James M. DeCou

Successful Ex Utero Intrapartum Treatment (EXIT) Procedure for Congenital High Airway Obstruction Syndrome (CHAOS) Owing to Laryngeal Atresia

Congenital high airway obstruction syndrome (CHAOS) caused by laryngeal atresia was diagnosed by prenatal ultrasound in a male fetus at 18-weeks-gestation. Findings included enlarged lungs, inverted diaphragms, dilated trachea distal to the obstruction, and ascites. At 35 weeks gestation, a planned ex utero intrapartum treatment (EXIT) procedure was performed, allowing bronchoscopic evaluation of the airway and placement of a tracheostomy. The postnatal course was marked by gradual recovery of diaphragmatic function with corresponding weaning from conventional ventilation to positive airway pressure. The sustained improvement in diaphragmatic and pulmonary function after the EXIT procedure for laryngeal atresia is one of the first reported for this entity and provides encouragement for future attempts.

Feeding Roux-en-Y jejunostomy in the management of severely neurologically impaired children

Feeding problems are legion in severely neurologically impaired children. Many patients do well with a gastrostomy with or without fundoplication. Unfortunately, fundoplication is not without complication, emphasizing the need for other options in the management of these difficult patients. Since 1990 six patients (age range, 2 months to 6 years) have been treated by the creation of a permanent Roux-en-Y end-jejunostomy and a gastrostomy. The Roux limb is closed tightly around a mushroom catheter. The gastrostomy is left for gastric decompression, and the jejunostomy tube is used for all nutrition and most medications. One patient died in the postoperative period. All the others have done well, without significant complication and with a high level of parent satisfaction (average follow-up, 12 months). Previously four other patients were treated with a Roux-en-Y jejunostomy in which, rather than closing the Roux limb around the catheter, it was brought out as a catheterizable stoma. Frequent leakage and prolapse make this approach less desirable.

Infants With Metastatic Neuroblastoma Have Improved Survival With Resection of the Primary Tumor

Although infants (age less than 1 year) with neuroblastoma have a favorable overall prognosis, metastatic disease is associated with poorer treatment outcome. To assess the role of surgery in these patients, the authors reviewed survival data for 151 infants treated for neuroblastoma, focusing on patient and tumor characteristics, biological markers, and surgical management among the 99 patients with metastatic disease. Patients were divided into early (1961 to 1978) and contemporary (1979 to 1993) treatment eras. Potential prognostic factors were statistically tested to determine their significance in affecting survival. Five-year survival by Pediatric Oncology Group stage was: A, 100% (+/- 0%); B, 94% (+/- 6%); DS, 77% (+/- 9%); C, 73% (+/- 9%); and D, 61% (+/- 8%). Survival for infants with metastatic disease (stages C, D, and DS) was affected significantly by treatment era (P = .0001). Analyses restricted to patients treated during the contemporary era showed prognostic significance for DNA index (P = .02), N-myc copy number (P = .007), serum lactate dehydrogenase level (P = .001), and extent of resection (P = .01). A > or = 95% resection of the primary tumor was found to be associated with improved survival. Significantly more surgical complications were associated with resections performed at the time of diagnosis (P = .007), and delaying surgery until after several courses of chemotherapy did not decrease survival. In conclusion, multiple factors affect the outcome of treatment for infants with metastatic neuroblastoma, and whenever feasible, a > or = 95% resection of the primary tumor should be performed in this patient subgroup.

Pediatric bronchogenic cyst complicated by atypical mycobacterium infection: a case report

IntroductionBronchogenic cysts are lesions of congenital origin derived from the primitive foregut and are the most common primary cysts of the mediastinum. They are most frequently unilocular and contain clear fluid. Respiratory distress is the most common presentation in pediatric patients, manifested by recurring episodes of cough, stridor, wheezing and retractions.Case presentationWe report the first pediatric case of bronchogenic cyst complicated by atypical Mycobacterium infection. This case describes a 13-year-old Caucasian American female with a large cystic lesion and extensive pulmonary involvement. Pathology studies revealed necrotizing granulomatous inflammation, multiple nodules, and acid-fast bacilli. She was successfully treated with surgical excision and a six-week course of clarithromycin, rifampin and ethambutol. Other unusual aspects of this case include multilocular intraparenchymal cyst appearance, its turbid drainage, and late symptom onset.ConclusionBronchogenic cyst should be included in the differential diagnosis of a child with cough, dyspnea, and fever. Although rare, we stress the importance of keeping mycobacterial infection in mind in cases of an infected cyst. Acid-fast culture should be done on sputum and cyst contents. Due to the frequency of negative cultures, stains should also be performed on resected cyst specimens. Antibiotic therapy should be considered and administered based on the extent of infection. All symptomatic or enlarging cysts warrant surgical excision. Prophylactic removal of asymptomatic cysts is recommended due to higher rates of perioperative complications once cysts become symptomatic.

Continuous hemoglobin monitoring in pediatric trauma patients with solid organ injury

BACKGROUND/PURPOSEnHemoglobin monitoring is required in pediatric trauma patients with solid organ injury. We hypothesized that noninvasive hemodynamic monitoring (NIHM) represents an effective, safe alternative to laboratory hemoglobin (LabHb) monitoring in clinically stable patients.nnnMETHODSnA retrospective cohort study was conducted regarding pediatric trauma patients (<18 years old) with blunt solid organ injury over six consecutive months. Continuous NIHM was initiated at the time of admission, and LabHb measurements were obtained per institutional guidelines. Measurements were correlated within two hours of assessment and patient outcomes were analyzed.nnnRESULTSnTwenty-one patients met inclusion criteria and had evaluable data. Blunt trauma was the exclusive mechanism of injury, and mean injury severity score was 16.6 for the cohort. Bland Altman analysis showed an average deviation of 0.80 g/dL between NIHM and LabHb values for all data pairs. Measurement trends were highly correlated in patients with stable hemoglobin levels and those requiring blood transfusion.nnnCONCLUSIONSnNIHM demonstrated clinically acceptable accuracy when following hemoglobin trends in the defined pediatric trauma patient population. Slight variances between NIHM and LabHb values were occasionally noted, but did not affect clinical management. Continuous NIHM represents a potentially valuable adjunct to traditional laboratory hemoglobin monitoring.nnnLEVEL OF EVIDENCE RATINGnIV.

Benefits of Transperitoneal Approach to Bilateral Pretransplant Laparoscopic Nephrectomies in Pediatric Patients

INTRODUCTIONnPediatric renal transplant patients may require native nephrectomy to avoid complications at the time of kidney transplantation. We have performed unilateral and bilateral transperitoneal pretransplant laparoscopic nephrectomies (PLNs), followed by living-related renal transplantation. The benefits of transperitoneal versus retroperitoneal dissection remain controversial.nnnSUBJECTS AND METHODSnA retrospective review of patients who underwent unilateral or bilateral transperitoneal PLNs between May 1, 2001 and April 30, 2009 was performed. A transperitoneal approach was used in all patients.nnnRESULTSnEight patients underwent transperitoneal PLN. Four patients were female, and the average age was 91 months (range, 9-199 months). Five of the cases involved bilateral nephrectomy. Average operating times, including hemodialysis catheter placement and other procedures, was 4.93 hours (range, 4.25-5.97 hours) for bilateral PLN and 3.93 hours (range, 2.57-5.48 hours) for unilateral PLN. The average hospital stay was 5.9 days. All patients underwent successful renal transplantation following PLN without rejection at an average 2.3 years of follow-up.nnnCONCLUSIONSnPLN appears to be an effective, safe method of removing potentially problematic, diseased kidneys prior to planned renal transplantation. A transperitoneal technique using four midline ports affords excellent access to both kidneys and, more importantly, allows for additional procedures to be completed simultaneously.