A Novel Homozygous AMRH2 Gene Mutation in a Patient with Persistent Müllerian Duct Syndrome.

Persistent müllerian duct syndrome (PMDS) is characterized by the presence of müllerian duct derivatives in otherwise phenotypically normal males. Homozygous or compound heterozygous alterations in AMH or AMHR2 have been identified in approximately 88% of PMDS cases. We report on a male patient with bilateral undescended gonads, müllerian derivatives, and normal serum AMH levels. A novel homozygous missense mutation, c.119G>C;p.Gly40Ala, in exon 2 of AMHR2 was detected that supported the clinical diagnosis of PMDS.

A safer way of suturing in Foker's technique.

BACKGROUND/PURPOSE: Foker's technique allows esophageal lengthening facilitating end to end anastomosis in long gap esophageal atresia. The problem faced with this technique is that the traction sutures cut through the tissues leading to re-operations. Our aim was to find a technique of suturing that will prevent the sutures from cutting through the esophagus. METHODS: After dissection of the upper and lower esophageal pouches, purse string sutures were placed, two each on both pouches. Clips were applied at the ends of both the pouches. Sutures were brought out on the posterior chest wall and traction applied. This was tried in a total of three cases. Case 1 was a newborn with pure esophageal atresia, Case 2 was an eighteen month old child with cervical esophagostomy and gastrostomy, and Case 3 had esophageal atresia with distal fistula. Two cases were done thoracoscopically and the third one by thoracotomy. RESULTS: In all three cases sutures held and lengthening could be obtained. In the first case it took twelve days, in the second case six days, and in third case eight days for the ends to come together. CONCLUSION: This modification of traction sutures is simple and reduces the risk of suture disruption.

Laparoscopic inguinal hernia repair in children: a single-centre experience over 7 years.

BACKGROUND: To evaluate 209 consecutive children who underwent laparoscopic hernia repair over a 7-year period. Technical details and clinical results are reported. MATERIALS AND METHODS: A total of 284 open internal rings were closed laparoscopically in 209 children (142 boys and 67 girls, aged 30 days to 15 years, mean 44 months). One 5-mm and two 3-mm instruments were used to access the peritoneal cavity. A 270° anterolateral peritoneal incision was made. The internal inguinal ring was closed with a nonabsorbable suture. RESULTS: There were no significant intraoperative complications. Length of procedure ranged between 15 and 65 minutes with a mean of 30 minutes. Postoperative hospital stay ranged from 1 to 9 days. Thirty children who presented with a right-sided hernia and 23 with a left hernia (total of 53) were found to have a patent contralateral internal ring on laparoscopy. Mean follow-up was 30 months. There were 2.4% hernia recurrences and cosmesis was excellent. CONCLUSIONS: Laparoscopic hernia repair in children can be a routine procedure with increasing experience and better learning curve of surgeons. There is clear visualization of structures and vas remains untouched. The recurrence rate is comparable to that of the traditional open approach with a superior cosmetic result.

Intravesical pressure: A new prognostic indicator in congenital diaphragmatic hernia.

AIMS: To evaluate the usefulness of intravesical pressure as a prognostic indicator in congenital diaphragmatic hernia. MATERIAL AND METHODS: In 25 cases, bladder pressure was measured intraoperatively during repair. RESULTS: Cases were divided into three groups according to the intravesical pressure. Group 1: pressure <10 cm (n.9), Group 2: 10-15 cm (n.11) and Group 3: >15 cm (n.5). Number of ventilated days was tabulated against these groups. Median number of ventilated days for Group 1, with the lowest pressure, was 3 days, while that for Group 2 was 5 and for Group 3, with the highest pressure, was 10 days. This was significant, with a P-value of 0.016. CONCLUSION: Measurement of intravesical pressure is a reliable prognostic indicator in newborns with congenital diaphragmatic hernia. It also helps in predicting postoperative ventilatory requirement.

A simple and safe technique for trocar positioning in vesicoscopic ureteric reimplantation.

PURPOSE: Anchoring of the urinary bladder to the anterior abdominal wall is essential to prevent perivesical gas leak and dislodgement of trocar. Existing techniques have the risk of going through the peritoneal cavity and injuring the bowel. Our aim was to find a safe technique to anchor the bladder wall to the abdominal wall. MATERIALS AND METHODS: Nineteen cases were studied prospectively. A curved urethral dilator was introduced and made to point on the anterior abdominal wall. Abdominal wall was incised over the tip of the dilator till the bladder was seen which was then sutured to the anterior abdominal wall. The bladder was then incised and the trocars were railroaded on the dilator into the bladder. Cohen's reimplantation was then done. RESULTS: Out of the 19 cases, conversion to open was done in two early cases. In one case, the trocar got pulled out, but was easily reintroduced. Mean operating time was 210 min. Mean follow up was 20.9 months. There was a resolution of reflux in 13, reduction in the grade of reflux in 1 and no failures. In five cases, post op MCU is awaited. CONCLUSION: This technique of fixing the bladder to the anterior abdominal wall is safe and easy to perform. It is recommended while doing vesicoscopic reimplantation.

Large inguinal hernia in infants: is laparoscopic repair the answer?

PURPOSE: Infants with large hernias present a challenge to the pediatric surgeon. We present our experience of laparoscopic repair of large hernias in 16 infants, the youngest aged 40 days. Fifteen of the infants were younger than 18 months and one was < or = 2 years old. MATERIALS AND METHODS: Sixteen male infants with large inguinal hernias (hernia extending to the bottom of the scrotum and the diameter of the open internal ring > 2 cm on laparoscopy) underwent laparoscopic hernia repair. Diagnostic laparoscopy was performed under general anesthesia with a telescopic port at the umbilicus. If the internal ring was open, two working ports were introduced pararectally on either side. An open internal ring was considered an indication for hernia repair. The needle, carrying 3-0 nylon nonabsorbable sutures, was introduced through the groin skin at the internal inguinal ring and the internal ring was closed by taking continuous sutures to approximate the edges of the ring. The needle was then taken out through the entry point and the knot was tied extracorporeally and buried in the subcutaneous tissue. In 4 patients in the initial part of the study, intracorporeal (knot tied internally) suturing was done to close the internal ring. A contralateral repair was done if the internal ring was open. RESULTS: A total of 18 repairs were performed in 16 patients: 10 had a right sided hernia, 4 had a left sided hernia, and 2 had an open contralateral ring (suggestive of contralateral patent processus vaginalis). The contents of the hernia were bowel (1 patient), omentum (3 patients), and bowel adhesions at the internal ring (1 patient). There was 1 conversion. The mean operative time was 23 minutes for unilateral repair and 29 minutes for bilateral repair. The mean follow-up was 19 months (range, 3 months to 3 years). There are no recurrences to date. There was no morbidity or mortality. CONCLUSION: Laparoscopy is safe and feasible for repair of large inguinal hernias in infants 2 years and provides treatment of contralateral patent processus vaginalis in the same setting with no significant increase in operating time. Extracorporeal knot tying has made the procedure feasible in the limited working space available in these babies. Though there were no recurrences or post-operative hydrocele, the number of patients in the study is too small to comment on rates of recurrence or hydrocele.

Primary pulmonary hydatid cysts in children-a report of three cases.

Three children with a total of six primary pulmonary hydatid cysts, all of whom underwent surgical management, are presented. Of these six hydatid cysts, two were treated with thoracotomy with cyst enucleation and capittonage, three with thoracoscopy-assisted minithoracotomy with enucleation and capittonage, and one with limited resection.