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.

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.

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.

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.

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.