Richa Jaiman

Noncommunicating isolated enteric duplication cyst in childhood

Duplications of the alimentary tract are spherical or tubular structures lined by epithelium similar to intestine that are firmly attached to or share the wall of the alimentary tract and have a common blood supply with the adjacent segment of the bowel. Completely isolated duplication cysts are an extremely rare variety of gastrointestinal duplications with their own exclusive blood supply and do not communicate with the intestine. There are only 4 cases reported in the English literature-3 in childhood and 1 in an adult, and all are male. We report a case of noncommunicating isolated ileal duplication cyst in a 3-year-old female child that presented as a mass in the right iliac fossa, mimicking an intussusception. The duplication had its own blood supply arising from the terminal ileal mesentery. This report describes the first female patient with this condition and reviews the English literature.

Duplication cyst of pyloroduodenal canal: a rare cause of neonatal gastric outlet obstruction: a case report

BackgroundA 21 day old male child presented with non bilious vomiting and abdominal mass.Case presentationThis case is reported because pyloroduodenal duplication cysts are an extremely rare congenital anomaly, whose clinical presentation often mimics those of hypertrophic pyloric stenosis. Ultrasound examination showed cystic mass at pyloric region and barium study was suggestive of extrinsic mass compressing the pyloric region. A laparotomy, a tense cystic mass was present at the pyloroduodenal junction (PDC) which was resected and end to end anastomosis was done. Patients followed an uneventful recovery and doing well.ConclusionThe clinical and radiological analysis can reveal configurational changes consistent with a large extrinsic mass rather than muscular hypertrophy and can lead to accurate preoperative diagnosis.

A child presented with bilateral congenital constriction ring in lower extremity: a case report

IntroductionThe congenital constriction ring of lower extremity is very uncommon and rare condition. The actual incidence in general population is not known. In English literature, very few cases are reported time to time as congenital constriction band syndrome associated with musculoskeletal disorder like congenital talipes equino varus. The lesion can involve skin only or goes to deeper structure up to bone, which can lead to gangrene of foot or auto amputation.Case presentationWe are presenting a case of bilateral congenital constriction ring in lower limb who presented at age of 4 year without any other associated congenital anomaly, simply managed by Z-plasty, which improves quality of life after physiotherapy.ConclusionCongenital constriction ring of lower limb is extremely rare condition in children. Early diagnosis and management is mandatory, either in single stage or by stage procedure, to prevent auto-amputation of limb and to improve quality of life on feet.

Atresia of ileocecal junction, ileocecal valve: Rare variants of bowel atresia

Atresia of ileocecal junction and isolated atresia of ileocecal valve are rare types of intestinal atresia with very few reports in literature. We report two such cases. Radiology showed dilated ileal segment and distal micro colon in both the cases. At laparotomy there was atresia of ileocecal junction in the first case and isolated ileocaecal valve atresia with normal ileocecal junction in the other case. Both the babies were managed by ileocolic resection with an end to end anastomosis. The prognosis of ileocecal atresias is satisfactory.

Preduodenal Portal Vein Associated with Duodenal Obstruction of other Etiology: A Case Series

DuodenalPreduodenal portal vein is a rare and interesting entity which often causes duodenal obstruction. It is also associated with other congenital anomalies. We report here three cases of preduodenal portal vein associated with other anomalies causing duodenal obstruction not related to direct compression by portal vein itself.

Giant Splenic Lymphangiomatosis in Adult: a Diagnostic Dilemma

Cystic lymphangiomatosis of the spleen occurs secondary to developmental malformation of the lymphatic system. It is one of the rare entities generally seen in children. It is usually seen in children. We report a case of cystic lymphangiomatosis of the spleen occurring in an adult woman presenting with massive splenomegaly. Total splenectomy was considered to be the treatment of choice for making definitive diagnosis and to exclude the presence of malignancy or other causes of massive splenomegaly

GIANT MECONIUM HYDROCELE: A RARE PRESENTATION OF ANTENATAL GASTROINTESTINAL PERFORATION

Meconium peritonitis is a sterile chemical peritonitis due to leakage of meconium from the bowel in utero. Perforation usually results from obstruction, which can be due to volvulus, intestinal atresia or inspissated meconium associated with cystic fibrosis or vascular compromise of the gut. Occasionally the perforation is idiopathic; gastrointestinal disorder is evident at birth. Although the processus vaginalis is still patent in 80–90% of full-term babies, extension of intra-abdominal pathology into the scrotum is relatively rare. The processus vaginalis forms by the sixth foetal month and enters the scrotum by the seventh month. Subsequently the testes descend into the scrotum. The processus vaginalis remains patent until birth or shortly thereafter, leaving the residual tunica vaginalis. Following an in utero bowel perforation, a fluid meconium-filled ‘hydrocele’ may thus be present at birth. We present a typical giant meconium hydrocele with ascitis of unknown aetiology not reported in our country. A full-term, 1-day-old male baby weighing 2.7 kg presented to us at 36 h after birth with bilateral scrotal swelling of 8 ¥ 6 ¥ 5 cm; soft, cystic, normal colour of overlying mature scrotal skin; no impulse on crying; non-compressible, positive transillumination in the root of scrotum and negative below that (Fig. 1). The baby passed urine normally on the first day of life but had not passed meconium since birth. Abdomen was distended and free fluid was present. The general condition was poor and respiratory rate was high. The baby was put on intravenous fluid and antibiotics. X-ray of the abdomen was suggestive of pneumoperitonium with opacity of scrotum. Abdominal paracentesis drained 100 ml of meconium stained fluid following which explortory laparotomy was done. On exploration, the peritoneum was filled with liquefied meconium, which was communicated with scrotum via the inguinal canal but the perforation site was not detected in the whole gastrointestinal tract. The peritoneal cavity was cleaned by normal saline and antibiotics solution and the bilateral inguinal ring was closed by absorbable suture after aspiration of fluid from the scrotal sac. The abdomen was closed after placement of a drain in the pelvis. The baby passed stool after 3 days and the drain was removed after 5 days. Oral feed was allowed on the seventh day and the baby was discharged well on the 10th day. The baby was well in the follow-up. A patent processus vaginalis in the male foetus may lead to the migration of meconium into the scrotum, and these infants can present with soft meconium-filled hydrocele at birth. Local tissue reaction during the first several weeks of life results in calcification of this hydrocele into hard, tumour-like lesions that may mimic testicular neoplasia. The simple bilateral hydrocele can be differentiated from meconium hydrocele and testicular neoplasia clinically, in the form of the fluctuation and transillumination test and also by ultrasonological examination of the scrotum. Tanaka et al. noted that operation of meconium peritonitis was difficult because the patient’s general condition was poor, and dissection of the adhesion was difficult, owing to severe inflammation and bleeding. The patient undergoing an ultrasoundguided drainage of the cystic meconium peritonitis before surgery had better outcome. In our case, the cause of bilateral meconium hydrocele may be an antenatal perforation in the gastrointestinal tract due to unknown cause becomes sealed off in later period of gestation. It is mandatory to rule out this lifethreatening condition by antenatal sonography, post-natal clinical examination and radiological study to confirm the diagnosis of gastrointestinal perforation.