Aesthetic Unit Boundary Approach to Large Fibroadenoma Resection.

BACKGROUND: Fibroadenomas are the most common benign breast tumors in adolescents. Surgical excision is indicated when the tumor becomes large or symptomatic. Multiple approaches have been described. However, unsightly scars, excess skin, and breast asymmetry are common challenges after tumor resection. The aims of our study were to describe a concentric circumareolar approach combining the round-block technique and geometric principles in the management of large benign breast tumors. METHODS: This was a retrospective review of pediatric patients who have undergone excision of large fibroadenoma with concentric circumareolar approach from June 2007 to May 2017. Preoperatively, the excess skin that needed to be resected was marked based on geometric principles. Under general anesthesia, circumareolar deepithelialization of the excess skin and tumor resection were performed. Purse-string suture technique was used to achieve the proper nipple-areola complex size. RESULTS: Satisfactory breast symmetry and minimal scarring were achieved in all 6 patients. One patient developed a small seroma, which resolved spontaneously without intervention. CONCLUSIONS: Concentric circumareolar approach can be used to resect large benign breast tumors while concealing the scar along the aesthetic unit boundary of the breast. The cosmetic outcome and recovery were promising. The approach is simple to execute, highly reproducible, and less dependent on intuition.

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.

Metastatic Neuroblastoma Presenting as a Submandibular Mass with Mandibular Bone Involvement in a Three-Year-Old Child.

Although neuroblastoma is one of the most common extra-cranial tumors in the pediatric population, it is rarely seen as a metastasis to the mandibular bone. The following is a case report of a 3-year-old male who initially presented with a submandibular mass that was proven to be a poorly differentiated metastatic neuroblastoma through excisional biopsy. This report is one of the few case reports that demonstrates metastatic submandibular neuroblastoma with mandibular bone involvement in the pediatric population.

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.

Differences in the Management of Perforated Appendicitis in Children by Race and Insurance Status.

This study was conducted to assess whether race and socioeconomic status influence the management method used to treat pediatric perforated appendicitis. Nonelective pediatric admissions with a primary diagnosis of appendicitis were analyzed using data from the 2001-2010 Nationwide Inpatient Sample. Bivariate and multivariate analyses were used to determine the association between race, insurance status, median household income, rural/metropolitan location, and the risk adjusted odds of undergoing surgery, laparoscopic appendectomy, percutaneous drainage, or neither surgery nor percutaneous drainage. A total of 46,211 admissions of perforated appendicitis were identified. Surgery was performed in 90.5 per cent of them. Black children were less likely to have surgery [adjusted odds ratio (AOR) = 0.53] and more likely to be managed nonsurgically with percutaneous drainage (AOR = 1.79). Self-pay patients were less likely to have laparoscopic surgery (AOR = 0.80). Children from rural counties were more likely to undergo surgery than those from larger metropolitan areas (AOR = 1.30). Higher estimated household income did not predict the method of treatment. Although previous studies have attributed racial disparities in outcomes for appendicitis to different rates of perforation and access to care, these findings demonstrate significantly dissimilar management strategies for patients presenting with a similar disease process.

Management of Patients with Gastroschisis Requiring Extracorporeal Membrane Oxygenation for Concurrent Respiratory Failure.

Treatment of gastroschisis often requires multiple surgical procedures to re-establish abdominal domain, reduce abdominal contents, and eventually close the abdominal wall. In patients who have concomitant respiratory failure requiring extracorporeal membrane oxygenation (ECMO), this process becomes further complicated. This situation is rare and only five such cases have been reported in the ECMO registry database. Management of three of the five patients along with results and implications for future care of similar patients is discussed here. Two patients had respiratory failure due to meconium aspiration syndrome and one patient had persistent acidosis as well as worsening pulmonary hypertension leading to the decision of ECMO. The abdominal contents were placed in a spring-loaded silastic silo while on ECMO and primary closure was performed three to six days after the decannulation. All three patients survived and are developmentally appropriate. We recommend avoiding aggressively reducing the abdominal contents and using a silo to conservatively reducing the gastroschisis while the patient is on ECMO therapy. Keeping the intra-abdominal pressure below 20 mm Hg can possibly reduce ECMO days and ventilator time and has been shown to decrease morbidity and mortality. Patients with gastroschisis and respiratory failure requiring ECMO can have good outcomes despite the complexity of required care.

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.

Staging paratesticular rhabdomyosarcoma in the "as low as reasonably achievable" age: the case for PET-CT.

Rhabdomyosarcoma accounts for 40% of paratesticular malignancies and 5% of testicular and paratesticular malignancies in children and adolescents. The Children's Oncology Group currently recommends computed tomography (CT) or magnetic resonance imaging for staging of paratesticular rhabdomyosarcoma in children. The present case illustrates a 9-year-old boy with paratesticular rhabdomyosarcoma who had negative findings on a staging CT scan and a subsequent positron emission tomography-CT scan demonstrating retroperitoneal lymph node metastasis. In the era of "as low as reasonably achievable" imaging, positron emission tomography-CT should be considered first-line imaging for staging to improve the sensitivity and specificity of staging for paratesticular rhabdomyosarcoma.

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.