Mark V. Mazziotti

Transanal one-stage soave procedure for infants with Hirschsprung's disease☆

PURPOSE Many centers perform a one-stage pull-through procedure for Hirschsprungs disease (HD) diagnosed in infancy. The authors have developed a one-stage pullthrough procedure using a transanal approach that eliminates the need for intraabdominal dissection. METHODS Nine children aged 3 weeks to 18 months with biopsy-proven HD underwent a transanal pull-through procedure over a 13-month period. A rectal mucosectomy was performed starting 0.5 cm proximal to the dentate line, and extending proximally to the level of the intraperitoneal rectum. In the first eight children, intraperitoneal position was confirmed with a laparoscope placed through a 3- to 5-mm port in the base of the umbilicus. The muscular sleeve was divided circumferentially to allow full-thickness mobilization of the rectosigmoid junction. Manual transanal traction permitted direct visualization and division of mesenteric vessels with transanal mobilization above the transition zone. Ganglion cells were confirmed by frozen section, and the bowel was transected. The rectal muscular cuff was divided longitudinally, and the anastomosis was completed. The laparoscope confirmed orientation and adequate hemostasis. In a ninth patient, the identical procedure was performed, but with the laparoscope used only for confirmation at the end of the procedure. RESULTS Operative time, including frozen sections, averaged 194 minutes (range, 169 to 250 minutes), and the average length of bowel resected was 12 cm (range, 7.5 to 22 cm). Four of the nine patients were discharged on postoperative day (POD) 1, four on POD 2, and one patient with Downs syndrome was discharged on POD 6. Median follow-up was 6 months (range, 3 to 14 months). One death occurred 2.5 months postoperatively secondary to sudden infant death syndrome. Complications included postoperative apnea spells (n = 1), mild enterocolitis (n = 2), constipation (n = 1), anastomotic stricture(n = 1), and muscularcuff narrowing (n = 1); each responded to nonoperative management. Stool output has ranged from four to eight per day. CONCLUSION A one-stage pull-through for HD can be performed successfully using a transanal approach without intraperitoneal dissection. This procedure is associated with excellent clinical results and permits early postoperative feeding, early hospital discharge, and no visible scars.

Intestinal rotation abnormalities without volvulus : The role of laparoscopy

BACKGROUND Intestinal rotation disorders may be discovered during investigation for abdominal symptoms. Two questions are raised in this setting: are the patients symptoms from the rotation abnormality, and is the base of the small bowel mesentery so narrow that it places the patient at risk for midgut volvulus? Previously, laparotomy was necessary to answer these questions, and then it was necessary to do a Ladd procedure and appendectomy if necessary. STUDY DESIGN We used laparoscopic surgery to evaluate seven patients, ages 4 days to 23 years of age (median age 7 years), when upper gastrointestinal series revealed intestinal rotation abnormalities without volvulus. RESULTS Two patients had nonrotation. One had Ladds bands across the duodenum that were divided, and the appendix was removed. The other had diffuse peritoneal soilage from a ruptured appendix; irrigation and appendectomy were performed. Three patients had duodenal malrotation and underwent laparoscopic Ladd procedure and appendectomy. Two patients had combined duodenal and cecal malrotation. One of these patients had a previous appendectomy for what in retrospect was primary peritonitis; malrotation was confirmed radiologically after the operation. She underwent a laparoscopic Ladd procedure 3 months later. The other patient was believed to have combined duodenal and cecal malrotation based on radiographic studies performed during workup for gastroesophageal reflux. At laparoscopy the small bowel mesentery was believed to have a broad enough base to prevent midgut volvulus, and an appendectomy was done. No patient required conversion to an open procedure. The sole complication was intra-abdominal abscess in the child with ruptured appendicitis that required prolonged hospitalization and operative abscess drainage. Operative times ranged from 1.25-3.25 hours (median 2 hours). Time to a regular diet was 1-20 days (median 2 days). Resolution of symptoms was seen in 5 of the 7 patients, with a median followup of 15 months. CONCLUSIONS Laparoscopy is an excellent technique for the evaluation and definitive management of patients without midgut volvulus with intestinal rotation abnormalities.

Clinical Characteristics and Risk Factors for Symptomatic Pediatric Gallbladder Disease

Objective: Our center previously reported its experience with pediatric gallbladder disease and cholecystectomies from 1980 to 1996. We aimed to determine the current clinical characteristics and risk factors for symptomatic pediatric gallbladder disease and cholecystectomies and compare these findings with our historical series. Study Design: Retrospective, cross-sectional study of children, 0 to 18 years of age, who underwent a cholecystectomy from January 2005 to October 2008. Results: We evaluated 404 patients: 73% girls; 39% Hispanic and 35% white. The mean age was 13.10 ± 0.91 years. The primary indications for surgery in patients 3 years or older were symptomatic cholelithiasis (53%), obstructive disease (28%), and biliary dyskinesia (16%). The median BMI percentile was 89%; 39% were classified as obese. Of the patients with nonhemolytic gallstone disease, 35% were obese and 18% were severely obese; BMI percentile was 99% or higher. Gallstone disease was associated with hemolytic disease in 23% (73/324) of patients and with obesity in 39% (126/324). Logistic regression demonstrated older age (P = .019) and Hispanic ethnicity (P < .0001) as independent risk factors for nonhemolytic gallstone disease. Compared with our historical series, children undergoing cholecystectomy are more likely to be Hispanic (P = .003) and severely obese (P < .0279). Conclusion: Obesity and Hispanic ethnicity are strongly correlated with symptomatic pediatric gallbladder disease. In comparison with our historical series, hemolytic disease is no longer the predominant risk factor for symptomatic gallstone disease in children.

Recognition and management of nonpenetrating cardiac trauma in children

OBJECTIVE To characterize the evaluation and clinical course of children with nonpenetrating injury to the heart. METHODS We reviewed the medical records for children admitted to St. Louis Childrens Hospital between the years 1987 to 1992 with traumatic cardiac injury. Patients with penetrating trauma were excluded; eight children, ages 4 to 13 years, were the study subjects. Chest x-ray studies, electrocardiograms, and serum creatine kinase values were obtained on admission. Two-dimensional echocardiography was performed when indicated by unexplained hemodynamic instability or abnormal radiographic findings. RESULTS All children with nonpenetrating cardiac trauma were involved in a motor vehicle accident. The principal cardiac diagnoses were ventricular septal defect (1), mitral regurgitation (1), pericardial effusion (2), contusion (3), and arrhythmia (1). Multisystem injury was present in each case, but cardiac injury was not suspected at the time of admission in seven of the eight patients. The hemodynamic status of four children was compromised 12 to 48 hours after admission; echocardiography was diagnostic in each instance, but the electrocardiogram and creatine kinase values were nonspecific. Two patients eventually required cardiac surgery. CONCLUSIONS Recognition of blunt cardiac trauma in children may be confounded by associated multisystem injury and the delayed onset of clinical manifestations. Echocardiography is a sensitive diagnostic tool for hemodynamically significant disease, and should be performed promptly when patients have unexplained hypotension or diminished peripheral perfusion.

Laparoscopic full-thickness intestinal biopsies in children.

Background Laparoscopy may reduce postoperative pain and hospital stay, compared with laparotomy. The use of laparoscopic surgery to obtain full-thickness intestinal biopsies in children has not been previously reported. Methods Eleven children aged 1.6 to 19 years (median, 4.5 years) underwent laparoscopic full-thickness biopsy of the stomach, small bowel, colon, or a combination thereof. Each procedure used one 12-mm and two 5-mm ports. Results Eight children with obstructive symptoms after a pull-through for Hirschsprung disease underwent multiple colon and small bowel biopsies (range, 3–6; median, 5); intestinal neuronal dysplasia was found in two. Two patients with cystic fibrosis had diffuse colonic narrowing; a diagnosis of enzyme-induced fibrosing colonopathy was made in one and nonspecific inflammation was found in the other. One child had a thickened stomach, and a gastroscopic-directed full-thickness biopsy revealed plasmacytoma. Nine of the 11 patients had a previous laparotomy, and ports were placed through preexisting scars. Median hospital stay was 2 days. No patient required more than 24 hours of narcotics. There were no leaks, and no other morbidity or mortality occurred. None of the patients required conversion to an open procedure. Biopsy results significantly affected treatment for each patient. Conclusions Laparoscopic full-thickness intestinal biopsy is safe and effective for a variety of gastrointestinal problems in children. This technique is associated with a short hospital stay, minimal pain, and a very low risk of complications and can be performed even in patients who have had a previous laparotomy.

Complications related to the Nuss procedure: Minimizing risk with operative technique

INTRODUCTION Pectus Excavatum (PEx) is the most frequent congenital chest wall deformity; surgical correction has a complication rate of 10%-50%. The purpose of this study was to evaluate outcomes in a recent cohort of pediatric patients from a single institution and investigate factors associated with complications. METHODS A review of all patients with PEx treated with a Nuss procedure from 2003 to 2011 was performed. Complications included hemo/pneumothorax, infection, bar migration, and operative injury. Chi-square, Students t-test, and logistic regression were performed. RESULTS The study included 127 Nuss patients with a the median age of 15.2 years (5.4-18.7) and a mean Haller index of 4.2 (+1.6). The total complication rate was 26% and bar migration rate was 18%. The use of a stabilizer was associated with fewer overall complications (17% vs 41%,p=0.006), decreased reoperation (16% vs 41%,p=0.003), decreased readmission (15% vs 39%,p=0.004), and decreased bar migration rate (9% vs 36%,p=0.001) compared to patients without a stabilizer. On multivariate analysis, the use of a stabilizer (OR 0.18,p=0.011,95% CI 0.049-0.68) and the use of a pericostal suture (OR 0.19,p=0.03,95% CI 0.41-0.85) were associated with decreased rates of bar migration. CONCLUSION The use of a lateral stabilizer and pericostal sutures decreased complication and reoperation rates for the Nuss procedure.

Continuous communicating esophageal and gastric duplication

Duplications of the esophagus or stomach alone are infrequent, and complete foregut duplication has only rarely been described. Most combined esophagogastric duplications present within the first year of life, and if communication with the normal alimentary tract does occur, it does so only either above or below the diaphragm. This report illustrates a case of continuous duplication of the esophagus and stomach with communication to the normal alimentary tract at both proximal and distal ends. Operative management and a review of the literature and embryology are described.

Roux-en-Y jejunostomy button in infants.

Abstract Establishing enteral feeding in high-risk infants with significant gastroesophageal reflux is a difficult challenge. Some patients are considered at very high risk for fundoplication and gastrostomy due to unstable medical conditions, dense upper-abdominal adhesions due to previous surgical procedures, or unfavorable anatomy. We describe a less invasive operation that provides jejunal feeding in a way that is safe, reliable, and convenient for the family.

An analysis of outcomes and treatment costs for children undergoing splenectomy for chronic immune thrombocytopenia purpura

PURPOSE Indications and timing for splenectomy in pediatric chronic immune thrombocytopenic purpura (cITP) are controversial because of high spontaneous remission rates and concern for overwhelming postsplenectomy infection. The objective of this study was to assess the risks, costs, and benefits of medical and surgical intervention for children with cITP. METHODS After receiving institutional review board approval, medical records for all children with cITP who underwent splenectomy from 2002 through 2009 were retrospectively reviewed (n = 22). Preoperative and postoperative data were collected. Medical and surgical costs were calculated based on pharmacy charges per dose and hospital charges, respectively. RESULTS The median age at diagnosis was 11 years (range, 3-16 years). Medical management included steroids (n = 21), intravenous gamma globulin (n = 19), anti-D antibody (n = 19), or a combination of these therapies (n = 22). Nineteen patients (86%) reported side effects from medical therapy. Median age at splenectomy was 13 years (range, 6-18 years), and time to surgery was 23 months from diagnosis (range, 6-104 months). Splenectomy increased platelet counts in all children from a median of 25,500 to 380,000 postoperatively (P < .0001). One child experienced overwhelming postsplenectomy infection after a dog bite (n = 1). At the last follow-up (15 months; range, 1-79 months), 19 patients (86%) were asymptomatic with platelet counts greater than 50,000. Of the 3 children with persistent thrombocytopenia, 2 were diagnosed with secondary cITP. Median cost of splenectomy was significantly less than the cost of medical therapy (

Anomalous Development of the Hepatobiliary System in the Inv Mouse

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