Müllerian-type, cutaneous ciliated cyst in the gluteal cleft mimicking a pilonidal cyst.

A cutaneous ciliated cyst is a rare entity found predominantly in the lower extremities and perineal region of young females. Although initially described by Hess in 1890, the present day term, "cutaneous ciliated cyst," was proposed by Farmer in 1978 and includes a wide array of cyst types. Despite their typical female predominance and location, many have described cutaneous ciliated cysts in males and atypical locations. In addition, Mullerian cysts in the posterior mediastinum and the retroperitoneum have been reported. To date, only 40 cases have been reported in the literature of a Mullerian-type, cutaneous ciliated cyst. Here, we report a case of 13-year-old female with one in the gluteal cleft, initially presenting as a pilonidal cyst. We also discuss the differential diagnosis of pediatric sacrococcygeal lesions and pathogenesis of a Mullerian-type, cutaneous ciliated cyst.

Laparoscopic treatment of simultaneously occurring pyloric stenosis and malrotation.

Hypertrophic pyloric stenosis (HPS) is a common cause of nonbilious vomiting in the neonatal period with an incidence of approximately 1 to 3 per 1000 live births. The Ramstedt pyloromyotomy has been the standard treatment since 1912. In 1991, Alain et al. reported a novel approach to HPS using laparoscopy. Since this original description, the laparoscopic pyloromyotomy has become progressively more popular and, in many institutions, has replaced the open approach. Similarly, malrotation is a condition affecting the neonatal population, resulting from incomplete intestinal rotation about the superior mesenteric artery during weeks 10 through 11 of development. If left untreated, it can lead to abnormal mesenteric attachments and a narrowed mesenteric base, placing the patient at risk for midgut volvulus. The standard surgical treatment has been the open Ladd procedure first described in 1932. In 1996, Gross described a minimally invasive procedure to address malrotation. The association of concurrent pyloric stenosis and malrotation has rarely been reported in the pediatric literature. This is the first published report of a laparoscopic treatment of HPS and malrotation simultaneously.

Analysis of 334 Cases of Pediatric Esophageal Foreign Body Removal Suggests that Traditional Methods Have Similar Outcomes Whereas a Magnetic Tip Orogastric Tube Appears to Be an Effective, Efficient, and Safe Technique for Disc Battery Removal.

Procedures and outcomes for pediatric esophageal foreign body removal were analyzed. Traditional methods of battery removal were compared with a magnetic tip orogastric tube (MtOGT). A single institution retrospective review from 1997 to 2014 of pediatric patients with esophageal foreign bodies was performed. Balloon extraction with fluoroscopy (performed in 173 patients with 91% success), flexible endoscopy (92% success in 102 patients), and rigid esophagoscopy (95% in 38 patients) had excellent success rates. A MtOGT had 100 per cent success in six disc battery patients, when other methods were more likely to fail, and was the fastest. Power analysis suggested 20 patients in the MtOGT group would be needed for significant savings in procedural time. Thirty-two per cent of all foreign bodies and 95 per cent of batteries had complications (P = 0.002) because of the foreign body. Overall, 1.2 per cent had severe complications, whereas 10 per cent of batteries had severe complications (P = 0.04). Each technique if applied appropriately can be a reasonable option for esophageal foreign body removal. Magnetic tip orogastric tubes used to extract ferromagnetic objects like disc batteries had the shortest procedure time and highest success rate although it was not statistically significant. Disc batteries require emergent removal and have a significant complication rate.

Venovenous Extracorporeal Membrane Oxygenation in Pediatric Respiratory Failure.

Conventional treatment of respiratory failure involves positive pressure ventilation that can worsen lung damage. Extracorporeal membrane oxygenation (ECMO) is typically used when conventional therapy fails. In this study, we evaluated the use of venovenous (VV)-ECMO for the treatment of severe pediatric respiratory failure at our institution. A retrospective analysis of pediatric patients (age 1-18) placed on ECMO in the last 15 years (1999-2014) by the pediatric surgery team for respiratory failure was performed. Five pediatric patients underwent ECMO (mean age 10 years; range, 2-16). All underwent VV-ECMO. Diagnoses were status asthmaticus (2), acute respiratory distress syndrome due to septic shock (1), aspergillus pneumonia (1), and respiratory failure due to parainfluenza (1). Two patients had severe barotrauma prior to ECMO initiation. Average oxygenation index (OI) prior to cannulation was 74 (range 23-122). No patients required conversion to VA-ECMO. The average ECMO run time was 4.4 days (range 2-6). The average number of days on the ventilator was 15 (range 4-27). There were no major complications due to the procedure. Survival to discharge was 100%. Average follow up is 4.4 years (range 1-15). A short run of VV-ECMO can be lifesaving for pediatric patients in respiratory failure. Survival is excellent despite severely elevated oxygen indices. VV-ECMO may be well tolerated and can be considered for severe pediatric respiratory failure.

Single-stage reconstruction of perforated choledochal cyst: case report and review of the literature.

Choledochal cysts represent a rare disease in the Western world. We reviewed our recent experience with a case of perforated choledochal cyst, define the currently accepted treatment options, and review the literature of this unusual disease. An 11-month-old girl presented with abdominal pain and distention as well as non-bilious vomiting. Subsequent workup included endoscopic retrograde cholangiopancreatography revealing a perforated type I choledochal cyst. She underwent single-stage excision and reconstruction with a Roux-en-Y hepaticojejunostomy. Perforated choledochal cyst is a rare event, and prompt surgical intervention is warranted. Single-stage cystectomy and Roux-en-Y reconstruction is possible in select patients. A thorough understanding of the pathophysiology, management, and follow-up is required.

The use of multiple serial transverse enteroplasty (STEP) procedures for the management of intestinal atresia and short bowel syndrome.

The serial transverse enteroplasty procedure (STEP) was introduced as a bowel-lengthening procedure to reduce complications related to short bowel syndrome (SBS). Although some have described it as a useful adjunct to the Bianci procedure, others have acknowledged it as a primary procedure. We present a case of jejunal atresia in which two STEP procedures were performed 7 months apart to increase small bowel length. A 1-day-old, term girl presented with a known bowel obstruction diagnosed in utero. A laparotomy revealed a Type IIIb jejunal atresia with no remaining small bowel or cecum. A STEP procedure with an end jejunostomy and ascending colon mucous fistula lengthened the small bowel from 35 to 50 cm. A repeat procedure 7 months later lengthened it to 89 cm. The STEP procedure results in slower intestinal transit time and increases enterocytes contact with oral intake. We performed it during our initial exploration to increase small bowel size by 30 per cent. A repeat procedure 7 months later increased length to 89 cm. The use of multiple, staged STEP procedures avoided the need for bowel transplantation and long-term total parenteral nutrition dependence, demonstrating its effectiveness as a primary procedure for the surgical management of SBS.

Popliteal pseudoaneurysm caused by Nora's lesion of the femur in a young child: a rare presentation and first report.

Bizarre parosteal osteochondromatous proliferation (BPOP), or Nora's lesion, was first described by Nora et al. in 1983 as a rare, tumor-like lesion involving the bones of the hands and feet. Popliteal artery pseudoaneursyms in the pediatric population are also unusual. Here, we present a case of a young male with a popliteal artery pseudoaneurysm and distal femur lesion originally thought to be an osteochondroma. A 10-year old, Caucasian male was referred to our facility following an MRI concerning for a popliteal artery pseudoaneurysm. On physical exam, there was a palpable 5 × 5-cm pulsatile mass in the upper popliteal fossa with a normal pulse exam bilaterally. A computed tomographic angiogram demonstrated a 4.5-cm by 1.8-cm by 3.6-cm pseudoaneurysm adherent to a 3.5-cm thick, exostotic lesion of the posterior right femur. He was taken to the operating room for repair of the popliteal pseudoaneurysm and resection of his bone lesion. The final pathology was consistent with a popliteal pseudoaneurysm, osteochondroma, and bizarre parosteal osteochondromatous proliferation (BPOP), otherwise known as Nora's lesion. The location of the lesion and the age of our patient were both atypical for BPOP and to our knowledge, this represents the first report of a resulting popliteal artery pseudoaneurysm.

Heterotopic gastric mucosa of the upper esophagus following repair of esophageal atresia with tracheoesophageal fistula.

A term female newborn underwent uncomplicated repair of esophageal atresia with tracheoesophageal fistula (type C) then coarctation of the aorta. Subsequently, she developed symptomatic esophageal strictures, which required serial dilations. In addition, she suffered feeding intolerance from esophageal dysmotility and gastroesophageal reflux disease, necessitating Nissen fundoplication with gastrostomy. At 6 years of age, surveillance esophagogastroduodenoscopy revealed mild stenosis and heterotopic gastric mucosa of the upper esophagus at the level of the anastomosis. This is the second report of heterotopic gastric mucosa of the upper esophagus at the level of the anastomosis following repair of esophageal atresia with tracheoesophageal fistula.