DANDY-WALKER SYNDROME;

Abstract

Introduction: Dandy-Walker Malformation is an inborn condition that includesthe triumvirate of cystic enlargement of the fourth ventricle, posterior fossa distension with riseof the tentorium and agenesis of cerebellar vermis. The purpose of this study is to determinethe Clinical presentation and associated complications, mortality and morbidity of patientundergoing placement of shunt with Y- connector in Dandy Walker Malformation. StudyDesign: Case Series comprised of 85 cases. Setting: Neurosurgery Ward of Tertiary CareHospital in Karachi. Period: February 2011 to December 2015. Methodology: All patients whowere presented in OPD with complaints reminiscent of DWM with accompanied Hydrocephalusand then underwent CT scan Brain without contrast to confirm the diagnosis were admitted.The patients who have previous history of operation and other co-morbid anomalies wereomitted. These patients undergo drainage of Ventricular system and Posterior Fossa cyst wasdone mutually via Dual Shunt including Ventricular-peritoneal and Cysto-peritoneal shunt withY connector. There were few complications and mortality after this surgical intervention whichwas noted for a month. All records were recorded and evaluated by SPSS v.20. Result: Avast majority of patients included were Female. The average age of patients was 2.5 years+/- 1 Standard Deviation. The chief complaint was Hydrocephalus, existing in all patients,after that cerebellar signs in 55 (64.7%) and other signs in 10 (11.8%) patients. Others signsincludes; milestones were delayed, atypical gait and few patients were suffered from psychiatricproblems. Complications which were observed were shunt; Fracture/Dislocation in 10 (11.8%),shunt blockage in 8 (9.4%), malpositioning is also 8 (9.4%), Intracranial Hemorrhage in 7(8.24%) patients and Infection in 10 (11.8%). These complications were observed within amonth of surgery. Unfortunately, two (2.35%) patient were also expired after surgical approach.Conclusion: It is concluded that Dandy Walker Malformation is categorized by a triumvirate.Hydrocephalus is a most common symptom and principal cause of referral to hospitals. Thecommonest difficulty acknowledged after shunting is Malfunction and shunt infections. Thelowest mortality is related to Dual Shunt with Y connector when compared to other approachesfor management of shunt with Y-connector. Thus, it is found to be a worthy decision in givenDWM patients.