Richard R. Ricketts

RUPTURE OF THE AIRWAYS FROM BLUNT TRAUMA: TREATMENT OF COMPLEX INJURIES

Tracheobronchial rupture from blunt trauma is usually single and transverse but may be longitudinal or complex, a combination of various sites and forms of rupture. From 1970 to 1990, 183 cases of rupture of the airways were reported in the literature: 136 (74%) transverse, 33 (18%) longitudinal, and 14 (8%) complex. During the same 20 years at Grady Memorial Hospital, 6 patients with such injuries were treated. One had complex injury consisting of rupture of the distal trachea and both main bronchi, 1 had a longitudinal tracheal rupture and rupture of the innominate artery, and 4 had a transverse rupture, 1 of whom also had a traumatic false aneurysm of the left pulmonary artery. Cardiopulmonary bypass was used only for the repair of the complex injury, whereas the repair of the left main bronchial rupture associated with a false aneurysm of the left pulmonary artery was done with standby cardiopulmonary bypass. All 6 patients had satisfactory results from the correction of their lesions except 1 child in whom stenosis developed at the rupture site. This study suggests that complex injuries are rarely seen, and their repair is often quite involved. In some of these cases, the use of cardiopulmonary bypass increases the margin of safety during operation and may encourage repair rather than resection of the affected lung.

Interferon-alpha-2a for the treatment of complex hemangiomas of infancy and childhood.

ObjectiveThe authors describe the use of interferon–alpha-2a (IFN–α-2a) in the treatment of complex hemangiomas and review the role of interferon (IFN) in this example of an angiogenic disease. Summary Background DataHemangiomas are the most frequent tumors of infants and children. They grow rapidly for 6 to 8 months and then resolve over a period of years. Approximately 5% produce life-, sight-, or limbthreatening complications, with mortality rates between 20% and 50%. Aggressive therapy with steroids, arterial ligation or embolization, or surgery has been used in these situations with variable results and high morbidity. Recently, IFN–α was found to be effective treatment in these complex hemangiomas. MethodsFour infants and one child were treated with IFN–α-2a at an initial subcutaneous dose of 1 million units/m2/day and a sustained dose of 3 million units/m2/day for 5 to 11 months. Appropriate laboratory values were monitored and adverse reactions and ultimate response to therapy were recorded. ResultsTwo patients experienced minor complications that were managed easily. Three patients had total or near-total regression of the hemangioma, one had partial (50%) regression, and one had stabilization but no regression after an average of 7.1 months of IFN therapy. ConclusionInterferon–α inhibits angiogenesis and endothelial cell migration and proliferation in vitro. The patients in this study add to the growing number who have benefited from IFN therapy. As such, IFN–α should be considered as a first-line agent in treating complex hemangiomas of infants and children.

Fetus-in-fetu with malignant recurrence

Fetus-in-fetu is an unusual condition in which a vertebrate fetus is enclosed within the abdomen of another fetus. These occurrences are usually benign. This report describes an instance of malignant recurrence after resection of a fetus-in-fetu.

Antireflux Surgery Outcomes in Pediatric Gastroesophageal Reflux Disease

OBJECTIVES:Antireflux surgery is performed frequently in children with gastroesophageal reflux disease (GERD). Few comparative studies exist which assess the indications for and short- or long-term outcome of open Nissen fundoplication (ONF) and laparoscopic Nissen fundoplication (LNF) for pediatric GERD. We investigated the frequency of reoperation and factors that might influence its occurrence.METHODS:We performed a retrospective, follow up cohort study of all children ≤5 years, who underwent LNF or ONF at our institution from January 1, 1997 to December 31, 2002, where five pediatric surgeons perform fundoplication. Mean follow up time was 36.2 months. The following information was obtained: surgical indications, hospital course data, and long-term surgical outcomes. Data were analyzed using univariate and multiple logistic regressions.RESULTS:Overall, 456 (150 [32.9%] ONF vs. 306 [67.1%] LNF) cases were analyzed. Reoperation was performed in 55 (12.06%), LNF 43 (14.05%), and ONF 12 (8%). The mean interim to reoperation for LNF was 11 months compared to 17 months for ONF (P = 0.007). The reoperation rate at 12 and 24 months were 10.5%, 13.4% and 4%, 6.7% respectively, when LNF was compared to ONF (P = 0.01). The multivariate analysis showed that initial LNF and prematurity were the main predictors for reoperation.CONCLUSIONS:The majority of reoperations for both LNF and ONF occurred in the first year after initial operation; LNF had a significantly higher reoperation rate than ONF. The probability of reoperation for LNF and ONF increases with the presence of comorbidities, especially prematurity and chronic respiratory conditions.

The anatomic pattern of biliary atresia identified at time of kasai hepatoportoenterostomy and early postoperative clearance of jaundice are significant predictors of transplant-free survival

Objective:The goals of this study were to describe the clinical and anatomic features of infants undergoing Kasai portoenterostomy (KPE) for biliary atresia (BA) and to examine associations between these parameters and outcomes. Methods:Infants enrolled in the prospective Childhood Liver Disease Research and Education Network, who underwent KPE were studied. Patients enrolled in a blinded, interventional trial were excluded from survival analysis. Primary endpoints were successful surgical drainage (total bilirubin less than 2 mg/dL within the first 3 months), transplant-free survival (Kaplan-Meier), and time to transplant/death (Cox regression). Results:KPE was performed in 244 infants (54% female; mean age: 65 ± 29 days). Transplant-free survival was 53.7% and 46.7% at 1 and 2 years post-KPE. The risk of transplant/death was significantly lower in the 45.6% of patients who achieved successful bile drainage within 3 months post-KPE (HR: 0.08, P < 0.001). The risk of transplant/death was increased in patients with porta hepatis atresia (Ohi type II and III vs type I; HR: 2.03, P = 0.030), nonpatent common bile duct (Ohi subtype: b, c, and d vs a; HR: 4.31, P = 0.022), BA splenic malformation syndrome (HR: 1.92, P = 0.025), ascites > 20 mL (HR: = 1.90, P = 0.0230), nodular liver appearance compared to firm (HR: = 1.61, P = 0.008), and age at KPE ≥ 75 days (HR: 1.73, P < 0.002). Outcome was not associated with gestational age, gender, race, ethnicity, or extent of porta hepatis dissection. Conclusion:Anatomic pattern of BA, BASM, presence of ascites and nodular liver appearance at KPE, and early postoperative jaundice clearance are significant predictors of transplant-free survival.

Enterostomy in necrotizing enterocolitis: An analysis of techniques and timing of closure

Resection and enterostomy are the standard operative procedures for necrotizing enterocolitis (NEC). In order to compare the results of two different methods of enterostomy, a study was carried out in 100 infants with NEC who underwent enterostomy formation and closure. A single surgeon at each of the two collaborating institutions conducted the majority of operations. Level of enterostomy was jejunum in 10, ileum in 75, and colon in 15. Type of enterostomy was separate stomas (usually brought out side by side) in 50, Mikulicz enterostomy in 39, single stoma with Hartmanns pouch in 10, and loop colostomy in 1. Complications of enterostomy formation occurred in 24 infants (24%). When infants with separate stomas were compared with those with the Mikulicz enterostomy, there was no difference in the rate of stomal or wound complications. The separate stomas had a higher rate of stricture formation in the distal bowel (36% v 18%), which may be accounted for by earlier reestablishment of intestinal continuity in the Mikulicz group. Both methods exteriorized the bowel ends close to one another, which was advantageous because subsequent closure was usually performed without a formal laparotomy. After enterostomy closure, 17 (17%) infants had complications. There was no difference in complication rate between early (before 3 months or under 2.5 kg) v late closure, or between closure of the Mikulicz enterostomy v separate stomas (although the Mikulicz enterostomy closure was accomplished more rapidly than closure of separate stomas). Morbidity was unrelated to level of enterostomy, type of enterostomy, maturing the stoma, bringing it through a separate incision, or age or weight of the infant at closure.(ABSTRACT TRUNCATED AT 250 WORDS)

Management of biliary tract stones in heart transplant patients

OBJECTIVE The authors report their experience with biliary tract stones in adult and pediatric heart transplant patients, and review the current literature relative to this problem. SUMMARY BACKGROUND DATA Prior studies in adults have noted that heart transplant patients frequently have cholelithiasis, but offer no consensus about treatment strategy. Few studies exist for pediatric heart transplant patients. A higher rate of hemolysis and cyclosporine-induced changes in bile metabolism may contribute to lithogenesis in this population. METHODS A chart review was conducted for 211 patients who had heart transplants between January 1988 and September 1994 to determine prevalence of biliary tract stones, management strategies used, and outcome. RESULTS Of 175 long-term heart transplant survivors, 52 (29.7%) had stones detected: 32.8% of adults (47/143) and 15.6% of children (5/32). The majority of patients (31) were diagnosed 4 months (mean) after transplantation; cholelithiasis developed in 10 of these patients (32%) within 11 months (median) after a negative ultrasound. Symptoms developed in 45% of patients. All patients underwent either elective (36) or urgent (6) cholecystectomy via an open (32) or laparoscopic (10) approach, or endoscopy for common bile duct stones (2). There were no deaths or complications during a follow-up period of up to 7 years. CONCLUSION Heart transplant patients have a high prevalence of symptomatic biliary tract stone disease. They can be treated safely via an open or laparoscopic approach after transplantation. The authors recommend routine gallbladder ultrasound screening and elective cholecystectomy in the post-transplant period if stones are detected.

NUT Midline Carcinoma in a Newborn with Multiorgan Disseminated Tumor and a 2-Year-Old with a Pancreatic/Hepatic Primary

NUT midline carcinoma (NMC) is a rare and aggressive malignant epithelial tumor defined by rearrangement of the NUT gene on chromosome 15. In two thirds of cases, NUT is involved in a balanced translocation with BDR4 on chromosome 19, while in the remaining cases, NUT is rearranged with variant fusion partners such as BRD3. These undifferentiated tumors primarily affect midline structures, usually in the upper aerodigestive tract and mediastinum. Most reported cases have followed a rapidly lethal clinical course. We report the clinical and pathological findings of NMC in the youngest patients identified so far. The 1st case involves a newborn who presented with a supraorbital mass and extensive multiorgan involvement, including the spine, lungs, liver, pancreas, adrenal glands, and subcutaneous tissue. The 2nd patient was a 2-year-old male with an abdominal mass involving the liver and pancreas with pulmonary metastasis. Histopathological analysis of both tumors showed undifferentiated malignant neoplasms, and immunohistochemistry showed positivity for epithelial markers. Both tumors demonstrated t(15;19), and immunohistochemistry with NUT monoclonal antibodies and fluorescent in situ hybridization confirmed NUT rearrangement. The patients died from disease at 1 and 2 months postpresentation. Thus far, 25 cases have been reported, including our 2 current cases. Presentation ages range from 0 to 78 years (mean, 23 years). Herein, we report the 2 youngest reported cases of NMC, including the 1st congenital case and the 1st case arising within the liver/pancreas. Increased awareness and further molecular studies are required for a better understanding of NMC pathobiology and improved therapeutic outcomes.

Surgical therapy for necrotizing enterocolitis.

Fifty-one infants were treated surgically for necrotizing enterocolitis utilizing a uniform protocol from July 1980 through July 1983. The indications for surgery were pneumoperitoneum or a paracentesis indicative of bowel infarction. Segmental intestinal resection and exteriorization of the bowel ends through the upper abdominal transverse incision was the usual procedure. Intestinal continuity was reestablished when the patient reached 10 pounds, or sooner if he was failing to thrive with his ileostomy. The overall survival was 72.5%, and it was 82% for those patients have a definitive surgical procedure. This survival rate was not adversely affected by the patients weight or age at the time of operation, nor by the presence of bowel perforation. The 37 survivors endured multiple postoperative complications. The most significant long-term sequela was short-gut syndrome, which occurred in 11% of survivors.

Circular esophagomyotomy for primary repair of long-gap esophageal atresia

Twelve of forty patients (30%) treated for esophageal atresia at the Childrens Memorial Hospital in Chicago between June 1976 and May 1980 required a circular esophagomyotomy with or without upper pouch bougienage to achieve an anatomical repair. Ten patients had a distal tracheoesophageal fistula; 1 had a proximal fistula as well; and 2 had no fistula. Over 40% of the patients were small for gestational age. One half had severe associated anomalies including congenital heart disease in 1/3 and the VATER association in 1/4. Three patients died, all from congenital heart disease. Immediate complications in 6 patients included anastomotic leaks (3), tracheal injuries (2), and mucosal entry at the myotomy site (1). Late complications included symptomatic gastroesophageal reflux (60%), anastomotic strictures (40%), and severe tracheomalacia requiring long-term tracheotomy (20%). Altered esophageal motility (77%) contributed to poor weight gain (less than 3rd percentile) in most patients. In spite of these complications, satisfactory results were achieved in 8 of the 9 long-term survivors. The ninth patient had a turbulent neonatal period and has persistent swallowing difficulties at 36 mo follow-up. Although an anatomical repair can now be achieved in all patients with esophageal atresia utilizing bougienage and myotomy, some ill neonates may be better served by traditional staging techniques.