Hereditary Multiple Intestinal Atresia: A Case Report and Review of the Literature.

Hereditary multiple intestinal atresia (HMIA) is a rare form of intestinal atresia that affects the gastrointestinal tract from the pylorus to the rectum. A few cases have been reported in the literature. Here, we report the case of a three-day-old girl who was referred to our hospital as a case of upper intestinal obstruction. After initial resuscitation, the radiological examination revealed pyloric obstruction, multiple intra-abdominal calcifications, and rectal atresia making the diagnosis of HMIA most likely. Exploratory laparotomy revealed multiple intestinal atresia, the first started at the pylorus, the second was at the level of the duodenojejunal junction, and there were also multiple small bowel atresias. The colon was a cord-like structure, and there was rectal atresia. Multiple resections of the atretic intestinal segment followed by multiple anastomoses, terminal ileostomy, and the use of a trans-anastomotic tube were performed. In this study, the clinical picture, radiological findings, and management are described and compared to the findings reported in the literature.

Bilateral iliopsoas abscess associated with right hip septic arthritis in a neonate.

Iliopsoas abscess is a very rare pathology in the neonatal period. There is a lack of reports in the literature on bilateral psoas abscess with hip joint arthritis. We report a case of bilateral iliopsoas abscess with concomitant right hip septic arthritis, caused by methicillin-resistant Staphylococcus aureus in a 28-day-old male infant. The baby presented with bilateral diffuse swelling of the groins and upper thighs. He was treated successfully by ultrasound-guided percutaneous drainage along with systemic antibiotic therapy. Clinical improvement was observed within 24-48 hours of drainage.

Assisted laparoscopic excision of huge abdominal cysts in newborns and infants using the umbilical laparoscopic port incision.

PURPOSE: The objective of this article was to present our experience in successfully managing a variety of abdominal cysts in newborns and infants using a mini-invasive technique performed in 8 cases using an umbilical laparoscopic port incision. METHODS: Assisted laparoscopic excision using an umbilical port incision was performed in 8 cases. Six were neonates (5 ovarian cysts and one hepatic cyst) and 2 were infants (6-months old with intestinal duplication and 11-months old with lymphatic malformation). RESULTS: The procedure was performed with ease and was successful in all 8 cases. Oral feeding was started early after the surgical intervention. Seven patients were discharged the next day. The last case was discharged on the fifth day after surgery because of intestinal resection. CONCLUSION: This proposed mini-invasive technique using the umbilical port incision proved to be less invasive, easier, and less technically demanding than the classic laparoscopic approach and, hence, may be applied to different abdominal cystic pathologies in newborns and infants.

Management of omphalocele using the sac as supportive material.

OBJECTIVE: To document retrospectively the outcome of a newly modified procedure for closure of large omphalocele, where the sac was conserved and used as autogenous supportive material. METHODS: The medical records of 10 patients with omphalocele major admitted to Al-Noor Specialist Hospital in Makkah, Kingdom of Saudi Arabia in the last 15 years, October 1990 to October 2005, were retrospectively reviewed. The defects were considered by the same treating surgeon too big to be closed primarily so the sac was preserved to reduce the contents gradually. RESULTS: In the first stage, complete reduction was achieved in all the cases followed by second stage, the surgical closure. There was no mortality related to this intervention. CONCLUSION: This proposed technique is safe, simple and effective for the treatment of large omphalocele.

Precocious puberty in a female with Proteus Syndrome.

Proteus syndrome is a complex disorder comprising malformations and overgrowth of multiple tissues and characterized by its polymorphism and mosaicism. The syndrome is rare and sporadic. Oliveira M da C et al reported the first case of Proteus syndrome associated with precocious puberty in a boy. We are reporting a case of a 7-month old girl with Proteus syndrome who developed a juvenile granulosa cell tumor in one ovary causing precocious puberty. At our knowledge this is the first case of Proteus syndrome with precocious puberty in a female.