Gursev Sandlas

Outcome analysis of shunt surgery in hydrocephalus

Aim: To study the clinical outcome of shunt surgeries in children suffering from hydrocephalus. Methods: A prospective study of 50 children with hydrocephalus who underwent a ventriculo-peritoneal shunt insertion over a period of two years. These patients were then followed up for shunt related complications, shunt revisions and outcome. Results: Twenty six of the 50 patients (52%) suffered from complications. The most common complications were shunt blockage (n=7) and shunt infection (n=6). These complications necessitated repeated shunt revisions. Conclusions: Infective complications of hydrocephalus are more likely to leave behind an adverse neurological outcome in the form of delayed milestones and mental retardation.

Acute acalculous cholecystitis causing gall bladder perforation in children

We report two cases of children who presented with acute abdomen due to gall bladder perforation and biliary peritonitis. Cholecystectomy with peritoneal lavage proved curative.

Intra-abdominal cystic lymphangiomas in children: A case series

Background: Lymphangiomas are hamartomas of lymphatic vessels commonly arising in the head, neck, and axilla. Intra-abdominal cystic lymphangiomas are rare and are located in the retroperitoneum, the mesentery, omentum, or visceral organs. Radiological imaging in the form of ultrasound and computed tomography (CT) scan helps in preoperative diagnosis. The objective of this study is to present the management of children with intra-abdominal cystic lymphangioma in our institution. Materials and Methods: Six cases of intra-abdominal cystic lymphangiomas comprising three males and three females, which presented over 15 months, were studied. Clinical presentation, location, mode of surgical intervention, and outcome were studied. Results: The site of origin was retroperitoneum, omentum, and the mesentery, with two cases of each. Age of presentation ranged from 5 days to 7 years. Abdominal lump was the most common presenting feature. All the patients underwent complete surgical resection - four had open excision, one had a laparoscopy converted to open excision, and one had a complete laparoscopic excision. Postoperative recovery was uneventful in all of them, with no recurrence at 6 months follow-up. Conclusion: Intra-abdominal cystic lymphangiomas in children are usually symptomatic. CT scan is more accurate than ultrasound to diagnose the lesion. Complete excision of the lesion with or without intestinal resection provides symptomatic relief and prevents recurrence.

Spontaneous Bowel Perforation in a Neonate with Anorectal Malformation

Gastrointestinal perforation in neonates with anorectal malformations is extremely uncommon. Delayed patient presentation is an important factor that demands special attention. We present a neonate with anorectal malformation and meconium peritonitis following spontaneous bowel perforation. A day 1 neonate was referred with features suggested of peritonitis. After adequate resuscitation and drainage under local anesthesia, patient was successfully operated for a sigmoid perforation and is now awaiting definitive surgery for the anorectal malformation.

Bleomycin: A worthy alternative?

Context: Lymphangiomas are developmental anomalies presenting mainly in the first two years of life. Surgical excision has been the mainstay of treatment; however a potentially disfiguring surgery along with presence of important structures in the vicinity and infiltration into surrounding structures makes the dissection difficult. Aims: To study the safety and efficacy of Bleomycin as a sclerosing agent for lymphatic malformations in children. Settings and Design: Prospective non comparative nonrandomized trial. Materials and Methods: The study was carried out in 15 children between Day 5 of life to 12 years of age who presented between May2008 to May 2009. Bleomycin aqueous solution was injected intralesionally at a dose not exceeding 0.6 to 0.8 mg. /kg Body wt. The response to therapy was monitored clinically by measuring the length, breadth and area as well as by measuring the two largest perpendicular dimensions. The response was graded as excellent [total disappearance], good [>50% reduction] and poor [<50% decrease]. Those patients with diffuse lymphangiomas associated predominantly with hemangiomatous malformations, mediastinal, spinal or retroperitoneal extensions, visceral lymphangiomas, those with infections were excluded from the study. Statistical analysis used: None applicable. Results: The reduction in the size of the mass usually took between two weeks to ten months. The average duration of follow up has been ten months. A significant response was seen in 8 out of the fifteen [53.33%] patients. 5 patients [33.33%] patients showed a good response to therapy and achieved >50% reduction in the size of their swellings. 2 patients [13.33%] showed a poor response to therapy and achieved less than 50% reduction in the size of the swelling. Complications of the therapy were few and far between. 2 patients developed fever after injection, one patients reported a transient increase in size of swelling, 2 patients have developed discoloration of the overlying skin and are currently being followed up for final outcome. None of the patients developed leucopenia or leukocytosis. All of the complications were managed with conservatively. Patients are on long term follow up to evaluate long term effects, if any.

Post meningocele repair urinary ascites in a neonate—a rare presentation ☆

A full-term female neonate with a large lumbosacral meningocele developed sudden abdominal distension and urinary retention after meningocele repair. An erect abdominal radiograph showed homogeneously opacified areas with a paucity of intestinal gas. Abdominal ultrasound revealed ascites with multiple internal echoes. With a provisional diagnosis of hollow viscus perforation, an emergency laparotomy was performed, which failed to reveal any leak from the urinary tract. However, fluid analysis confirmed the diagnosis of urinary ascites. The patient developed repeated episodes of urinary retention after catheter removal. She was started on clean intermittent catheterization (CIC) to ensure adequate bladder drainage. At 6 months of follow-up, ultrasound of the urinary tract, voiding cystourethrogram, and magnetic resonance imaging of the spine were all within normal limits. The CIC was discontinued, and the patient was observed. At present, she is voiding normally with a good stream. Failure to establish normal micturition after meningocele repair and CIC requirements suggested a neurogenic cause. Bladder rupture, secondary to spinal shock resulting in bladder atonia, could not be ruled out. The perforation (leading to urinary ascites) could be owing to bladder atonia and spinal shock rather than detrusor sphincter dyssynergia.

Anterior urethral valves without diverticulum, a rare cause of infravesical obstruction and vesicoureteral reflux in children: Report of two cases and literature review

Congenital anterior urethral valve is a rare condition causing significant obstructive uropathy in pediatric age group. It is much rarer than posterior urethral valve. However, the clinical course is similar. We present two cases of anterior urethral valves in children.

Aphallia (Penile agenesis): A preliminary report of three cases

Aphallia (penile agenesis) is an extremely rare abnormality with the reported incidence of 1 in 30 million births. The cause of this anomaly is associated with no genital tubercle formation or its development impairment. The majority of patients have 46XY Karyotype. The scrotum, testes and testicular function are usually normal. We report the preliminary experience with 3 cases of aphallia in different age groups along with a review of the literature.

Congenital rectovaginal fistula with anorectal agenesis: A rare anorectal malformation

Background Rectovaginal fistula is a rare type of anorectal malformation; the incidence being less than 1%. We describe five cases of rectovaginal fistula managed at our institution. Materials and methods Case records of five female neonates with rectovaginal fistula managed at our institute between 2010 and 2016 were reviewed and analysed with respect to age at presentation, clinical presentations, physical findings, investigations, management and outcome. Results The age at presentation varied from 1 day to 2 years of age. Three of them presented in the neonatal period, one presented at 1 month of age and one at two years of age with sigmoid loop colostomy done elsewhere. All had absent anal opening; two neonates passed small amounts of stools through vagina, but little in amounts. The one-month old patient had history of passing stools through vaginal orifice, but had presented to us with obstruction. All patients underwent high sigmoid loop colostomy followed by definitive procedure at a later date – Posterior Sagittal Anorectoplasty. One patient is awaiting definitive repair. Conclusion Rectovaginal fistula is a rare anorectal malformation and needs thorough investigation and appropriate management for good outcome.

Single-stage Modified Duhamel procedure for Hirschsprung's disease : Our experience

INTRODUCTIONnPrimary single-stage pull-through for Hirschsprungs disease (HD) has been reported to give comparable surgical outcomes to staged operations with less morbidity. Herein, we present our experience with single-stage Modified Duhamel procedure for management of HD.nnnPATIENTS AND METHODSnThis was a review of 48 cases of HD who underwent single-stage Modified Duhamel procedure without a protective colostomy.nnnRESULTSnThe age at surgery ranged from 6 months to 10 years (median - 9 months, mean - 2.3 years). The average weight of the child was 7.2 kg (range, 4.9-22 kg). 38 (79.2%) patients had classical rectosigmoid HD, the rest being long segment HD (the proximal most level being the splenic flexure). The average duration of surgery was 175 minutes (range, 130-245 minutes). The average blood loss was 45 ml. The average hospital stay was 7.2 days (range: 6-10 days). The major postoperative complications (n=3) included postoperative adhesive intestinal obstruction, anastomotic leak and persistent constipation due to residual aganglionosis. Each required a re-exploration. Minor complications included surgical site infection (n=3) and post-operative enterocolitis (n=3), which were managed conservatively. Six patients had constipation for a limited period post-operatively. All patients have a satisfactory functional outcome and normal development and growth.nnnCONCLUSIONSnFor HD, we recommend that single-stage Modified Duhamel procedure should be the preferred approach in view of its low morbidity, satisfactory functional outcome and avoidance of stoma, multiple surgeries and economic benefit in view of decreased hospital stay.