Faculty Bibliography

A 70-year-old man presented with a contained aortic rupture above the aortic bifurcation in the setting of a chronic type B aortic dissection. The celiac and right iliac arteries were supplied by the false lumen, and the superior mesenteric, left iliac, and bilateral renal arteries were supplied by the true lumen. An open repair was not possible due to right-sided heart failure. The "cheese wire" maneuver is a technique used to fenestrate an intimal flap, alleviating malperfusion in aortic dissection. In our modification, a Glidewire (Terumo Medical, Somerset, NJ) was passed across the intimal flap using a Ross Modified Colapinto needle (Cook Medical, Bloomington, Ind) and pulled downward to shear through the membrane to the aortic bifurcation. The fenestration was followed by a unibody endograft stent repair across the contained rupture.

BACKGROUND:Sinonasal tumors in the pediatric population are exceedingly rare. MATERIALS AND METHODS/METHODS:Surveillance, Epidemiology, and End Results database was used to identify 250 cases of sinonasal malignancy in patients aged <20 y (1973-2010). Malignant histology codes were based on the International Classification of Disease for Oncology, third edition coding scheme. Incidence rates were adjusted to the 2000 U.S. population. Survival outcomes were plotted using the Kaplan-Meier method and compared with the log-rank test. All other analyses were performed using standard statistical methods. RESULTS:Overall incidence was 0.052 per 100,000. Rhabdomyosarcoma had the highest incidence among histologic groups. Regional stage was the most common at diagnosis (59%). Overall survival at 5-y follow-up was 62.5%. Patients in age groups 1-4 and 15-19 y had the worst survival rates, as median survival was 205 and 104 mo, respectively. Distant metastases at the time of diagnosis signified a poor prognosis. These were associated with a 39-mo median survival. Survival improved during the study period, P=0.003. Gender, race, site of lesion, or histology did not appear to affect mortality. CONCLUSIONS:Sinonasal tumors are rare in children and adolescents. Long-term survival is dependent on age and clinical stage at the time of diagnosis. Cancer-related surgery confers a survival advantage. Gender, race, and histologic type are not associated with mortality.

Malignant melanomas are the most common skin cancer in the pediatric population. Melanoma incidence is extremely low in infants, and metastatic disease is even less common. We present the case of an 11-month-old girl who presented with a non-pigmented lesion that progressed to an ulcerated lesion. Pathology was found to be Spitzoid melanoma of 7.6-mm thickness. Micrometastases were found on examination of the sentinel lymph node. The family chose expectant observation following the excision procedure. A pediatric melanoma registry may be helpful in developing future analyses of incidence in survival in this specialized population.

A 17-year-old girl presented with episodic vomiting associated with chest pain, a 20-pound weight loss over the past year, and multiple hospitalizations for pneumonia. She was bradycardic, cachectic (<3rd percentile), pale, and had anterior cervical lymphadenopathy. CT angiography suggested an obstructive vascular ring (formed by a right aortic arch with an aberrant left subclavian artery), diverticulum of Kommerell, and a tracheoesophageal fistula (TEF). She underwent left thoracotomy with ligation and division of the ligamentum arteriosum to relieve the vascular ring. Flexible bronchoscopy at the time demonstrated a large H-type TEF. Eight weeks later, she had the TEF closed via a right cervical incision and recovered uneventfully. Our case is unique, with symptomatic presentation of a congenital TEF and vascular ring in a teenager. Such major congenital anomalies are rarely discovered outside of childhood, and TEFs virtually always (>90%) present as neonatal emergencies secondary to esophageal obstruction. They have a high incidence of associated abnormalities, cardiovascular being the most common. Unexplained recurrent respiratory symptoms in an otherwise normal child with dysphagia should prompt the clinician to evaluate patients for foreign bodies, reflux and other more common problems. Unusual etiologies, however, do occur - as in this case - and warrant more complex workup.

Superior mesenteric artery (SMA) syndrome is an uncommon condition where the third portion of the duodenum is compressed and obstructed between the SMA and the aorta. An otherwise healthy 17-year-old female presented with a 2-month history of postprandial abdominal pain and weight loss. Upper gastrointestinal (UGI) series demonstrated SMA obstruction of the third portion of the duodenum. Despite nasojejunal tube feedings over 4months with appropriate weight gain, symptoms continued and repeat UGI demonstrated persistent SMA syndrome. A laparoscopic Ladd's procedure served as definitive treatment. The steps of the procedure include mobilization of the Ligament of Treitz, mobilization of the right colon, complete derotation of the duodenum, delivery of the small bowel to the right upper quadrant, and appendectomy. Following the procedure, a postoperative UGI showed complete resolution of SMA compression of the duodenum. The patient had an uneventful postoperative course with immediate resolution of symptoms. She was discharged home tolerating a regular diet.

BACKGROUND:The aim of this study was to examine national outcomes in newborn patients with esophageal atresia and tracheoesophageal fistula (EA/TEF) in the United Sates. METHODS:Kids' Inpatient Database (KID) is designed to identify, track, and analyze national outcomes for hospitalized children in the United States. Inpatient admissions for pediatric patients with EA/TEF for kids' Inpatient Database years 2000, 2003, 2006, and 2009 were analyzed. Patient demographics, socioeconomic measures, disposition, survival and surgical procedures performed were analyzed using standard statistical methods. RESULTS:A total of 4168 cases were identified with diagnosis of EA/TEF. The overall in-hospital mortality was 9%. Univariate analysis revealed lower survival in patients with associated acute respiratory distress syndrome, ventricular septal defect (VSD), birth weight (BW) < 1500 g, gestational age (GA), time of operation within 24 h of admission, coexisting renal anomaly, imperforate anus, African American race, and lowest economic status. Multivariate logistic regression identified BW < 1500 g (odds ratio [OR] = 4.5, P < 0.001), operation within 24 h (OR = 6.9, P < 0.001), GA <28 wk (OR = 2.2, P < 0.030), and presence of VSD (OR = 3.8, P < 0.001) as independent predictors of in-hospital mortality. Children's general hospital and children's unit in a general hospital were found to have a lower mortality rate compared with not identified as a children's hospital after excluding immediate transfers (P = 0.008). CONCLUSIONS:BW < 1500 g, operation within 24 h, GA < 28 wk, and presence of VSD are the factors that predict higher mortality in EA/TEF population. Despite dealing with more complicated cases, children's general hospital and children's unit in a general hospital were able to achieve a lower mortality rate than not identified as a children's hospital.

INTRODUCTION/BACKGROUND:Bilateral pulmonary sequestrations are rare congenital anomalies. Despite its benign nature, the potential complications of pulmonary sequestration (PS) are significant, including recurrent pulmonary infections, hemoptysis, congestive heart failure, and malignant potential. Therefore, the main treatment is surgical excision, even for patients with asymptomatic PS. CASE/METHODS:We present an infant in whom an intralobar PS of the right lung and an extralobar PS of the left lung were diagnosed on prenatal screening ultrasonography. Both were found to have venous drainage into the portal vein. Surgical excision was performed via video-assisted thoracic surgery (VATS) at 14 months of age. CONCLUSION/CONCLUSIONS:PS may present with unique vascular connections, including venous drainage into the portal vein. VATS resection for pulmonary sequestration is feasible and effective as an alternative to bilateral thoracotomies, in the setting of extensive preoperative planning and performance by an experienced thoracoscopic surgeon.

BACKGROUND:Population-based analysis of the incidence, demographics, and management outcomes in children with malignant tumors of the parotid gland. METHODS:Surveillance, Epidemiology, and End Results database (1973-2009) was researched for all patients younger than 20 years. RESULTS:Overall, 284 patients were identified. Annual incidence of these tumors was 1.43 cases per million. The highest incidence occurred in girls (0.86/1,000,000), black children (0.849/1,000,000), and adolescents (1.56/1,000,000). Median age at diagnosis was 13.5 years. Most patients were 10 years or older (n = 256, 90%). Most patients presented with local disease (n = 207, 76%). Only 3% had metastasis at time of diagnosis. Most tumors were mucoepidermoid carcinomas (n = 139, 49%) or acinar cell carcinomas (n = 113, 40%). There were no differences in survival between mucoepidermoid and acinar cell carcinomas (96% vs 98% respectively, P = 0.317). Overall mortality was 4.6% over the study period. Overall survival was 96% at 5 years, 95% at 10 years, and 83% at 20 years. Adolescents had significantly higher mortality rates (7.1% vs 1.6% for children <15 years of age, P = 0.23). Multivariate analysis identified the use of adjuvant radiation therapy (hazard ratio, 6.01; 95% confidence interval, 1.15-31.45; P = 0.034) as the only independent predictor of poor outcome. CONCLUSIONS:Malignant parotid gland tumors are most common in adolescents, and this subgroup has worse outcomes. The role of radiotherapy remains controversial.

We report an otherwise well developing 2-year-old girl who presented with a urachal sinus with its opening located at the midline between umbilicus and the pubic symphysis. Diagnosis was made by ultrasound preoperatively, and confirmed during surgery. Additionally, columnar epithelium was found in the portion of sinus tract traversing the abdominal wall. Metaplasia of the sinus tract is worrisome, as urachal carcinomas have been traditionally characterized to result from this process.

BACKGROUND:The inferior pedicle technique remains the most popular approach to breast reduction in the United States. Modifications to this procedure have enhanced versatility, patient safety, and outcome satisfaction in patients with all degrees of macromastia. METHODS:A 6-year retrospective review of 241 patients who underwent bilateral inferior pedicle breast reduction was conducted at our institution. Modifications analyzed included methylene blue tattooing to provide preoperative landmarks, preoperative hydrodissection to reduce intraoperative blood loss, incorporation of inframammary darting to reduce tension at the "T-junction," preservation of superomedial volume for enhanced medial fullness, and dermatome blade-guided tissue resection. RESULTS:Inframammary darting reduced the incidence of wound dehiscence. Preoperative hydrodissection reduced intraoperative blood loss by a factor of 2. Dermatome blade use reduced operative times at no increased incidence of postoperative seromas or hematomas. CONCLUSIONS:Outcomes resulting from these modifications appear to be at least comparable to, and perhaps better than, those previously reported.