogenic drugs. Continuous surveillance at regular interval must be conducted in patients with VHL disease.

An analysis of factors determining the requirement of ventriculoperitoneal shunt surgery in the children suffering from posterior fossa tumour: a single centre retrospective review

Background: Hydrocephalus is a relatively common occurrence in a children suffering from the posterior fossa tumour (PFTm). However, there is a divided opinion regarding the ventriculoperitoneal shunt (VPS) surgery before the posterior fossa tumour resection in a child. For the better clinical outcome, we should be able to predict which patient will require VPS following the resection of PFTm. Purpose of our retrospective analysis is to analyse various factors that predicts the necessity of VPS following PFTm resection.Methods: A consecutive series of twenty-six patients who underwent PFTm resection without undergoing VPS preoperatively are analysed in our series.Results: In our series, we found that the younger age at presentation, incomplete tumour resection, longer period of artificial ventilatory support, insertion of external ventricular drain (EVD) and its duration during the postoperative period correlate the necessity of VPS following PFTm resection. However, the severity of hydrocephalus prior to tumor surgery, tumour size, anatomical location of the tumour, tumour dissemination, use of Dural grafts during closure and histopathological type do not predict the requirement of the VPS following PFTm.Conclusions: Patients who are younger at diagnosis should be treated with utmost importance. Gross total resection should be the goal. Factors which predict the likelihood of the EVD which parallels the likelihood of postresection hydrocephalus must be prevented for the better clinical outcome.