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: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.

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.

PURPOSE/OBJECTIVE:Initial hematocrit (Hct) is generally not considered a marker of acute blood loss because it is assumed that physiologic response of fluid conservation to hemorrhage is delayed. We challenged this idea by theorizing that admission Hct correlates with conventional signs of shock and predicts the use of blood transfusion during resuscitation of pediatric trauma patients. METHODS:Data from 1928 pediatric admissions (<18 years) at a Level I trauma center (2000-2012) were compared using standard statistical analyses and logistic regression modeling to identify factors associated with blood transfusion during initial trauma resuscitation. RESULTS:Overall mortality rate was 3.5%, with a transfusion rate of 10.7%. Factors significantly associated with transfusion were initial Hct, Glasgow Coma Score, base deficit, and injury severity score (all p<0.001). Initial Hct is a stronger predictor for transfusion (area under receiver operator curve (AUC: 0.728) compared to age-specific tachycardia (AUC: 0.689), age-specific hypotension (AUC: 0.673), and altered mental status (AUC: 0.654)). On multivariate analysis, initial Hct was an independent predictor (OR [95% CI]: 2.94 [1.56, 5.52]) along with hypotension (6.37 [2.95, 13.8]), base deficit (4.14 [1.38, 12.4]), and tachycardia (3.07 [1.62, 5.81]). CONCLUSIONS:Initial Hct correlates significantly with conventional signs of shock and is a strong independent predictor for blood transfusion with better predictability than other clinical factors.

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: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.

We report an elderly patient with maternally inherited diabetes with deafness (MIDD). A 69-year-old woman was found to be diabetic for the first time when she visited her local medical doctor for the symptoms of a common cold. Her casual plasma glucose level was 311 mg/dl and HbA1c was 8.3%. She had been aware of muscle atrophy of the lower extremities and hearing disturbance since age 66. As for her family history, her mother, older sister and younger brother were diabetic with hearing difficulty and all of them had died suddenly in their middle age. Her 45-year-old daughter was also diabetic with some difficulty in hearing. Therefore, we suspected both the patient and her daughter had MIDD, and found alterations in mitochondrial DNA3243A-G. MIDD is a condition that needs to be diagnosed accurately and treated at an early stage, since diabetic complications can progress rapidly and could cause myocardial complications and mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS). According to a report of 115 cases of MIDD in Japan, MIDD had been diagnosed at the age of 32.8 on average and our case was strikingly old for the age of onset of the disease.