Faculty Bibliography

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:Patent ductus arteriosus (PDA) is a condition that commonly affects premature and low birth weight (BW) infants at times necessitating surgical intervention. We examined outcomes after surgical ligation (SL). MATERIALS AND METHODS/METHODS:We analyzed the Kids' Inpatient Database for premature infants diagnosed with PDA, admitted at <8 d of age. Patient demographics, disposition, morbidity, and mortality were analyzed. All cases were weighted appropriately to project nationally representative estimates. RESULTS:A total of 63,208 patients were identified with diagnosis of PDA. Of these, 6766 (10.7%) underwent SL. Lower gestational age (GA) and BW patients had higher incidence of PDA and rates of SL. Overall survival was 90.8% for the cohort. Survival for the SL group was 88.0% and 91.2% for the non-SL group; however, infants undergoing SL had higher survival rates up to 28 wk and 1250 g for GA and BW, respectively. GA did not affect post-SL survival adversely. Rather, lower BW was associated with extremely high mortality rates. Black infants and boys had lower survival compared with other races and girls, respectively. Larger hospitals had higher survival rates, but hospital location, teaching status, and type did not affect survival. Payer status and income quartile did not affect survival. CONCLUSIONS:PDA and SL are more common in lower BW and GA groups. Higher survival rates are found for infants with SL versus non-SL in the lowest BW and GA groups. Morbidity and mortality are not affected by SL timing. BW, rather than GA, determines survival of infants undergoing SL.

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.

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: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.

BACKGROUND:Histiocytoses are rare disorders affecting the pediatric population. MATERIALS AND METHODS/METHODS:Surveillance, Epidemiology, and End Results database was searched for pediatric cases (<20 y old) of histiocytosis diagnosed between 1973 and 2010. Demographics, clinical characteristics, and survival outcomes were analyzed using standard statistical methods. Class I disease (Langerhans cell histiocytosis) and class III (malignant histiocytosis) were included in the data set. RESULTS:A total of 828 cases were identified. Overall incidence was 0.142/100,000 persons per annum. Incidence was highest in younger children and those of Asian or Native American descent. Class III disease had a higher incidence versus class I. Adolescents tended to present with class III, whereas young children presented with class I. Disseminated disease was present in most cases of class III, whereas class I had more localized cases. Surgical excision was more likely to be performed in class I. Overall median survival was 349 mo. Patients 15-19 y old and children<1 y old had the worst outcomes. Class I had higher survival compared with class III, which had a median survival of 33 mo. Cases with hematologic spread carried the worst prognosis. Surgical excision conferred a survival advantage while radiation had no effect. Survival improved over the study period. Gender and race had no association with survival. CONCLUSIONS:Class I disease had localized cases and showed benefit from surgical intervention. Class III disease had a higher incidence and was associated with disseminated disease and lower survival. Radiation therapy did not affect survival. Overall survival increased over the previous 40 y.

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.

The chief therapeutic mechanism of fingolimod in multiple sclerosis (MS) is considered to be sequestration of pathogenic lymphocytes into secondary lymphoid tissues. B cells have recently been recognized as important immune regulators in MS. In this study, the effects of fingolimod on B cells in MS patients were analyzed. MS patients treated with fingolimod (MS-F) had a significantly lower number of B cells in the circulation. The remaining B cells in the blood of MS-F had a reduced proportion of memory B cells and an increased proportion of naïve B cells, expressed lower levels of the costimulatory molecule CD80, and produced less tumor necrosis factor-α and more interleukin-10. These observations in MS-F were based on an increased proportion of the transitional B-cell subpopulation within the naïve B-cell compartment. The observed findings in B cells of MS-F might be related to the therapeutic effect of this drug in MS.

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.