Low lying umbilicus in a neonate with anomalous position of penis and anus

A healthy thriving neonate was brought with the complaint of passage of stool like material from glans penis. In this healthy baby umbilicus was found to be placed caudally 4cm above symphysis pubis. The penis was located away from scrotum and anus was anteriorly placed with mucosal protrusion. There were no complaints related to passage of urine and stool. Extensive investigations revealed no associated anomaly and any communication between urinary tract and anorectal canal. Reassurance was given to the family and advise for regular follow up given

Hirschsprung's disease: pattern of clinical presentation

To report clinical symptoms and signs and age at initial presentation of patients with Hirschsprung's disease. Descriptive case series. Department of Paediatric Surgery, National Institute of Child Health Karachi, From September 2008 to December 2013. Patients who were proven cases of Hirschsprung's disease on rectal biopsy report were included. The data was collected prospectively. Variables recorded included clinical presentation including symptoms, signs, age, gender, mode of presentation either elective or acute etc. The data was analyzed using descriptive statistics like frequency, percentage and proportions in relation to quantitative and qualitative variables. A total of 84 patients with Hirschsprung's disease were managed during the study period. There were 63 male and 21 female patients with male to female ratio of 3:1. Fifty-five [65.5%] patients belonged to Karachi. Fifty percent [n=42] patients presented by day 6 of life. The commonest presentation was delayed passage of menonium but it was reported in 39 [46.4%] patients only. All these patients were operated and extent of the disease was up to rectosigmoid level in 78 [93%] patients. Clinical presentation in present study had similarities to reported literature but important differences were noted with regards to passage of meconium and extent of aganglionosis

Neonatal duodenal obstruction: clinical presentation and outcome

To find out causes, modes of presentations and surgical outcome in neonates with duodenal obstruction. Descriptive case series. Department of Paediatric Surgery National Institute of Child Health [NICH] Karachi, from September 2011 to August 2013. All neonates presenting with duodenal obstruction were included. Age at presentation, symptoms and signs, associated anomalies, treatment provided and outcome were recorded. Investigations done included CBC, serum electrolytes, urea / creatinine, x-ray abdomen and echocardiography. Surgical procedures were tailored according to the cause of obstruction. Data was recorded on a performa. Analysis was done using SPSS version 17. A total of 17 patients [males 13, females 4] were managed. Age ranged from 1 day to 20 day [mean=4 day]. Weight of the babies ranged from 1.5 kg to 3.0 kg [mean=2.24 kg]. One patient was delivered via cesarean section. The causes of obstruction were duodenal atresia [n=8], annular pancreas with duodenal atresia [n=4], annular pancreas with duodenal atresia and malrotation [n=3] and only malrotation [n=2]. Double duodenal atresia was present in one patient. Associated imperforate anus was found in five cases, multiple atresias of gut and jejunal atresia in one case each. Three patients had Down syndrome. All patients were operated. Two patients had anastomotic leak. A total of seven patients survived. Those who expired had duodenal atresia [n=5], annular pancreas group [n=4] and malrotation [n=1]. Sepsis was the major contributor to the mortality. Late presentation with low birth weight were the salient observations. Multiple associated gut related surgical anomalies added to operative stress. High mortality [59%] was of concern and sepsis remained the major contributor

Surgical biliary tree pathologies in paediatric patients

To find out various pathologies affecting biliary tree in paediatric age group and mode of investigations performed to arrive at diagnosis. Descriptive case series. Department of Paediatric Surgery Unit B, National Institute of Child Health Karachi, from January 2007 to December 2007. All paediatric patients below the age of 12 years who presented with symptoms related to biliary tree were included. A detailed history was taken including presence of jaundice, color of stool, mass an pain in right upper abdomen etc. all wee subjected to ultrasound as a screening test. Laboratory investigations included blood complete picture, liver function tests including bleeding profile, TORCH titre where appropriate, HIDA scan and CT scan as indicated. Laparoscopy was done in cases with persistent jaundice for operative cholangiogram and liver biopsy. Surgical procedure was tailored according to the pathology found. A total of 18 patients were managed. The number of male and female patients was nine each. The ages of the patients ranged from one month to nine years. Most common pathology was biliary atresia [n7]. Eleven patients presented wit persistent jaundice with age range from one month to 18 months. In all them HIDA scan showed failure of visualization of excretion of contrast into duodenum. All underwent laparoscopy and in seven of these biliary atresia found. There were four patients in whom extrahepatic biliary tree was found patent on cholangiogram. They were in older age range [from three months to 18 months]. All had grossly cirrhotic liver. Three patients had choledochal cyst [two males and one female]. The age ranged from 1 1/2 month to 9 year. A female of 2 1/4 year presented with history of fever and failure to thrive. Ultrasound showed presence of gall stones. Cholecystectomy was done in this child. Three patients had common bile duct [CBD] pathology. All had history of fever, recurrent jaundice with dilated CBD on ultrasound. All had CBD stone/ sludge. In two choledochotomy and removal of stone/ sludge was done while other had cholecystectomy in addition because of inflamed gall bladder. No T tube was places. One patient with idiopathic variety of gall stone had cholecystectomy. Ultrasound in expert hands is a good screening test in patients suspected of having surgical biliary tree pathology. HIDA scan can be safely omitted from list of investigations and laparoscopy is recommended in all cases of prolonged neonatal jaundice where surgical pathology is suspected

Anterior sagittal approach for anorectal malformations in female children: early results

To determine the technical applicability and early postoperative outcome of anterior sagittal approach for anorectal malformations in female children. Study Design: Case series. Place and Duration of Study: Surgical Unit B of National Institute of Child Health [NICH], Karachi, from April to November 2007. Female patients with congenital anorectal malformation who underwent anorectoplasty through anterior sagittal approach were included in the study. Surgery was done either as primary or staged procedure [with initial colostomy or cut back]. Operative details were recorded. Follow-up was done in OPD. Thirty patients with mean age of 11.5 months underwent anorectoplasty through anterior sagittal approach. Eighteen patients had ASARP as a primary procedure. Staged procedure with initial colostomy was done in 9 patients. Initial cut back was done in 2 cases and one redo surgery done. Vaginal tear occurred in one, while partial tear of most distal part of fistula occurred in 4 children. At follow-up, 2 patients with primary ASARP developed wound infection with superficial disruption. Bleeding with wound disruption occurred in one case. Anal mucosal prolapse, anastomotic stricture and recurrent fistula occurred in one patient each. Cosmetic appearance of perineum was good in 10, satisfactory in 5 and poor in 3. Among patients staged with colostomy, bleeding with wound disruption, anal stenosis and retraction occurred in one case each. Cosmetic results were good in 7, satisfactory and poor in one case each. Two patients with initial cut back did not have any complication and one operated for disrupted wound developed disruption again. Anorectoplasty can satisfactorily be done through anterior sagittal approach in females with anorectal malformations. Primary ASARP has almost the same results as staged procedure, which should be done in selected patients

Video assisted thoracoscopy in children

To determine the outcome of video-assisted thoracoscopic surgery [VATS] in terms of its efficacy, safety and usefulness in children. Case series. Department of Paediatric Surgery Unit B, National Institute of Child Health, Karachi, from May 2006 to December 2006. This study was carried out on patients who were admitted with various intra thoracic pathologies and had video assisted thoracoscopy. Total of 13 patients underwent VATS. There were 6 patients of empyema thoracis who underwent thoracoscopic decortication and done successfully. There were four patients of hydatid disease among which three were converted into open thoracotomy. Two patients were of mediastinal mass and one of bronchopleural fistula. In these patients only biopsy was done which was conclusive in one patient, who was diagnosed as having ganglioneuroma. In other biopsy was inconclusive. Biopsy report was that of tuberculosis in patient with bronchopleural fistula. Video-assisted thoracoscopy is a safe and effective diagnostic and therapeutic procedure in children and this new approach has an important place in pediatric thoracic surgical practice. Thoracoscopic decortication can be treatment of choice for early empyema thoracis. As the learning curve progresses, more and more procedures can be done by VAT

Posterior urethral valves: varied presentations and outcome after preliminary surgical treatment

To find out various modes of presentation and outcome after preliminary surgical treatment of posterior urethral valves in pediatric population. Surgical Unit B National Institute of Child Health, Karachi, during the years 2004-2005. All consecutive patients coming in outpatient / emergency departments, diagnosed as having posterior urethral valves and those who had received initial treatment and came for follow up, were included in the study. All the neonates underwent vesicostomy initially, followed by valve ablation at appropriate age with plan for undiversion at later date. In infants valve ablation was performed if urethral size admitted pediatric cystoscope while older children underwent primary valve ablation. There were total of 22 patients managed during the study period. They included 5 neonates [0-1 month], 11 infants [1-12 months] and 6 older children [1year-12 years]. The main presenting complaint in majority of neonates was inability to pass urine and one had urinary ascites. Infants presented mainly with either difficulty in passing urine [n 5] or dribbling and acute urinary retention in 2 patients each. Majority of older children [n 3] presented with poor urinary stream and dribbling while two had diurnal enuresis and one came with straining at micturition. In all the neonates' vesicostomy was performed. Out of these 3 received valve ablation therapy, followed by undiversion. Two of them improved while 1 had bladder dysfunction. Of the remaining 2 patients with vesicostomy 1 is still waiting for definitive procedure while the other is lost to follow up. Of infant group, 8 were subjected to vesicostomy and valves ablated at later stage whereas 2 received primary valve fulguration therapy. One patient of this group died before any intervention performed. In older age group, 3 received valve ablation as initial treatment while 3 were lost to follow up before any intervention. Outcome assessed clinically, biochemically and radiologically. Out of total 22, four had not received any intervention. Of the remaining 18, treatment completed in 12 patients, 8 of these improved and 4 had residual disease. Two patients expired and 4 were lost to follow up. The overall improvement rate observed at the end of our study was 44%. In 22% patients there were still residual ongoing problems that need to be addressed on long term basis

Prenatal counseling and informed consent: ethical dilemma

With advancement in technology, congenital anomalies are increasingly recognized on antenatal ultrasound with great accuracy. This has opened up an entirely new field where all those who care for expectant parents and prospective baby like obstetricians, neonatologists, pediatric surgeons, psychologists, social workers etc are required to prove their expertise to deal with psychological issues and be able to guide expectant couple and their families as to what to expect and approach the baby born with congenital anomaly. If some life threatening condition is recognized at anomaly scan the decision of termination of pregnancy is even more difficult to make. This report describes an experience gained in dealing with one such couple whose baby on antenatal ultrasound, well beyond the age of termination of pregnancy, found to have omphalocele major. Many counseling sessions were held but the desired result was not achieved. How such problems should be dealt with in future, is discussed

Childhood nephrectomies for benign renal lesions

To conduct an audit of nephrectomies done in children for benign renal pathology. Evidence based study. Place and Duration: National Institute of Child Health, Karachi, during the year 2004-2005. The record of all the paediatric age group patients undergoing unilateral nephrectomy for benign renal diseases over a 2-year period were reviewed for the underlying diagnosis and indication for nephrectomy. Patients of Wilms` tumor subjected to nephrectomy were excluded. In the study nephrectomy was carried out in a total of 12 patients. There were 8 males and 4 females. The age range was 4 to 12 years. Nephrectomy was performed for renal tuberculosis in 5, pelvi-ureteric junction obstruction [PUJO] in 5, congenital hypoplastic kidney in 1 and one for multicystic dysplastic kidney. All the patients following nephrectomy remained well in the immediate post operative period and thereafter. Late presentation and delay in diagnosis of benign lesions of the kidney remained the main culprit in loosing a vital organ in childhood

Surgical management of renal tumors in children

To analyze case records of children with renal tumors. Case series. Surgical Unit B, National Institute of Child Health, Karachi during years 2004-2005. Case records of all patients managed during the study period were reviewed for clinical presentation, investigations and surgical management. National Wilms' Tumor Study. Group [NWTSG] and International Society of Pediatric Oncology [SIOP] protocols were used depending upon stage in cases of Wilms' tumor. Trucut biopsy was done for tissue diagnosis as proposed by UK Children's Cancer Study Group [UKCCSG]. Touch imprints were also made. Surgical procedure was analyzed in terms of ease of dissection, tumor spillage and extent of excision. Twenty-one patients of renal tumors were managed in two years period. Nearly 60% of patients were less than 2 years of age. Majority [n 14] presented with abdominal mass. Few had complaints of abdominal pain. One patient an infant, presented with profuse hematuria. Trucut biopsy was done in 18 cases to have tissue diagnosis. Three patients underwent primary exploration. There were 17 cases of Wilms' tumor and in one case it was suspected on touch imprints. Fourteen patients of Wilms' tumor were given pre operative chemotherapy [SIOP protocol]. Twelve of them were in stage III and IV. Nine out of this have undergone nephrectomy. Marked tumor regression in size of tumor was noted. The tumor also became firm. Only one tumor ruptured during excision. In one tumor with horse-shoe kidney, residual tumor left at margins of dissection. Three patients underwent primary nephrectomy. Two of these were in stage I and one in stage III [NWTSG protocol]. Patient in stage III died in immediate post operative period because of hemorrhage. There was one case each of mesoblastic nephroma, cystic nephroma and rhabdoid tumor. In all these nephrectomy was done following trucut biopsy. Patient with rhabdoid tumor received pre operative chemotherapy. This tumor ruptured during surgery and gross spillage occurred. Wilms' tumor was the most common pediatric renal tumor. Most of the patients were younger than the reported age and presented with advanced stage of disease. SIOP protocol found more appropriate in our group of patients

Renal hydatid cyst: an isolated involvement

Hydatid cyst can occur in any part of the body. Isolated involvement of viscera other than liver and lung is rare In this report we present a case of seven years old male child who had an isolated involvement of left kidney with hydatid cyst. CT scan showed multiple cysts of left kidney with no renal function. His indirect hemagglutination [IHA] test showed a value of 1:8192. He was initially put on albendazole to which he showed partial response. At surgery multiple hydatid cysts were removed from kidney. Post operative radio isotope renal scan revealed the function of 15%

Neonatal intussusception: a rare entity

Intussusception is a common cause of intestinal obstruction in infancy. It has been reported in pre-matures and during fetal life as well. In full term neonates it is a rare entity. In this case report we describe our experience of management of a six days old male baby. Who turned out to be a case of idiopathic ileoileal intussusception. As the gut was non viable, resection and anastomosis were performed. Post operative recovery were uneventful