BACKGROUND: To describe the structure and efficacy of a modified cerebrospinal fluid (CSF) shunt system with Bhatti CSF access chamber and ventriculoperitoneal shunt, for the management of hydrocephalus associated with pyogenic ventriculitis.

METHODS: This was an observational study performed at the National Medical Center and Ziauddin Medical University, Karachi, Pakistan. Chemically bonded Bhatti CSF access chamber and ventriculoperitoneal shunt was inserted into 443 patients with hydrocephalus and pyogenic ventriculitis to allow injection of antibiotics into the lateral ventricles.

RESULTS: In all patients, symptoms and signs of hydrocephalus were relieved immediately after shunt insertion and infection brought under control within two weeks of intraventricular injection of antibiotics (vancomycin or linezolid), via the CSF access chamber. In two patients peritoneal catheter was blocked by a precipitate of vancomycin. Reduction in the dose of intraventricular vancomycin from 50mg to 20mg and increased dilution (additional 2ml of 0.9% N/S solution) prevented recurrence of this complication. All patients recovered from ventriculitis within two weeks of initiating this treatment. Follow-up was from 3 months to 2 years to monitor continued relief of hydrocephalus and absence of recurrence of ventriculitis.

CONCLUSION: Bhatti CSF access chamber and the ventriculoperitoneal shunt is the only system available at present that fulfills the management of hydrocephalus associated with pyogenic ventriculitis. It provides continuous internal drainage of CSF combined with access to the ventricles for injection of antibiotics.

Melorheostosis is a rare and progressive mesenchymal dysplasia characterized by thickening (hyperostosis) of the cortical bone. It is a developmental error that has been described to occur due to a mutation in LEMD3 gene. It affects bone and soft tissue development resulting in severe functional limitation; chronic pain, malformed or immobilized muscles, tendons or ligaments limb deformities and leg length discrepancies. X-ray imaging is the preferred diagnostic tool for melorheostosis, which often reveals a pattern of thickened bone resembling dripping candle wax. Treatment of melorheostosis is limited and generally conservative that includes physical and occupational therapy, and in severe cases, surgery may be considered. We report a case of 68-year-old female with melorheostosis of the middle finger, with her chief complaint being gross swelling, complete restriction of movement of all joints of the middle finger and limitations in movements of joints of index and ring finger, making the hand almost non-functional. It was treated with amputation, a rare surgical indication.

 BACKGROUND: Early detection of congenital heart defects provides better postnatal treatment strategy and prognosis. Therefore, we aimed to determine the frequency of congenital heart defects on fetal echocardiography in high-risk mothers referred to the Children hospital, Lahore.

METHODS: A cross-sectional observational study was conducted in the Cardiology department, of Children Hospital, Lahore from July to December 2015. The data were collected from 138 high-risk pregnant mothers. Both maternal and fetal risk factors associated with congenital heart defects were considered for indication of fetal echocardiography.

RESULTS: Of the 138 high-risk pregnancies, 131 had maternal and 7 had fetal risk factors. Of the fetuses with maternal risk factors, the prevalence of congenital heart defects was 6%. We did not find any congenital heart defects in fetuses with fetal risk factors. 2.17% congenital heart defects presented with a history of gestational diabetes mellitus and 3.62% presented with poor obstetric history (p ≤ 0.05). Atrial septal defects and ventricular septal defects each were present in 2 (1.4%) fetuses while 1 (0.72%) fetus had complete atrioventricular septal defect, 2(1.4%) had septal hypertrophies and 1 (0.72%) had pericardial effusion.

CONCLUSION: We observed a 6% frequency of congenital heart defects in high-risk mothers. With this high frequency of congenital heart defects, fetal echocardiography should be included as part of second-trimester anomaly scan in all high-risk mothers.

BACKGROUND: Orthotopic liver transplantation (OLT) includes the implantation of partial or complete liver graft from a living or deceased donor into the recipient. The purpose of this study is to analyze health associated quality of life among OLT recipients.

METHODS: This study was conducted at a tertiary care center from January 2011 to January 2015. The quality of life questionnaire was completed before OLT and 6 months after OLT by 32 patients.

RESULTS: Mean age of liver transplant recipients was 45±11 years, body mass index (BMI) was 24.2±4.2 kg/m2 and 28/32 (87.5%) patients were males. Good health was reported by 96.9% after OLT in contrast to 81.2% patients before OLT (p=0.0001). Vigorous exercise capability was 40.6% after OLT in contrast to 28.1% before OLT (P=0.43).

CONCLUSION: We found a significant increase in quality of life scores among patients who underwent OLT. However, compared to pre-OLT, recipient’s participation in vigorous activities did not change 6 months after OLT.