Jacob C. Langer

Prevention of respiratory syncytial virus infections: Indications for the use of palivizumab and update on the use of RSV-IGIV

The Food and Drug Administration recently approved the use of palivizumab (palē-vizhū-mäb), an intramuscularly administered monoclonal antibody preparation. Recommendations for its use are based on a large, randomized study demonstrating a 55% reduction in the risk of hospitalization attributable to respiratory syncytial virus (RSV) infections in high-risk pediatric patients. Infants and children with chronic lung disease (CLD), formerly designated bronchopulmonary dysplasia, as well as prematurely born infants without CLD experienced a reduced number of hospitalizations while receiving palivizumab compared with a placebo. Both palivizumab and respiratory syncytial virus immune globulin intravenous (RSV-IGIV) are available for protecting high-risk children against serious complications from RSV infections. Palivizumab is preferred for most high-risk children because of ease of administration (intramuscular), lack of interference with measles–mumps–rubella vaccine and varicella vaccine, and lack of complications associated with intravenous administration of human immune globulin products. RSV-IGIV, however, provides additional protection against other respiratory viral illnesses and may be preferred for selected high-risk children including those receiving replacement intravenous immune globulin because of underlying immune deficiency or human immuno-deficiency virus infection. For premature infants about to be discharged from hospitals during the RSV season, physicians could consider administering RSV-IGIV for the first month of prophylaxis. Most of the guidelines from the American Academy of Pediatrics for the selection of infants and children to receive RSV-prophylaxis remain unchanged. Palivizumab has been shown to provide benefit for infants who were 32 to 35 weeks of gestation at birth. RSV-IGIV is contraindicated and palivizumab is not recommended for children with cyanotic congenital heart disease. The number of patients with adverse events judged to be related to palivizumab was similar to that of the placebo group (11% vs 10%, respectively); discontinuation of injections for adverse events related to palivizumab was rare.

Comparative analysis of laparoscopic versus open splenectomy

BACKGROUND Laparoscopic splenectomy (LS) has been used to treat a variety of splenic disorders. However, there have been few direct comparisons of this approach with open splenectomy (OS). METHODS Results and outcomes were compared retrospectively in 46 consecutive patients treated by laparoscopic (n = 26) or open splenectomy (n = 20) from January 1990 through March 1996. The two groups were similar in age, sex, and American Society of Anesthesiology classification. Splenectomy was performed for a variety of indications, and the majority of patients in both groups had normal or near-normal size spleens. All data are expressed as mean +/- standard deviation. RESULTS Laparoscopic splenectomy was successfully completed in all 26 attempted cases. Operative times were significantly longer for LS (202 +/- 55 minutes) than for OS (134 +/- 43 minutes) (P < 0.001); however, operative times in the last 13 LS cases (176 +/- 48 minutes) averaged 51 minutes less than in the first 13 cases (227 +/- 51 minutes). Estimated operative blood loss was less for LS (222 +/- 280 mL) than for OS (376 +/- 500 mL) (P = not significant). A mean of 2.0 units of red blood cells was transfused in 4 (15%) of 26 patients during LS vs 1.0 unit transfused in 2 (10%) of 20 patients who had OS (P = NS). Patients who underwent LS required significantly less parenteral pain medications, had a more rapid return to regular diet, and were discharged sooner than patients who had OS. Complication rates were similar in the two groups. CONCLUSIONS These results suggest that LS is technically safe and has several advantages over OS. Laparoscopic splenectomy should become the procedure of choice for the removal of normal and near-normal size spleens.

Diagnosis and management of hydatid disease of the liver: a 15-year North american experience

Since 1967, 40 patients with hydatid disease of the liver have been treated at our hospital. Diagnosis was made using clinical criteria, serology, skin tests, and imaging techniques. Thirty-five patients were operated upon. In 18 patients the cyst was uncomplicated (Group I), and in 17 the cyst was infected or communicated with the biliary tract (Group II). Three forms of surgical treatment were used: A) cyst evacuation, scolicidal irrigation, and primary cyst closure, B) evacuation, irrigation, and external drainage, and C) complete or partial cyst resection. Mebendazole was used in six patients, four of whom were also treated surgically. In Group I, one of 11 patients (8%) treated by primary closure had complications, versus four of five patients (80%) treated with external drainage (p < 0.001). Mean postoperative hospital stay for these two groups was 11.8 versus 20.8 days, respectively (p < 0.001). Complication rates in Group II were higher, and were evenly distributed among treatments. Patients have been followed yearly, with a median follow-up of 5 years. Active hydatid disease has been found in three patients, who all had known residual disease at initial operation. The best treatment for an uncomplicated hydatid liver cyst is evacuation, scolicidal irrigation, and primary closure. External drainage is used for infected cysts or those communicating with the biliary tract, and excision for extrahepatic and peripheral, easily resectable cysts. Mebendazole is used for intraperitoneal spillage of cyst contents and in patients with inoperable disease.

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.

Congenital cystic adenomatoid malformation in the fetus : Natural history and predictors of outcome

Cystic adenomatoid malformation (CCAM) is a rare lesion that often is diagnosed by prenatal ultrasonography. Outcome varies from hydrops and fetal death to resolution before birth. The authors reviewed their 7-year experience with 17 fetuses diagnosed with CCAM by prenatal ultrasonography to determine the natural history of the lesion and to identify factors that might predict outcome. Of the 17 fetuses, five died during intrauterine life (3 terminations, 2 fetal deaths); four of them had hydrops. Twelve fetuses were carried to term. Only one had prenatal intervention: a thoracoamniotic shunt at 24 weeks. All 12 infants survived and underwent resection. Only four required neonatal support (1 extracorporeal membrane oxygenation, 2 ventilator, 1 oxygen). The initial CCAM:chest ratio, degree of mediastinal shift at time of diagnosis, location of CCAM, and age at time of diagnosis did not correlate with outcome. Sonographically predicted pathological type did not correlate with pathological diagnosis after surgery, or with outcome. The only accurate predictors of outcome were presence of hydrops (all died) and decrease in size of CCAM during gestation (all survived). The outcome for fetuses with CCAM may be better than previously recognized. Many of the lesions decrease in size, despite significant mediastinal shift and lung compression at the time of diagnosis. Fetal intervention should be considered only for fetuses with hydrops. Others should be monitored with serial ultrasonography. Parental counseling, especially regarding pregnancy termination, should reflect the positive outcome noted in most cases.

One-stage transanal Soave pullthrough for Hirschsprung disease: a multicenter experience with 141 children.

Background: The surgical management of Hirschsprungs disease (HD) has evolved from the original 3-stage approach to the recent introduction of minimal-access single-stage techniques. We reviewed the early results of the transanal Soave pullthrough from 6 of the original centers to use it. Methods: The clinical course of all children with HD undergoing a 1-stage transanal Soave pullthrough between 1995 and 2002 were reviewed. Children with a preliminary stoma or total colonic disease were excluded. Results: There were 141 patients. Mean time between diagnosis and surgery was 32 days, and mean age at surgery was 146 days. Sixty-six (47%) underwent surgery in the first month of life. Forty-seven (33%) had the pathologic transition zone documented laparoscopically or through a small umbilical incision before beginning the anal dissection. Mean blood loss was 16 mL, and no patients required transfusion. Mean time to full feeding was 36 hours, mean postoperative hospital stay was 3.4 days, and 87 patients (62%) required only acetaminophen for pain. Early postoperative complications included perianal excoriation (11%), enterocolitis (6%), and stricture (4%). One patient died of congenital cardiac disease. Mean follow-up was 20 months; 81% had normal bowel function for age, 18% had minor problems, and 1% had major problems. Two patients required a second operation (twisted pullthrough, and residual aganglionosis). One patient developed postoperative adhesive bowel obstruction. Conclusion: To date, this report represents the largest series of patients undergoing the 1-stage transanal Soave pullthrough. This approach is safe, permits early feeding, causes minimal pain, facilitates early discharge, and presents a low rate of complications.

Hypertrophic pyloric stenosis: ultrastructural abnormalities of enteric nerves and the interstitial cells of Cajal.

Dysfunction of pyloric inhibition has been implicated in the pathophysiology of hypertrophic pyloric stenosis. Normal inhibition likely is mediated by peptidergic enteric nerves and also may involve interstitial cells of Cajal (ICC). The authors used electron microscopy to qualitatively assess these structures in infants with pyloric stenosis and in normal controls. Pyloric muscle strips from five infants with hypertrophic pyloric stenosis, from three normal pediatric organ donors, and from three adults were examined. The following observations were made. (1) Muscle cells were primarily in a proliferative phase in pyloric stenosis and exhibited very few gap junctions between smooth muscle cells or ICC compared with the control specimens. (2) The circular muscle layer in pyloric stenosis was characterized by near absence of large granular vesicle-containing nerve fibers compared with the control specimens. (3) There were fewer nerve cell bodies in the myenteric plexus in pyloric stenosis, and the total number of ganglia was lower than that in control samples. (4) Interstitial cells of Cajal were almost completely absent in patients with hypertrophic pyloric stenosis, but there was a group of cells resembling ICC that was termed ICC-like cells. These cells may represent a failure or delay in the maturation process of the ICC. These findings show that there are significant structural abnormalities of the inhibitory nervous system in hypertrophic pyloric stenosis. The ontogenic origins and functional significance of these results require further investigation.

One-Stage Versus Two-Stage Soave Pull-Through for Hirschsprung's Disease in the First Year of Life

Several investigators have reported good results after a one-stage Soave procedure without a stoma for infants with Hirschsprungs disease. The authors reviewed their concurrent experience with the one- and two-stage approaches, comparing the two groups with respect to rate of complications and clinical outcome. Over a 3-year period, 36 infants with colonic Hirschsprungs disease presenting in the first year of life were treated with a Soave pull-through. Thirteen had a one-stage pull-through, and 23 had a two-stage procedure using an initial stoma. There was no difference with respect to median age at time of diagnosis, median follow-up period, length of aganglionosis, or male:female ratio between the groups. The incidences of major complications such as small bowel obstruction, segmental or acquired aganglionosis, anastomotic leak, and malabsorption were equal between the two groups. However, 13% of the two-stage patients required revision of the stoma. All major complications in the one-stage group were in those who weighed less than 4 kg at the time of surgery. Minor complications such as wound infection, perianal excoriation, and need for repeated dilatation were similar between the groups, but minor stoma-related complications (prolapse or retraction) occurred in 26% of the two-stage infants. When complications were stratified using a more sophisticated scale of severity, no significant difference was found between the groups. The overall complication rate was 1.5 events per patient in the one-stage group and 2.0 events per patient in the two-stage group. This small difference was related to the presence of a stoma in the two-stage group. Overall, 10 of 12 survivors in the one-stage group and 22 of 23 in the two-stage group were doing well, with normal bowel function noted on long-term follow-up (mean period, of 14 and 19 months, respectively). Both one- and two-stage approaches were associated with a significant complication rate, although long-term outcome was excellent in both groups. The higher complication rate in the two-stage group was attributable to the presence of a stoma. For small infants, it may be beneficial to delay the one-stage pull-through until weight exceeds 4 kg.

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.

Optimal management of patent ductus arteriosus in the neonate weighing less than 800 g

Between January 1988 and December 1990, 132 neonates weighing < 800 g were admitted to our neonatal intensive care unit. Of the 76 who survived initial resuscitation, 42 had developed a hemodynamically significant patent ductus arteriosus (PDA) (mean +/- SD): gestational age 25.3 +/- 1.9 weeks, birth weight 650 +/- 93 g. Two infants were referred for primary surgical ligation because of contraindications to indomethacin. Forty infants were initially treated with indomethacin. Seventeen of 40 (43%) were subsequently referred for surgical ligation because of indomethacin failure. Infants requiring surgical duct closure were a lower gestational age (24.6 +/- 1.3 v 25.7 +/- 2.0 weeks, P = 0.49) and had a greater left atrial-aortic (LA/Ao) ratio on echocardiography (1.71 +/- 0.28 v 1.46 +/- 0.26, P = .04) compared with those treated successfully with indomethacin. There were 6 deaths (15%), all of which occurred in infants receiving indomethacin (5 indomethacin alone, 1 indomethacin+ligation). Indomethacin was directly associated with intestinal perforation in 3 patients, and acute renal failure in 1; all 4 died. Surgery was associated with minimal morbidity (intraoperative transfusion in 1, postoperative pneumothorax requiring chest tube in 1). These data suggest that in the extremely premature neonate with a hemodynamically significant PDA: (1) indomethacin therapy is associated with a high failure rate and significant complications; (2) PDA associated with a large LA/Ao ratio is unlikely to close with indomethacin therapy; and (3) surgical duct closure is associated with minimal morbidity. We conclude that primary surgical ligation may provide the optimal management for PDA in carefully selected patients.