Cystic lymphangiomatosis with severe intra-abdominal bleeding in a newborn: case report.

We report the case of a newborn girl with intestinal cystic lymphangiomatosis who presented with abdominal distension and intra-abdominal bleeding following a prenatal ultrasound diagnosis of intestinal anomaly. Postnatal abdominal ultrasound revealed disseminated submucosal and intramural cystic dilatations of various sizes in the bowel and intestinal lymphangiomatosis was diagnosed. The presence of severe bleeding diathesis and widespread disease led to conservative treatment. The patient died on postnatal day 7 and postmortem examination confirmed cystic lymphangiomatosis. Detection of intestinal hyperechogenicity and/or dilatation in prenatal ultrasonography and the persistence of these findings during pregnancy are suggestive for pathologies such as meconium ileus, meconium peritonitis, and intestinal atresia. Although rare, intestinal lymphangiomatosis should be kept in mind in patients whose prenatal sonographic findings persist until birth.

Diagnosis in childhood abdominal Burkitt's lymphoma.

BACKGROUND: The role of surgery has changed substantially over the years in abdominal Burkitt's lymphoma. Laparotomy without total excision of the tumor does not have a positive effect on survival, might cause complications, and delays initiation of chemotherapy. Here we present our diagnostic management of patients with abdominal Burkitt's lymphoma. MATERIALS AND METHODS: The diagnostic methods of abdominal Burkitt's lymphoma cases treated between January 1999 and December 2009 were evaluated retrospectively. RESULTS: Of the 48 abdominal Burkitt's lymphoma patients, 13 also had extra-abdominal site involvement. Diagnosis was made with ultrasound-guided tru-cut needle biopsy of the abdominal mass (n = 11), fluid cytology (n = 7), extra-abdominal site biopsy (n = 4), bone marrow aspiration (n = 2), gastroscopy (n = 1), and laparotomy (n = 23). In patients diagnosed with laparotomy, chemotherapy was started in 4-22 days (median 7) compared with patients diagnosed with other diagnostic interventions in 2-4 days (median 2) (P < .001). CONCLUSION: Although the most frequently used technique is laparotomy and open biopsy in our series, other methods provided quicker initiation of chemotherapy and less surgical morbidity. Especially in patients with high stages, cytological evaluation and tru-cut needle biopsy with radiological guidance is a better alternative of laparotomy.

Ultrasound guided reduction of intussusception with saline and comparison with operative treatment.

BACKGROUND: Reduction of intussusception under ultrasound guidance by saline has become popular in recent years. However, methods, duration of the procedure and causes of failure are not defined. In this study, we reviewed the patients who underwent ultrasound (US) guided saline reduction and compared them with those who were previously managed by operative intervention. METHODS: Patients with severe peritonitis or perforation, those over 3 years or younger than 1 month were excluded. Saline was applied by anus. Entry of saline into the ileum was the main indicator for successful reduction. Dramatic improvement in the clinical findings was considered as an additional sign of successful reduction. No limit was imposed on duration of the procedure. RESULTS: Hydrostatic reduction was successful in 41 out of 51 patients with intussusception. In three patients with partial resolution, hydrostatic reduction was attempted later and total reduction was achieved. No perforation or other complications were seen. In ten cases with reduction failure, one had an ileal lymphoma and another one had a duplication cyst as lead points. CONCLUSION: US guided hydrostatic reduction for childhood ileocolic intussusception is safe and, painless, has a high success rate and avoids radiation exposure risk. Presence of ultrasonographic and clinical changes is the best indicator of a successful reduction. In some cases, a second attempt may be necessary for reduction.

Martius repair in urethrovaginal defects.

AIM: To repair a urethrovaginal defect in childhood is a challenge for a pediatric surgeon. Martius fat-pad flap repair is being used in women successfully. Here, we report 2 girls who had Martius repair for their urethrovaginal defects. METHOD: Topical estriol and asiaticoside perineally were administered for preoperative 3 weeks to reinforce the tissues. Martius repair was done using 1-sided labial fat-pad flap. Urethral and bladder catheters were inserted. Urethral catheter was removed on postoperative day 14 and bladder catheter on day 21 after controlling residual urine. PATIENTS: Patient 1, a 6-year-old girl, had lipomeningocele repair at the age of 18 months and had an iatrogenic urethrovaginal fistula that is caused by catheter insertion. She developed urinary incontinence, and 3 primary repair attempts were unsuccessful. Patient 2 is a 5-year-old girl who had pouch colon with persistent cloacal malformation and had posterior anorectovaginourethroplasty. The urethrovaginal septum did not heal, and she was incontinent. One attempt of primary repair was unsuccessful. The urethrovaginal wall was completely open at the time of Martius repair in both patients. RESULTS: Urethral wall was completely healed after Martius repair in both patients. CONCLUSION: Martius fat-pad flap repair can be used to repair urethrovaginal fistulas in girls. It has both functionally and cosmetically good results, and neourethra is easily catheterizable.

A standardised protocol for the acute management of corrosive ingestion in children.

Oesophageal strictures developing after caustic ingestion in children are a serious problem, and several protocols to prevent stricture formation have been proposed. A prospective clinical trial was conducted for preventing strictures in caustic oesophageal burns in a single clinic, and the results are presented. All children with caustic ingestion who had oesophagoscopy for diagnosing the severity of the burn were included in the study. Eighty-one children were included in the series, with ages ranging between 3 months and 12 years. The patients were given nothing by mouth until oesophagoscopy. IV fluids, broad-spectrum antibiotics, ranitidine, and a single-dose steroid were given. Oral burns were positive in 66 patients. Oesophagoscopy revealed a normal oesophagus in nine patients, grade 1 burn in 24, grade 2a in 21, grade 2b in 23, grade 3a in two, and grade 3b in one. Patients with grade 1 and 2a burns were discharged after oesophagoscopy. Patients with grade 2b and all grade 3 burns were given nothing by mouth for a week except water when swallowing their saliva, and were fed via total parenteral nutrition. After the 1st week, if there was no problem with swallowing, liquid foods were introduced. No intraluminal tubes were used. At the end of the 3rd week, a barium meal was administered and an upper gastrointestinal series taken. Dilatation was performed at 2-week intervals for strictures, which developed in one grade 2a patient, six grade 2b patients, and the grade 3b patient. Only one of these patients is currently on an oesophageal dilatation program. Limiting oral intake and avoiding foreign bodies in the oesophagus seem to provide a good success rate; however, further prospective studies are needed to decrease the incidence of corrosive oesophageal strictures.