Management of Cervix Atresia with Hematometra by "Genitoscopic Ultrasound-Guided Cervix Fenestration and Balloon Dilatation": A Novel Approach.

Introduction: Cervix atresia causing hematometra usually presents at puberty with cyclic abdominal pain. The management depends upon the type and severity of cervix atresia. We present a novel technique of managing a case of cervix atresia in a 13 years old girl. Case report: A 13 years old girl presented with severe cyclic abdominal pain. Examination revealed normal external genitalia with a palpable tender mass in the suprapubic area. Ultrasonography and MRI suggested a large hematometra with a 1.5 cm long cervix atresia. Vaginoscopy performed under anesthesia showed a normal-looking vagina and a blind cervix. During vaginoscopy and dynamic ultrasound (US) guidance, a spinal needle was passed through the vagina and center of the atretic cervix into the uterine cavity containing the hemolyzed blood. Aspiration of the hemolyzed blood and US visualization confirmed the location of the needle in the uterine cavity. A three-stage (esophageal) balloon dilator was passed over a guidewire through the atretic area. The balloon dilator was dilated up to 10 mm using pressure-controlled dilatation. A satisfactory dilatation was achieved, and all the retained hemolyzed blood was drained through the newly created channel. A size 24 Foleys catheter was retained in the uterine cavity and removed after 1 week. A repeat ultranography before subsequent periods showed a recurrent collection and obliteration of the cervix. US-guided balloon dilatations were repeated. A third dilatation was required after an interval of few months. The child remained well after the third dilatation, having regular periods, and is symptom free at 8 months follow-up, however, may need more sessions of cervix dilatations. Conclusion: Genitoscopic US-guided cervix fenestration and balloon dilatation is an effective technique for treatment of cervix atresia. It is a safe technique, avoids major surgical reconstructions, is a minimally invasive procedure, and can be used in selected cases of cervix atresia.

Planned Second look laparotomy in neonatal volvulus - A safe approach for bowel salvage.

Midgut volvulus can result in gangrene and loss of large segments of intestine. After correction of volvulus the viability of intestine may improve and if given sufficient time a large portion of intestine may be saved. A planned second look laparotomy in babies with volvulus and doubtful gut viability can be helpful in saving large bowel segment. We present a case of a newborn baby admitted with bilious vomiting, abdominal distension and melena. An urgent exploratory laparotomy showed midgut volvulus with near gangrene of about 35 cm of proximal jejunum and bluish discoloration of the rest of the small bowel. After de-rotation and warm packs, the vascularity of ileum & distal jejunum returned to normal, however the proximal jejunum remained dusky and bruised. Ladd's procedure was done and a decision was made to close the abdomen with a plan for a 2nd look laparotomy after 24 hours. A relook laparotomy performed after 24 hours showed, a well vascularized small bowel. No bowel resection was required and abdomen was closed. The baby improved and was discharged in stable condition with no long-term ischemic complications. This case indicates that a second look laparotomy may provide time for revascularization of the intestine with doubtful vascularity and avoid unnecessary bowel resection.

Obstructive Jaundice Caused by Hepatic Torsion in an Infant with Congenital Diaphragmatic Hernia.

Right sided congenital diaphragmatic hernia may cause biliary obstruction. We present a 2 months female infant who had respiratory distress and persistent jaundice since birth. Investigations suggested direct hyperbilirubinemia, right-sided diaphragmatic hernia with liver herniation in the thorax, and intra- and extrahepatic biliary dilatation. Laparotomy showed herniation of liver in the chest with hepatic torsion of about 180° causing obstruction of bile ducts. Liver torsion was corrected and liver relocated in the abdomen. An operative cholangiogram confirmed free passage of contrast to the intestine after correction of hepatic torsion. Repair of the diaphragmatic hernia was performed. Only skin closure was performed leaving a ventral hernia to avoid abdominal compartment syndrome. Postoperatively, the baby was kept on ventilator for 2 days and then extubated. She showed rapid recovery and was discharged in a stable condition. The ventral hernia was repaired at the age of 6 months. Her total bilirubin levels dropped gradually from 12.50 mg/dl into its normal values within 3 months.

Distal Esophageal Duplication Cyst with Gastro-Esophageal Reflux Disease: A Rare Association and a Management Challenge.

Esophageal duplication cysts are rare congenital abnormalities of the foregut and may be associated with other conditions. Association of esophageal duplication with Gastro-Esophageal Reflux Disease (GERD) has not been reported in children. We are reporting a case of a 16 months baby who had antenatal diagnosis of diaphragmatic hernia. Postnatal CTchest, however, suggested a distal esophageal duplication cyst and a contrast esophagogram showed grade-IV GER. A thoracoscopy in another hospital excluded esophageal duplication at that time. Later, he presented with hematemesis in our department and was re-evaluated. Repeat CTconfirmed a persistent 2.5 x 1.3 cm cyst in distal esophagus. Upper GI endoscopy suggested grade-II esophagitis with a wide patent gastro-esophageal junction. The child was treated with left thoracotomy, excision of the duplication cyst and thoracic fundoplication. He had an uneventful post-operative recovery and is doing well at 6 months follow-up.

Simultaneous occurrence of intussusception and duodenal haematoma in a patient with Glanzmann's thrombasthenia.

Glanzmann's thrombasthenia (GT) is a rare autosomal recessive disorder in which the platelets have anomalies of fibrinogen receptors causing bleeding tendencies. The disease usually presents with mucosal bleeding, petechial haemorrhages or gastrointestinal (GI) bleeding. Only sporadic cases of intussusception or duodenal haematoma have been reported with GT. We present a case of 5-year-old girl admitted with non-bilious vomiting, watery diarrhoea and abdominal pain. She is a known patient of Glanzmann's thrombasthenia.

Posterior sagittal approach for resection of sacrococcygeal teratomas.

The aim is to evaluate the ease of access, surgical trauma and cosmetic results of posterior sagittal approach for sacrococygeal teratomas (SCTs). From January 2002 to June 2010, we operated 19 cases of SCT exclusively through posterior sagittal approach. Patients were placed in knee chest position. An elliptical incision was made for the excision of the tumor. Care was taken to preserve all the muscles and other vital tissue in this area. Coccygectomy was performed in all patients. Closure was performed in layers. It was possible to resect all tumors from this approach and none of the patients required revision of the incision. Complete resection was possible in 17 patients while two had residual disease due to local extension. Minimal wound dehiscence was noted in four patients that healed spontaneously. It was possible to perform excision even in very large masses. Wound scar was satisfactory in most patients with preservation of gluteal folds. It is therefore concluded that posterior sagittal approach for SCT is feasible, with good access and cosmetic results.

Rectal prolapse: an unusual presentation of malakoplakia in a child.

A case of a young girl is presented. The main concern was recurrent rectal prolapse not responding to conservative and sclerotherapy. Colonoscopy showed diffuse colitis with superficial ulcerations and pseudopolyps. Colon biopsies finally showed classical features of malakoplakia confirmed by histopathological test. Conservative treatment failed and ultimately proctocolectomy was performed with ileoanal endorectal anastomosis.