Karen W. West

Long-term analysis of children with esophageal atresia and tracheoesophageal fistula

BACKGROUND/PURPOSE For children with esophageal atresia (EA) or tracheoesophageal fistula (TEF), the first years of life can be associated with many problems. Little is known about the long-term function of children who underwent repair as neonates. This study evaluates outcome and late sequelae of children with EA/TEF. METHODS Medical records of infants with esophageal anomalies (May 1972 through December 1990) were reviewed. Study parameters included demographics, dysphagia, frequent respiratory infections (> 3/yr), gastroesophageal reflux disease (GERD), frequent choking, leak, stricture, and developmental delays (weight, height < 25%, < 5%, respectively). RESULTS Over 224 months, 69 infants (37 boys, 32 girls) were identified: type A, 10 infants; type B, 1; type C, 53; type D, 4; type E, 1. Mean follow-up was 125 months. During the first 5 years of follow-up, dysphagia (45%), respiratory infections (29%), and GERD (48%) were common as were growth delays. These problems improved as the children matured. CONCLUSIONS Children with esophageal anomalies face many difficulties during initial repair and frequently encounter problems years later. Support groups can foster child development and alleviate parent isolationism. Despite growth retardation, esophageal motility disorders, and frequent respiratory infections, children with EA/TEF continue to have a favorable long-term outcome.

Increased risk of necrotizing enterocolitis in premature infants with patent ductus arteriosus treated with indomethacin

OBJECTIVE The authors evaluated the risk of necrotizing enterocolitis (NEC) in very low birth weight infants receiving indomethacin (INDO) to close patent ductus arteriosus (PDA). BACKGROUND DATA Controversy exists regarding the best method of managing very low birth weight infants with PDA and whether to employ medical management using INDO or surgical ligation of the ductus. METHODS Two hundred fifty-two premature infants with symptomatic PDA were given intravenously INDO 0.2 mg/kg every 12 hours x 3 in an attempt to close the ductus. Patients were evaluated for sex, birth weight, gestational age, ductus closure, occurrence of NEC, bowel perforation, and mortality. RESULTS There were 135 boys and 117 girls. The PDA closed or became asymptomatic in 224 cases (89%), whereas 28 (11%) required surgical ligation. Ninety infants (35%) developed evidence of NEC after INDO therapy. Fifty-six were managed medically; surgical intervention was required in 34 of 90 cases (37.8%) or 13% of the entire PDA/INDO study group. Bowel perforation was noted in 27 cases (30%). Factors associated with the onset of NEC included gestational age < 28 weeks, birth weight < 1 kg, and prolonged ventilator support. The overall mortality rate was 25.5%, but was higher in infants with NEC versus those without. The highest mortality was noted in perforated NEC cases. The PDA/INDO patients were compared with a control group of 764 infants with similar sex distribution, birth weights, and gestational ages without PDA who did not receive INDO. Necrotizing enterocolitis occurred in 105 of 764 control patients (13.7%), including 13 (12.3%) with perforation. The overall mortality rate of controls was 25%, which was similar to the overall 25.5% mortality rate in the PDA/INDO study group. CONCLUSION These data indicate that there is increased risk of NEC and bowel perforation in premature infants with PDA receiving INDO. Mortality was higher in the PDA/INDO group with NEC than those PDA/INDO infants without NEC.

Complications and reoperation after Nissen fundoplication in childhood

Over a 10 year period, 429 Nissen fundoplications were performed on children with gastroesophageal reflux. Postoperative complications occurred in 69 children (16 percent), including wrap herniation or breakdown in 29; postoperative bowel obstruction in 18; stricture in 10; intraabdominal abscess and enterocutaneous fistula in 3 patients each; and wound infection, wound dehiscence, and inadvertent splenectomy in 2 patients each. The postoperative mortality rate was 0.9 percent (4 of 429 patients) and was related to sepsis in 1 patient, a metabolic disorder in 1 patient, and underlying pulmonary disease in 2 patients. All four patients were neurologically impaired. Fundoplication successfully controlled symptoms of gastroesophageal reflux in 395 children (92 percent) over a follow-up period ranging from 6 months to 10 years. Thirty-eight patients (8.8 percent) required a second antireflux operation because of recurrent symptoms. Twenty-nine patients had severe neurologic impairment (76 percent), 5 had associated congenital malformations (13 percent), and 3 had significant pulmonary problems (8 percent). Only one child requiring reoperation was considered otherwise normal. Indications for reoperation included wrap breakdown or herniation (28 patients), stricture (6 patients), and inadequate wrap (4 patients). Twenty-four of 28 children with wrap herniation or breakdown had neurologic impairment. A second fundoplication was successful in 35 of 38 patients (92 percent). A second procedure failed in three children, who required subsequent resection and colon interposition.

Short bowel syndrome in infancy and childhood: Analysis of survival in 60 patients☆

This report concerns 60 infants and children with short bowel syndrome, most commonly caused by necrotizing enterocolitis in this study. Resection of atretic or gangrenous bowel was performed in 53 patients, tapering enteroplasty and primary anastomosis was performed in 13 patients, and temporary enterostomies were performed in 40 patients. Second-look laparotomy was useful in two of four cases of questionable bowel viability. The ileocecal valve was resected in 33 patients and remained intact in 27. The mean length of remaining bowel was 58.4 cm (range 13 to 150 cm). Seven patients with total aganglionosis and mid to proximal small bowel extension were managed with an initial enterostomy, whereas three had a pull-through procedure with an aganglionic patch enteroplasty. All patients received total parenteral nutrition and early enteral feedings. Home hyperalimentation was attempted when 50 percent of the calorie intake was enteral. Intestinal adaptation required from 3 to 14 months. Frequent setbacks were related to catheter sepsis, rotavirus infection, carbohydrate intolerance, and liver dysfunction. The overall survival rate was 85 percent, with mortality due to liver failure and sepsis associated with total parenteral nutrition.

Diagnosis and treatment of symptomatic breast masses in the pediatric population

Between 1980 and 1993, 74 children and adolescents were referred for surgical evaluation of palpable breast masses. Thirty-two were managed nonoperatively for unilateral thelarche (26), fibroadenoma (3), gynecomastia (2), or hemorrhagic cyst (1). The other 42 children had surgical intervention for giant or painful fibroadenomas (19), breast abscesses (5), painful gynecomastia (6), metastatic disease (4), or other conditions (8). No instances of primary breast malignancy were noted. Physical examination and minimal (selective) diagnostic testing can conserve health care dollars in cases of pediatric patients with breast masses.

Inguinal hernia in children: Factors affecting recurrence in 62 cases☆

This report analyzes factors associated with 71 recurrent inguinal hernias in 62 children treated between 1976 and 1988. Cases were evaluated for sex, age, type of initial repair, interval to recurrence, the presence of comorbid conditions, and type of reoperation. There were 57 boys and 5 girls. Sixty percent of patients were less than 6 months old and 72% were less than 1 year of age at the time of the initial repair. Recurrence was on the right in 74%, left in 24%, and bilateral in 2%. Recurrence was noted by 6 months in 50%, by 2 years in 76%, and by 5 years in 96%. Comorbid conditions were present in 60% of cases, including increased intraabdominal pressure (ventriculoperitoneal [VP] shunts), growth failure, prematurity, chronic pulmonary disease, bladder exstrophy, connective tissue disorders, cryptorchism, seizure disorder, and malnutrition. Incarceration was a factor in four of the 62 cases. Seven patients had multiple recurrences. Fifty-one recurrences were indirect, whereas 20 were direct inguinal hernias. Inadequate high ligation (three with chromic catgut), wound infection, and groin hematoma were other findings. The direct hernias may be related to injury to the floor of the canal during initial repair. Recurrent repair included high ligation of the sac alone (20), high ligation plus snugging of a large internal ring (11), and high ligation with repair of the iliopubic tract in patients with VP shunts, connective tissue disorder, or weak floor (20). All direct hernias had a Coopers ligament (McVay) repair. Two direct hernias recurred again and were successfully repaired using a preperitoneal approach.(ABSTRACT TRUNCATED AT 250 WORDS)

Is the Grass Greener? Early Results of the Nuss Procedure

BACKGROUND/PURPOSE Minimal access surgery (MIS, Nuss Procedure) is gaining acceptance rapidly as the preferred method for pectus excavatum repair. This shift in operative management has followed a single institutions evaluation of the procedure. This report describes an additional experience with the Nuss procedure. METHODS Twenty-one patients with pectus excavatum underwent repair by the Nuss Procedure. The patients ranged in age from 5 to 15 years (average, 8.2 years). There were 19 boys and 2 girls. RESULTS In 1 patient (age 5 years) the MIS procedure was aborted because of persistence of chest wall asymmetry. The other 20 patients had completion of their procedure without intraoperative complication. The operating times ranged from 45 to 90 minutes; however, there was an additional anesthetic set-up time (average, 45 minutes). All cases utilized a single support bar (11 to 17 inches). Patients underwent extubation in the operating room and were admitted to a ward bed with an epidural catheter in place for pain control and received intravenous analgesia. The hospital stay ranged from 4 to 11 days and averaged 4.9 days. Early postoperative complications included ileus (n = 1), bilateral pleural effusion (n = 2), atelectasis (n = 1), fungal dermatitis (n = 1), pneumothorax (n = 1), and flipped pectus bar (n = 2). Delayed complications included flipped pectus bar (n = 2), marked pectus carinatum requiring bar removal (n = 1), mild carinatum (n = 1), mild bar deviation (n = 1), progressive chest wall asymmetry (n = 3) with 1 requiring bar removal and open pectus repair, pleural effusion (n = 1), and chronic persistent pain requiring bar removal (n = 1). The length of follow-up is 3 to 20 months with an average of 12.3 months. CONCLUSIONS The Nuss Procedure is quick, minimally invasive, and a technically easy method to learn; however, our data indicate there is a significant learning curve. Although previous reports suggest that few complications occur, we believe further assessment of patient selection regarding age, presence of connective tissue disorder, and severe chest wall asymmetry are still needed. Long-term follow-up also will be required to assure both health professionals and the public that this is the procedure of choice for patients with pectus excavatum.

Delayed surgical repair and ECMO improves survival in congenital diaphragmatic hernia.

One hundred ten infants with congenital diaphragmatic hernia (CDH) developed life-threatening respiratory distress in the first 6 hours of life. Associated anomalies were present in 33%. Twenty-eight of 65 infants (43%) treated before 1987 (pre-extracorporeal membrane oxygenation [ECMO] era) survived after immediate CDH repair, and mechanical ventilation with or without pharmacologie support. Only two of 16 (12.5%) infants requiring a prosthetic diaphragmatic patch survived. Since 1987, 31 of 46 (67.4%) infants with birth weight, gestational age, and severity of illness similar to the pre-1987 group survived. All patients were immediately intubated and ventilated. Seven (four with lethal chromosomal anomalies) infants died before treatment, and 30 stabilized (partial pressure of carbon dioxide [Pco2] < 50; partial pressure of oxygen [Po2] > 100; pH > 7.3) and underwent delayed CDH repair at 5 to 72 hours. Fifteen did well on conventional support and survived. Fifteen infants deteriorated after operation: 11 were placed on ECMO with eight survivors, and four infants were not considered ECMO candidates. Nine babies failed to stabilize initially and were placed on ECMO before CDH repair (alveolar-arterial gradient > 600 and oxygenation index > 40), and seven survived. The overall survival rate was 80% at 3 months in this ECMO-treated group. Early mortality was due to inability to wean from ECMO (one), intracranial hemorrhage (one), liver injury (one), and pulmonary hypoplasia (one). Nine of 11 babies requiring a prosthetic patch in the post-1987 ECMO group survived (81.8%). There were three late post-ECMO deaths (3 to 18 months) of right heart failure (two) and sepsis (one). Symptomatic gastroesophageal reflux occurred in nine cases, six requiring a fundoplication in the bypass babies. Recurrent diaphragmatic hernia occurred in nine cases (five ECMO). The overall survival rate was significantly improved in the delayed repair/ECMO group (67% versus 43%; p < 0.05) and was most noticeable in infants requiring a prosthetic diaphragm (81.2% versus 12.5%; p < 0.005). These data indicate that early stabilization, delayed repair, and ECMO improve survival in high-risk CDH. Early deaths are related to pulmonary hypertension and can be reversed by ECMO.

Laparoscopic splenic procedures in children: experience in 231 children.

Objectives:The purpose of this report is to evaluate the efficacy of and complications observed after laparoscopic splenic procedures in children. Methods:Review of a prospective database at a single institution (1995–2006) identified 231 children (129 boys; 102 girls; average age 7.69 years) undergoing laparoscopic splenic procedures. Results:Two hundred twenty-three children underwent laparoscopic splenectomy (211 total; 12 partial) by the lateral approach. Indication for splenectomy was hereditary spherocytosis (111), immune thrombocytopenic purpura (36), sickle cell disease (SCD) (51), and other (25). Four (2%) required conversion to an open procedure. Eight additional laparoscopic splenic procedures were performed: splenic cystectomy for epithelial (4) or traumatic (2) cyst, and splenopexy for wandering spleen (2). Average length of stay was 1.5 days. Complications (11% overall, 22% in SCD patients) included ileus (5), bleeding (4), acute chest syndrome (5), pneumonia (2), portal vein thrombosis (1), priapism (1), hemolytic uremic syndrome (1), diaphragm perforation (2), colonic injury (1), missed accessory spleen (1), trocar site hernia (1), subsequent total splenectomy after an initial partial (1), and recurrent cyst (1). Subsequent operations were open in 3 (colon repair, hernia, and missed accessory spleen) and laparoscopic in 2 (completion splenectomy, and cyst excision). There were no deaths, wound infections, or instances of pancreatitis. Conclusions:Laparoscopic splenic procedures are safe and effective in children and are associated with low morbidity, higher complication rate in SCD, low conversion rate, zero mortality, and short length of stay. Laparoscopic splenectomy has become the procedure of choice for most children requiring a splenic procedure.

The efficacy of early ERCP in pediatric pancreatic trauma

Recognition of pancreatic injuries is frequently delayed, and optimal treatment is often controversial. The use of endoscopic retrograde cholangiopancreatography (ERCP) has allowed accurate delineation of pancreatic ductal injuries; however, the small size of children and the concern with inducing pancreatitis and/or lesser sac contamination have limited its use in children. In 1988, the authors began using ERCP for selected pancreatic injuries. This report describes their experience with this technique and examines the role of ERCP in pediatric pancreatic injuries. Six children with pancreatic transections resulting from blunt trauma were treated between 1988 and 1993. The age range was 2 1/2 to 8 years, and the weight range was 13.6 to 27.9 kg. The average period from injury to referral to the hospital was 14 days (range, 2 to 30 days). All six children presented with chemical evidence of pancreatitis and had an initial computed tomography (CT) scan; five scans were interpreted as being normal. Five of the six patients had subsequent CT scans, which showed lesser-sac fluid collection. Three patients were treated with drainage (2 percutaneous, 1 open [outside hospital]), and when this failed, ERCP was performed, at 13.6 days (average) after presentation. These three patients underwent ERCP relatively early in the course (an average of 3 days after presentation). All six children had major ductal transections documented through ERCP. After ERCP, the serum amylase level remained elevated in three, increased in one, and normal in one. (It was not measured in one of the recent cases taken for immediate operation.)(ABSTRACT TRUNCATED AT 250 WORDS)