Rubber tourniquet technique: A simple, safe, and cost-effective methodof hepatic resection in neonates and infants

Background: Despite the improvements in patient care, understanding of surgical anatomy of liver and surgical techniques, liver resection is a high-risk procedure specifically in infants and neonates; whose blood volume is limited (80 ml/kg). This report shares the experience of hepatic resection with “Rubber tourniquet technique” in patients less than 6-months of age. Methods: Hepatic resection in a 4-month-old pair of Conjoined twins with shared liver and a 3-day old baby with large Congenital Hepatoblastoma was performed using Penrose rubber drain as a tourniquet around the liver parenchyma to reduce blood loss. Results: Blood loss was minimal (\u3c 20CC) and all the three babies survived the procedure without complications. Conclusion: Rubber tourniquet technique is simple, safe and cost-effective for liver resections in neonates and infants with hepatic lesions and separation of conjoined twins with shared liver and it can easily be employed in resource constraint settings

Feminizing genitoplasty in a young girl with glanzmann\u27s thrombasthenia-management of haemostasis

We report peri- and post-operative management of haemostasis in a 11-year old girl with Glanzmann Thrombasthenia (GT) who had feminizing genitoplasty for genital ambiguity due to Congenital Adrenal Hyperplasia (CAH-21 Hydroxylase deficiency). A blend of Glanzmann Thrombasthenia (GT) and DSD 46XX due to CAH is not reported in literature. Surgery particularly genitourinary reconstruction in patients with GT is challenging due to risk of intra and post-operative bleeding. Haemostasis can successfully be achieved with platelet transfusions, antifibrinolytic (Tranexamic acid) and judicious use of recombinant factor VIIa (rFVIIa) even in a resource limited setting

Choledochal cyst--a different disease in newborns and infants

Abstract We report experience of managing Choledochal Cyst (CC) in different paediatric ages. Eleven neonates and infants (aged 0-8 months) and 24 paediatric cases (aged 2.5 - 18 years) were managed over 24 years (1988 to 2012). Neonates and infants presented with jaundice, acholic stools and abdominal mass whereas most of the paediatric cases presented with intermittent non-specific abdominal pain. Morphology of CC was mostly cystic in neonates whereas it was fusiform in majority (62%) of paediatric cases. Biliary amylase was high and correlated with the presence of abnormal pancreaticobiliary junction (PBJ) in 20 /24 paediatric patients. Obstruction at the lower end of bile duct, liver fibrosis and cirrhosis were common in neonates. In conclusion, CC in newborns and infants is different and mimic correctable Biliary Atresia (BA). Early excision of CC and biliary reconstruction is promising in neonates, infants and children and it can be performed with minimal morbidity

Bacillus calmette-guerin (BCG) lymphadenitis: Changing trends and management

Background: Bacillus Calmette-Guérin (BCG) vaccination is performed as a part of expanded Programme of Immunization (EPI). Suppurative lymphadenitis is the common complication of BCG vaccination. The optimum treatment for this complication is debated.Methods: All cases of BCG lymphadenitis presenting to pediatric surgical service over a 17-year study period were reviewed to determine the optimum treatment for this problem.Results: Sixty children were referred for the management of BCG lymphadenitis during the study period. Most patients (n=43) presented during 1988 and 1990. Children were healthy and received BCG (Pasteur strain) vaccination at EPI during first 2 months of life. Initially all the patients received medical treatment with anti-tuberculous drugs for one to nine months. Fifty-two children who presented with large (approximately 3cm) fluctuant lymph nodes, required a surgical procedure to avoid spontaneous rapture and sinus formation (Group A), whereas medical treatment was effective in five out of eight patients presenting with small (approximately 1 cm) nodes (Group B). Conclusions: Medical treatment with antituberculous drugs is not effective to treat BCG lymphadenitis, when the involved lymph nodes are around 3.0 cm and have developed fluctuation and inflammation of over lying skin

Distal splenorenal shunt (DSRS) in children with extrahepatic portal hypertension

Portal hypertension (PH) is infrequent in children as compared to adults. Currently, repetitive endoscopic ablation of varices is standard treatment. However, endoscopic facilities are not readily available in resource-limited settings. We reviewed the indications, operative morbidity, mortality, shunt patency and re-bleeding episodes in nine patients (age range 6-16 years, M:F 6:3) who had DSRS for recurrent GI bleeding not responding to endoscopic treatment and hypersplenism. DSRS is a safe and effective treatment alternative in resource limiting setting for preventing bleeding and improving hypersplenism in children with extrahepatic portal hypertension

Formal specification and analysis of take-off procedure using VDM-SL

traffic management system is a complex adaptive and safety critical system which requires considerable attention for its modelling and verification. Currently Air traffic control (ATC) systems are heavily dependent upon human intervention at airport causing accidents and delays because of failure of communication. The purpose of this study is to develop, plan, manage and verify aircrafts movement procedures at the airport surface that prevent delays and collisions. The airport surface is decomposed into blocks and represented by the graph relation. The state space of the system is described by identifying all the possible components of the system. The ground and local controls monitor queues of the aircrafts moving from taxiway to take-off. It is insured that once an aircraft is inserted into a queue, it is eventually removed from it after the next queue has become available. The take-off procedure is provided using graph theory and Vienna Development Method Specification Language (VDM-SL) and analyzed using VDM-SL toolbox. Formal specification of graph-based model, taxiways, aircrafts, runways and controllers is provided in static part of the model. The state space analysis describing take-off algorithms is provided by defining optimal paths and possible operations in dynamic model expediting the departure procedure. The model is developed by a series of refinements following the stepwise development approach. The delays at airport surface require effective safety and guidance protocols to control air traffic at the airport. In static model, the safety criteria are described in terms of invariants over the data types carrying critical information. The safety is insured by defining pre/post conditions in description of operations for changing state space of the system. Although the proposed study is focussed more on the safety component, however, the efficiency is not ignored. Document type: Articl

Penile constrictive band injury

Penile strangulation caused by a thread or human hair is an uncommon cause of urethral injury described in paediatric piactice. Five children presented with this entity to the Aga Khan University Hospital, Karachi between August, 1991 and August, 1992. Three children had uneventful recoveries after removal of the contricting agent. Two patients developed urethrocutaneaus fistulae and partial amputation of the penis because of late presentation Early recognition and removal of the constricting agent is necessary to prevent serious complication

Maternal and fetal complications of antiphospholipid syndrome: a case report with long-term follow-up

We report a case of a 26- year old woman having antiphospholipid syndrome in pregnancy with long-term follow up. She presented with recurrent miscarriages, venous thrombosis, avascular necrosis of femoral head, mid-cerebral artery infarction and skin ulcers. Antiphospholipid syndrome is a recognized disorder of pregnancy. Diagnosis requires a high index of suspicion when evaluating women with recurrent pregnancy losses and vascular thrombosis. A low dose aspirin combined with heparin can reduce morbidity and improves the pregnancy outcome

Gastric teratoma: A rare benign tumour of neonates

We describe our experience of two neonates with gastric teratoma, one a low-birthweight, premature baby who had a massive, immature teratoma. Complete excision is the appropriate treatment