BACKGROUND/PURPOSE:Esophageal reconstruction for long gap esophageal atresia (LGEA) is still controversial. We successfully managed 7 cases of patients with LGEA by doing staged elongation of the native esophagus and subsequent end-to-end anastomosis. The technique and efficacy of this procedure are evaluated.METHODS:During the last 10 years, 7 patients with LGEA (Gross type A, 5; B, 1; C, 1) underwent multiple extrathoracic esophageal elongations (ETEEs) of the upper esophagus and subsequent esophagoesophagostomy. Medical records were reviewed in regard to the number of ETEE before definitive esophageal reconstruction, interval between each ETEE, operation time, time before initiation of sham feeding, duration of hospital stay, and complications.RESULTS:The definitive esophageal reconstruction was successfully achieved without major complications in all patients after 2 to 4 stages of ETEE. The interval between each ETEE was 72 days on average. The average operation time was 98 minutes. The elongation was 1 to 3.5 cm during each session. Oral sham feeding was recommenced 4.1 days after each ETEE, and the hospital stay was 9.6 days on average. Gastroesophageal reflux occurred in all patients, requiring antireflux surgery.CONCLUSIONS:We conclude from our experience (a) that effective esophageal lengthening with preservation of the native esophagus was achieved with multiple ETEE in LGEA and (b) that this procedure allows oral sham feeding at home until esophageal reconstruction.

Purpose: The authors determined the long-term outcome of patients who underwent surgical repair of long-segment congenital tracheal stenosis (LCTS) and compared the quality of cartilage graft and slide tracheoplasty techniques.Methods: Twenty-nine patients underwent surgical repair of LCTS at 29 days to 9 years of age. In 26 patients, more than 50% of the entire length of the trachea was involved. The early 12 patients (1981 through 1992) were treated with a costal cartilage graft for tracheal reconstruction. The remaining 17 patients (1997 through 2002) were treated with slide tracheoplasty. For both procedures, a median sternotomy was preferred because of convenience when using extracorporeal circulation and for simultaneous repair of associated cardiovascular anomalies.Results: Of the 12 patients who underwent a costal cartilage graft, 8 survived (66%). Six of these patients are free of disease, but 2 have persistent airway problems. Three patients died of residual stenosis and 1 of unrelated disease. Of the 17 patients who underwent slide tracheoplasty, 13 survived (76%), and 10 are free of disease. Three patients continue to require a stent because of tracheomalacia and granulation. Two patients died of cardiac failure, 1 of granulation, and 1 of aspiration pneumonia.Conclusions: The authors challenged LCTS with a unique surgical approach and succeeded in making an incurable disease curable. When the cartilage graft technique is compared with slide tracheoplasty, however, the latter is preferable because it preserves native tracheal tissue with less frequent complications.

Background/Purpose: Binding of Fas ligand (Fas-L) to the membrane-bound Fas receptor incites a series of intracellular events that results in programmed cell death or apoptosis. Although this apoptotic phenomenon plays a key role in down-regulating cytotoxic T cells, the authors have shown previously that pancreatic beta cells (bTC) overexpressing Fas-L paradoxically undergo accelerated rejection that is dependent on a Fas/Fas-L interaction. This study evaluates whether a neuroblastoma (NB) cell line manipulated to overexpress Fas-L undergoes similar destruction and whether tumor-specific protective immunity can be produced. Methods: The authors transfected NB cells (SK-N-MC) with either mFas-L cloned into a pcDNA3.1/Zeo plasmid vector (NB/Fas-L) or with the vector alone (NB/control). Successful transfection of Fas-L was characterized by reverse transcription polymerase chain reaction (RT-PCR) and the ability of transfectants to induce apoptosis of Fas-sensitive T cells (Jurkat). Expression of Fas and Fas-L in untransfected NB clones was characterized by immunohistochemistry and RNase protection assay (RPA). Apoptosis was measured by FACScan analysis using an Annexin V assay. A total of 3 × 106 NB/control and NB/Fas-L cells were implanted subcutaneously into the hind leg of Balb/C SCID mice. Tumor-specific protective immunity was also tested in this model by inoculating mice with NB/Fas-L before implanting NB/control cells. Results: Zeocin resistance and RT-PCR confirmed successful transfection of Fas-L into NB cells. Fas Ligand transfectants induced apoptosis in 17.6% ± 2.9% of Fas-sensitive T cells, whereas controls induced apoptosis in only 2.8% ± 1.2% (P =.01, n = 3). Although Fas appears to be constitutively expressed by NB in low amounts, introduction of Fas-L into NB cells did not induce suicide or affect tumor cell growth in vitro. In vivo, NB cells expressing Fas-L failed to grow in SCID mice (n = 3), whereas controls grew rapidly in all animals until death (n = 3). NB/control cells implanted into the opposite leg of mice that rejected initial NB/Fas-L transfectants also grew rapidly (n = 3) implying no protective immunity. Conclusions: Overexpression of Fas-L in NB clones targets such cells for rapid destruction even in immune compromised hosts, suggesting potential utility of Fas-L in combating NB. In this SCID mouse model, the observed effect is probably neutrophil mediated and does not provide tumor-specific protective immunity. J Pediatr Surg 35:375-379. Copyright © 2000 by W.B. Saunders Company.

Purpose: During the last 17 years, the authors have used the colon patch graft (CPG) procedure to treat 11 patients with extensive aganglionosis. This study evaluates the effectiveness of the CPG procedure in treating patients with this disorder.Methods: An initial ileostomy was created during infancy in all 11 patients. At 1 to 21 months (mean, 5.5 months) of age, an 11- to 40-cm (mean, 18 cm) side-to-side ileocolostomy (CPG) was created between the ileum and aganglionic ascending colon, (including aganglionic ileum in three patients). Ten patients later underwent a Swenson-type definitive operation, the CPG segment being brought down to the anus at 9 to 30 months (mean, 19 months) of age. These 10 patients have been followed up for growth, development, and bowel habit patterns for the subsequent 5 to 17 years (mean, 12 years).Results: Within 1 month after the CPG was created, intravenous nutrition could be discontinued and the patients treated at home until time for the definitive procedure. During this period, one patient died of aspiration. After the definitive operation, body weight for age returned to a normal range in 2 to 4 years. Currently, seven older patients have one to three bowel movements per day, whereas three younger patients have explosive diarrheal defecations. None are incontinent of stool. Iron deficiency anemia developed in four patients. Two are mentally retarded, but the others are doing well in school.Conclusions: (1)The colon patch graft procedure effectively reduces "ileostomy diarrhea," shortening the period of intravenous nutrition to 1 month, (2) bower habit patterns improve with the advance of time, (3) body weight for age returns to normal 2 to 4 years after the definitive procedure, and (4) during long term follow-up, patients must be evaluated for iron deficiency anemia. Copyright (C) 1988 by W.B. Saunders Company.

PURPOSE:In this study, the authors review cases of jejunoileal atresia (JIA) to evaluate their surgical treatment strategy. METHODS:Eighty-eight neonates who underwent surgical repair for JIA were divided into four groups for the type of lesion: group 1, membranous (n = 23), group II, interrupted (n = 49), group III, multiple (n = 9), and group IV, apple-peel (n = 7). Group I patients were treated with membranectomy or bowel resection and anastomosis, group II with resection of the dilated bowel and one anastomosis, group III with two to six multiple anastomoses to preserve bowel length, and group IV with minimal bowel resection and bowel anastomosis. During surgery a uniform protocol was used to minimize bowel resection and to perform an end-to-end single layer anastomosis using either Halsted horizontal mattress or conventional interrupted sutures. Mortality, morbidity, days for functional recovery, and central venous nutrition (CVN) were included in the review. RESULTS:Of 88 patients, three died of causes unrelated to operation for JIA. Nine patients underwent an additional laparotomy for leakage (n = 4) and obstruction (n = 5). Oral feeding was allowed on day 5.4+/-4.3 and full caloric intake via the enteric route on day 12.5+/-10.0. Twenty-one patients required CVN for 32.4+/-19.1 days. None required a long-term treatment for the short bowel syndrome. CONCLUSION:This study concludes that efforts to preserve bowel length are laudable to avoid the short bowel syndrome and that an end-to-end single layer anastomosis contributes to early recovery of bowel function.

PURPOSE:The purpose of this study is to examine the justification of diaphragmatic plication to treat diaphragmatic eventration. A retrospective review of 50 patients who underwent diaphragmatic plication for phrenic nerve injury (PNI) or congenital muscular deficiency (CMD) of the diaphragm was conducted. METHODS:During the last 26 years, 50 patients, aged 4 days to 7 years, were surgically treated for diaphragmatic eventration. Twenty-five patients had iatrogenic PNI and another 25 had CMD. Respiratory distress developed in all patients who had PNI and 10 required mechanical ventilatory support for 13 to 78 days (mean, 41 days) before operation. Respiratory symptoms developed in 17 of 25 patients who had CMD, and four required ventilatory support. In those who were asymptomatic, we justified surgical repair to optimize future lung growth. All patients underwent diaphragmatic plication by a thoracic approach. Reefing mattress sutures on pledgets were used for the plication. RESULTS:In patients who had PNI, ventilatory support could be discontinued within 0 to 6 days (mean, 3 days) after operation, with a dramatic improvement in their respiratory status. Two patients required reoperation because the plication was not tight enough. Seven patients died in this series, but none because of the diaphragmatic plication. CONCLUSION:This study suggests that symptomatic patients who have diaphragmatic eventration should be operated on immediately with an expected dramatic resolution of their respiratory problems.

This report describes surgical techniques used for the repair of congential diaphragmatic anomalies such as diaphragmatic hernia or eventration, using Dacron felt pledgets over the sutures to protect the tissue against tearing.

In the last 12 years, 21 patients age 1 month to 5 years (median, 7 months) underwent surgical treatment for severe airway obstruction associated with congenital heart disease. Plico-suspension of the pulmonary artery was carried out in 14 patients with documented severe left bronchial compression by a distended pulmonary artery together with repair of ventricular septal defects (11 patients), repair of large coronary artery fistula (1), division of ductus (1) and pulmonary artery banding (1), with subsequent significant relief of compression. Aortopexy was used in 2 patients with documented severe tracheal compression by a right aortic arch. Five patients had congenital tracheal stenoses (3 extensive, 2 localized). Two of 3 patients with associated complex intracardiac anomalies underwent concomitant repair of both lesions with the use of cardiopulmonary bypass and 1 had a tracheoplasty and pulmonary artery banding. Tracheal reconstruction with a cartilaginous graft together with repair of pulmonary artery sling was carried out in 2 infants. Nineteen patients are alive and well, at a mean follow-up of 87 months. In conclusion, we advocate early aggressive surgical intervention to both lesions to obtain a better result in the management of infants and small children with this difficult and often fatal combination.

For the surgical repair of long-segment tracheal stenosis, costal cartilage graft or extensive resection with end-to-end anastomosis has often been used. Both procedures have a risk of developing anastomotic leakage, which is potentially a lethal complication, or stenosis resulting from compromised blood supply to the tissue at the anastomosis. We have used omental pedicle flap (OPF) to seal the anastomotic line and to restore the vascularity of the graft and the trachea in an attempt to avoid fatal complications. During the period between 1986 and 1990, OPF technique was used in tracheobronchial reconstruction in six patients aged 4 months to 3 years; cartilage graft for extensive tracheal stenosis (4), tracheal resection and anastomosis (1), and bronchial resection and anastomosis (1). The omentum was separated from the colon to form an OPF with the right gastroepiploic vessels preserved. The OPF was brought to the upper trachea in the mediastinum through the retrosternal space. There was no immediate postoperative death due to anastomotic leak. Endotrachial tubes were removed in all patients. Four of the six are totally free of airway problems. One patient showed persistent stridor because of remaining stenosis at the cervical trachea. The remaining one patient who underwent bronchial resection developed anastomotic stenosis probably due to the compression of the floppy left main bronchus by adjacent aorta. The OPF seems to be an important surgical adjunct in order to eliminate fatal complications in tracheobronchial reconstruction.

This paper reports the case of an 8-year-old girl who successfully underwent tracheobronchial reconstruction for neoplastic obstruction at the carina. Reconstruction of the carina was performed using an end-to-end type anastomosis between the trachea and bronchi.

During the period between 1974 and 1988, 44 patients were treated for congenital intrinsic duodenal obstruction using a diamond-shaped anastomosis (35 atresias, including two multiple atresias and nine stenoses). Neither gastrostomy nor transanstomotic tube was used. Postoperatively, oral feeding was started on days 2 to 6 (3.66 ± 1.41 day). Intravenous fluid administration was discontinued on days 3 to 20 (7.54 ± 3.58 day). Fourteen patients died, none related to the operative procedure. Of 30 survivors, 21 patients have been followed from 6 months to 15 years. Body weight was within the normal range at the latest visit. Current barium study performed in 19 patients showed no blind loop, megaduodenum, or anastomotic malfunction. The diamond-shaped anastomosis provides the following advantages: (1) early recovery of anastomotic function, and (2) avoidance of later complications, such as formation of a blind loop or anastomotic stenosis.

From 1970 to 1987, 23 neonates with gastroschisis were treated at Kobe Children's Hospital. One patient died and 22 survived (96%). Seven patients were hypothermic upon arrival at the hospital, with body temperatures of 31.8 to 35.4 degrees C. Six patients were successfully managed by warm saline bathing (40 to 42 degrees C for one minute) to improve hypothermia. One patient who did not receive this rewarming procedure (body temperature, 31.8 degrees C) died of intractable metabolic disorders related to hypothermia. Ten patients were treated by primary fascial closure of the abdominal wall defect, eight by the skin flap method with secondary closure of the deliberately created abdominal hernia, and five by the silastic sac technique. Two patients required creation of intestinal stomas for ileal atresia. After surgery, 19 patients required ventilatory care with total paralysis (average 6.0 days). Nine patients required total parenteral nutrition (TPN), while 13 tolerated enteral feeding on days 3 to 11 (average, 6.0 days). In the latter 13 patients, intravenous fluid therapy was discontinued on day 11. Our experience suggests that (1) rewarming in a saline bath is effective treatment for hypothermia, (2) ventilatory assistance with total paralysis is mandatory, and (3) TPN can be avoided in 60% of the patients.

Since we introduced the surgical technique for the repair of congenital stenosis involving the entire trachea with a successfully treated patient in 1982, four more patients have undergone a tracheoplasty employing the same technique for congenital stenosis involving a long segment of trachea. This article describes the refinement of surgical technique, the management of patients during and after operation, and the long-term outcome.

During the period from 1979 to 1986, seven patients have been treated for extensive aganglionosis involving the colon and distal ileum (5 to 40 cm) employing the colonic patch graft (CPG) procedure. This consists of (1) ileostomy; (2) creation of a longitudinal side-to-side ileocolostomy between normal ileum and aganglionic ascending colon, forming a CPG; and (3) the definitive pull-through procedure. In all patients, the initial course after ileostomy was complicated by severe diarrhea, which was significantly improved by creation of the CPG. At the definitive operation several months later, the mesocolon attached to the CPG was severed to mobilize the ileocolostomy segment to the distal pelvis. Four patients in this series have been followed for 5 to 8 years. No patient has developed enterocolitis requiring hospitalization. Body weight became normal for age in all patients within 4 years of the definitive operation.

Despite the recent advent of total parenteral nutrition (TPN), the long-term survival of the patients with short gut syndrome is not satisfactory with complications of catheter-related sepsis and cholestasis. The causes of short gut syndrome in pediatric surgery are multiple intestinal atresias, necrotizing enterocolitis (NEC), and midgut volvulus. For the multiple atresias, multiple anastomosis without resection has been successfully undertaken to avoid developing short gut syndrome for the last 17 years. For the massive involvement of the intestines due to NEC or volvulus, high jejunostomy and peritoneal drainage with TPN support have been tried for the last two patients and successfully weaned from the TPN within two months after closure of jejunostomy. However, in case 2 malabsorption has been persistent presumably due to a severe degree of mucosal damage occurring in the remaining intestines. Whether this eventually gets back to the normal or not is unknown. For the patients with short gut syndrome, small bowel reversal procedure was successfully done and now doing well 10 years after surgery. This paper reports details of these 3 cases.

A staged approach consisting of (1) spiral myotomy (2 1/2 revolutions) to elongate the proximal esophagus with its distal end forming a cutaneous esophagostomy in the anterior chest, and (2) delayed reconstruction of the esophagus with an end-to-end anastomosis, has been successfully employed in a 1-year-old infant who had undergone an unsuccessful repair of tracheoesophageal atresia during the neonatal period.

Four unrelated infants with multiple segments of nonduodenal intestinal atresia and amyoplasia congenita are described. The extreme rarity of each of these conditions as a single entity indicates that their concurrent incidence in four separate unrelated patients is of significance with respect to pathogenesis. The nature of the relationship between the two conditions is unknown.