Posterior Sagittal Approach for Nonanorectal Malformation: Widening Spectrum.

Background: Posterior sagittal is a very well accepted approach in the treatment of anorectal malformations. This approach provides good access and exposure through the perineum to the deep pelvic structures. It reduces risk of injury to important structures as dissection remains in midline. Aims and Objectives: To access feasibility of posterior sagittal approach for non-anorectal malformation indications and to widen the spectrum. Material and Methods: We present a series of 10 cases of non-anorectal malformations operated by this approach for 4 years. Results: Six patients included in the study were of Disorders of Sexual Differentiation with pseudovagina, three of Y duplication of the urethra, and one of cervical atresia. All patients had good results. Conclusion: Posterior sagittal approach is feasible, safe with minimal bleeding, and no postoperative incontinence. It can safely be used for non-anorectal indications.

Initial experience with 3D laparoscopic choledochal cyst (CDC) excision and hepatico-duodenostomy (HD) in 21 children.

INTRODUCTION: Minimal access surgery has gradually become the standard of care in the management of choledochal cysts (CDC). Laparoscopic management of CDC is a technically challenging procedure that requires advanced intracorporeal suturing skills, and hence, has a steep learning curve. Robotic surgery has the advantages of 3D vision, articulating hand instruments making suturing easy and thus is ideal. However, the non-availability, high costs and necessity for large-size ports are the major limiting factors for robotic procedures in the paediatric population. Use of 3D laparoscopy incorporates the advantage of 3D vision and at the same time allows the use of small-sized conventional laparoscopic instruments. With this background, we discuss our initial experience with the use of 3D laparoscopy using conventional hand instruments in CDC management. AIM: To study our initial experience in the management of CDC in paediatric patients with 3D laparoscopy in terms of feasibility and peri-operative details. MATERIALS AND METHOD: All patients under 12 years of age treated for choledochal cyst in a period of initial 2 years were retrospectively analysed. Demographic parameters, clinical presentation, intra-operative time, blood loss, post-operative events and follow-up were studied. RESULTS: The total number of patients were 21. The mean age was 5.3 years with female preponderance. Abdominal pain was the most common presenting symptom. All patients could be completed laparoscopically. No patient needed conversion to open procedure or re-exploration. The average blood loss was 26.67 ml. None of the patients required a blood transfusion. One patient developed a minor leak postoperatively and was managed conservatively. CONCLUSION: 3D laparoscopic management of CDC in the paediatric age group is safe and feasible. It offers the advantages of depth perception aiding intracorporeal suturing, with the use of small-sized instruments. It is thus a 'bridging the gap' asset between conventional laparoscopy and robotic surgery. LEVEL OF EVIDENCE: Treatment study level IV.

An Unusual Case of Type A Posterior Cloaca Associated with 46XX Disorder of Sexual Differentiation with Y Duplication of Urethra.

We report an infant reared as a girl who presented with ambiguous genitalia and urine coming per rectum. On examination, she had minimal clitoromegaly with labial fusion and Y urethral duplication. On investigations, genetic, metabolic, and endocrine causes for disorder of sexual differentiation were ruled out. This girl was operated via a posterior sagittal approach. This case was unique in that the urogenital sinus was deviated posteriorly and opened in the anterior rectal wall and the accessory urethra opened in clitoris, along with ambiguity of external genitalia, thus making it a rare variant of the posterior cloaca. The diagnosis requires a high index of suspicion in clinician and a meticulous examination of the external genitalia. Very little literature is available for this rare anomaly. Few reports have classified them as female pseudohermaphroditism with cloacal and urogenital sinus defects. Hence, we hereby discuss and review the literature for previously reported cases.

Multidisciplinary Approach for Management of Pediatric Tumors: a Case Series.

Pediatric solid tumors have wide range of presentations. Multidisciplinary approach is often needed for their optimum management. There are no reports discussing such an approach to pediatric tumors involving pediatric surgeons, oncologists and cardiothoracic surgeons together for the management. We report 5 such cases being managed in our institution from 2010 to 2016. All cases needed chemotherapy followed by resection with a team of surgeons involving pediatric surgeon and cardiothoracic surgeon.

Posterior Sagittal Approach for Uterovaginal Anastomosis in a Case of Congenital Cervical Atresia with Anorectal Malformation.

Cervical atresia is a rare association with anorectal malformation (ARM) which can be missed till puberty in the presence of normal vaginal orifice. A 12-year-old girl operated for ARM in neonatal age presented with primary amenorrhea. She had a normal vaginal opening, short perineal body, and prolapsed anteposed anus and was diagnosed with cervical agenesis. As the posterior sagittal approach is standard to place the rectum in correct anatomical position, reconstruction of the anus along with adequate perineal body and uterovaginal anastomosis was performed through this approach. This report highlights the utility and versatility of this approach for the management of such complex cases.

Video-Assisted Thoracoscopic Surgery for Pediatric Empyema by Two-Port Technique: A Single-Center Experience with 167 Consecutive Cases.

BACKGROUND/PURPOSE: The aim of our study is to determine efficacy, safety, and feasibility of video-assisted thoracoscopic surgery (VATS) in childhood empyema with a technique of only two ports and open instruments at a tertiary care center in India. METHODS: This is a retrospective study of patients below 18 years, with empyema presenting to the Department of Pediatric Surgery of a Tertiary Care Referral Hospital in India, over a period of 9 years who underwent VATS decortication. Only two ports with open surgical instruments were used. The patients were assessed on the basis of mean duration of preoperative symptoms, duration of surgery, average blood loss, postoperative pain relief, complications, and need for redo surgery. RESULTS: A total of 97 patients underwent primary VATS decortications without inserting an intercostal drainage (ICD) tube and 70 patients as a secondary procedure after ICD tube was inserted. Mean duration of symptoms was 11 days. The average blood loss during surgery was estimated to be 170 cc. The mean duration of surgery was 90 min. The most common postoperative complication was air leak seen in 19.16% of patients. Minor leaks usually settled by 24 h. In eight patients, a negative suction had to be applied to the ICD tube for persistent air leak. The average length of postoperative stay was 4 days. Two patients required a repeat open decortication procedure due to nonresolution of symptoms and poor lung expansion after VATS. Patients had minimal pain and excellent cosmetic outcome after VATS. CONCLUSION: Two-port VATS decortication procedure is as feasible and effective as three-port procedure for decortication with better cosmetic result and pain relief.

Neonatal Sigmoid Colon Perforation: A Rare Occurrence in Low Anorectal Malformation and Review of the Literature.

Gastrointestinal perforation in neonates with anorectal malformation (ARM) is extremely uncommon. Delayed patient presentation is an important causative factor. A 2.5-kg neonate presented 72 hours after birth with abdominal distention and absent anal opening with meconium pearls. An abdominal X-ray revealed the presence of free gas. After adequate resuscitation patient underwent surgery. Closure of the sigmoid colon perforation with a proximal diverting loop colostomy with anoplasty was done. The literature reveals only two cases of sigmoid colon perforation with low ARM. Ours is the third case, in whom repair of the perforation and correction of the ARM was managed successfully at the same time.

OVOTESTICULAR DISORDER OF SEX DEVELOPMENT: A SINGLE-CENTER EXPERIENCE.

OBJECTIVE: Ovotesticular disorder of sex development (OT DSD) is a rare disorder of sex development characterized by the presence in the same individual of both histologically proven testis and ovary. There are scant data from the Indian subcontinent regarding this disorder. The aim of this study was to describe the clinical, biochemical, imaging, cytogenetic, surgical, and histopathologic findings and outcomes of patients with OT DSD from Western India. METHODS: The records of patients referred to our center for disorders of sex development between 2005 and 2013 were reviewed, and 7 patients were found to have histologically proven OT DSD. RESULTS: The median age at presentation was 8 years (range, 2 months to 25 years). Clinical presentation varied from genital ambiguity and inguinal swelling at birth to gynecomastia and cyclical hematuria after puberty. Karyotype was 46, XX in 6 patients and 46, XY in 1 patient. All patients underwent pelvic ultrasonography, laparoscopy, and surgery for removal of gonads not congruous with the chosen sex of rearing. Gender assignment for all the patients was done by the parents at birth, which was mainly influenced by the external genitalia and sociocultural influences, with 5 out of the 7 patients being reared as males. There was no evidence of gonadal tumors in our study. CONCLUSION: OT DSD should be considered as one of the differential diagnoses in cases of ambiguous genitalia with nonpalpable or asymmetrical gonads, pubertal gynecomastia, and cyclical hematuria, irrespective of the karyotype or internal genitalia.

Pyloric atresia--three cases and review of literature.

Pyloric atresia (PA) is a rare congenital anomaly that constitutes approximately 1% of all intestinal atresias, and its incidence is approximately 1 in 100,000 live births. PA may occur as an isolated condition or associated with other abnormalities, the most common being Junctional epidermolysis bullosa (JEB). Evidence suggests that PA-EB (Pyloric Atresia - Epidermolysis Bullosa) Syndrome is a distinct entity. In this report, we present three cases of pyloric atresia, one of which was associated with Junctional epidermolysis bullosa. The literature on the subject is also reviewed.

Two-port laparoscopic management of a giant pseudocyst complicating ventriculoperitoneal shunt.

Insertion of a ventriculo-peritoneal (VP) shunt is the most common operative procedure for the treatment of hydrocephalus in children. Of the several causes of shunt malfunction, cerebrospinal fluid (CSF) pseudocyst is relatively uncommon. There are several modalities to treat a CSF pseudocyst. Laparotomy is required, at times, more than once. We managed a patient of CSF pseudocyst with two-port laparoscopy, by deroofing the psuedocyst and repositioning of the shunt. This minimally invasive technique avoids morbidity associated with laparotomy and aids in early recovery.

To observe the intensity of the inflammatory reaction caused by neonatal urine and meconium on the intestinal wall of rats in order to understand etiology of intestinal damage in gastroschisis.

OBJECTIVES: The aim of this experimental study was to observe the intensity of the inflammatory reaction caused by neonatal urine and meconium on the intestinal wall of rats to better understand etiology of intestinal damage in gastroschisis. MATERIALS AND METHODS: A total of 24 adult Wistar rats were used as experimental models to simulate the effect of exposed bowel in cases of gastroschisis. The peritoneal cavity of the rats was injected with substances which constitute human amniotic fluid to study the effect on the bowel. Sterile urine and meconium were obtained from newborn humans. The rats were divided into four groups according to the material to be injected. In Group I (Control group) 3 mL of distilled water was injected, in Group II (Urine group) 3 mL of neonatal urine was injected, in Group III (Meconium group) 5% meconium suspension was injected, while in Group IV, a combination of 5% meconium suspension and urine was injected. A total of 3mL solution was injected into the right inferior quadrant twice a day for 5 days. The animals were sacrificed on the 6(th) day by a high dose of thiopentone sodium. A segment of small bowel specimen was excised, fixed in paraffin, and stained with hematoxylin-eosin for microscopic analysis for determination of the degree of inflammatory reaction in the intestinal wall. All pathology specimens were studied by the same pathologist. RESULTS: The maximum bowel damage was seen in Group II (Urine group) in the form of serositis, severe enteritis, parietal necrosis, and peeling. A lesser degree of damage was observed in Group III (Meconium group) as mild enteritis (mild lymphoid hyperplasia). The least damage was seen in Group IV (Combination of meconium and urine) and Group I (Control group). CONCLUSION: The intraabdominal injection of neonatal human urine produces significant inflammatory reactions in the intestinal wall of rats.

Should the ovary always be conserved in torsion? A tertiary care institute experience.

AIM: The aim of this study was to analyze our experience in conserving ovarian tissue in cases of ovarian torsion, irrespective of grade of necrosis at exploration. MATERIALS AND METHODS: All children with a diagnosis of ovarian torsion admitted to our hospital from January 2009 to January 2013 were included. Patients with underlying ovarian pathology were excluded. RESULTS: There were 13 torsions in 12 children (one bilateral). All underwent detorsion with or without evacuation of hematoma. Follow-up ultrasonography (USG) with color Doppler was done for all 13 ovaries, which showed an ovary with good vascularity and follicular development in 12 ovaries (92%). In 76% (10 of 13) of cases, intraoperatively, the ovary was judged to be moderately to severely ischemic/necrotic. Yet, follow-up sonograms showed the ovary with follicular development in all cases except one (7%). There were no major complications in our series. CONCLUSION: Simple detorsion, instead of traditionally advocated oophorectomy, was not accompanied by an increase in morbidity. On follow-up, almost all patients studied had functioning ovarian tissue despite the grave ischemia observed intraoperatively. Detorsion should be the procedure of choice for all cases of simple ovarian torsion in children.

Trocarless laparoscopic pyloromyotomy with conventional instruments: Our experience.

BACKGROUND: The incidence of hypertrophic pyloric stenosis is approximately 1-3 per 1,000 live births. Hypertrophic pyloric stenosis is seen more often in males, with a male-to female ratio of 4:1. Laparoscopic pyloromyotomy is becoming increasingly popular as the standard treatment for hypertrophic pyloric stenosis. MATERIALS AND METHODS: We describe our initial experience with laparoscopic pyloromyotomy in 16 infants using conventional laparoscopic instruments. Laparoscopic pyloromyotomy was performed through 5-mm umbilical port with 5mm 30 endoscope. Two 3-mm working instruments were inserted directly into the abdomen via separate lateral incisions. RESULTS: All patients were prospectively evaluated. The procedure was performed in 16 infants with a mean age of 36 days and mean weight of 3.1 kg. All procedures, except two, were completed laparoscopically with standard instruments. Average operating time was 28 mins, and average postoperative length of stay was 2.8 days. There were no major intraoperative and postoperative complications. CONCLUSION: Laparoscopic pyloromyotomy can be safely performed by using standard conventional laparoscopic trocarless instruments.

Meckel-Gruber syndrome: A rare and lethal anomaly with review of literature.

Meckel-Gruber syndrome is a rare autosomal recessive lethal malformation characterized by typical manifestations of occipital encephalocele, bilateral polycystic kidneys and post axial polydactyly. The worldwide incidence varies from 1 in 13,250 to 1 in 140,000 live births. Highest incidence was reported in Gujarati Indians. We report a rare case of Meckel-Gruber syndrome and review of literature.

Biliary fascioliasis: Management in a child using endoscopic retrograde cholangio pancreatography.

A 9-year-old boy presented with right upper abdominal pain and fever. The radiologic investigations revealed multiple cholangitic abscesses with cholangitis and worms in common bile duct. Endoscopic retrograde cholangio pancreatographic (ERCP) sphincterotomy, basketing, ballooning and extraction of Fasciola hepatica worms from the common bile duct were done.

Solid pseudo papillary tumor of the pancreas: An unusual tumor in children.

An 11-year-old girl with a pancreatic solid pseudo papillary tumor (SPT) is reported. Contrast enhanced computerized tomography (CECT) & Magnetic resonant imaging (MRI) abdomen revealed a well-defined inhomogenous mass arising from the retro-peritoneum in the left upper quadrant of the abdomen possibly from the tail of the pancreas. USG guided biopsy of tumor showed benign pseudo papillary tumor of pancreas. Complete excision of the tumor was carried out. Histopathology revealed pancreatic pseudo papillary tumor. Patient did well postoperatively & is on regular follow-up.

Minimal access surgery in newborns and small infants; five years experience.

AIMS AND OBJECTIVES: The aim of this study was to assess and present the outcome (initial experience and lessons learnt) of minimally invasive surgery for various indications in neonates and small infants (< 5 kg) at a single medical centre. MATERIALS AND METHODS: A retrospective analysis was performed on 65 patients (age day 2 to 10 months) managed with minimal access surgery (MAS) for various indications, between 2005 and 2010. We analyzed demographic information, procedures, complications, outcomes, and follow-up and overall feasibility of the procedure. RESULTS: No serious complications except one death in congenital diaphragmatic hernia (CDH) (due to other comorbidities) occurred. Intra operative hypercarbia and hypoxia were observed more frequently in thoracoscopic procedures. Intra operative hypothermia was not common and was well tolerated. Conversion to open procedure (n = 5), post operative ileus (n = 3), port site infection (n = 5) were other complications. CONCLUSION: MAS in neonates and small infants is a technically demanding but a feasible choice available. Some prior experience in older children is required for safe and effective outcome. Good quality optics, video equipments and instruments are required for safe and effective procedure. Intra operative measurement of oxygen saturation and temperature, and diligent post operative ICU care are mandatory for safe and successful outcome.