Primary repair of pulmonary artery sling with double outlet right ventricle and distal tracheal stenosis.

The fate of patients with pulmonary artery sling depends on associated tracheal lesions. Distal tracheal stenosis involving the carina frequently results in lethal obstruction due to secretion or inflammatory edema. Further-more, associated complex cardiac anomalies with excessive pulmonary flow make the situation more complicated. We present a case of successful simultaneous one-stage repair of pulmonary artery sling, double outlet right ventricle and distal tracheal obstruction. Pulmonary artery sling was relieved by relocation of the left pulmonary artery (left pulmonary artery to main pulmonary artery) and a tracheoplasty (resection and end-to-end anastomosis). We advocate early aggressive simultaneous repair of pulmonary artery sling with tracheal stenosis and concomitant repair of intracardiac anomalies whenever possible.

A new volumetric evaluation of partial splenic embolization for hypersplenism in biliary atresia.

BACKGROUND/PURPOSE: Partial splenic embolization (PSE) has become an important therapeutic modality in the management of hypersplenism in biliary atresia (BA). Fifty percent to 80% of spleen is usually devascularized by embolization. The functional outcome, however, has not been correlated with embolized volume of the spleen. The authors propose a new, reliable method of predicting functional outcome using nonembolized volume of the spleen (NEVS) as an index. METHODS: Between January 1993 and July 2000, 11 children with BA (2 boys and 9 girls, aged 5 to 10 years) underwent 12 PSE procedures. The follow-up period ranged from 6 to 77 months. The NEVS was calculated by enhanced computed tomography (CT) images, and an index was calculated by dividing NEVS with the predicted splenic volume for body weight (standardized NEVS ratio) 2 weeks after PSE. RESULTS: Splenic volumes before PSE ranged from 312 to 1,201 cm(3) (mean, 875.8 cm(3)). NEVS ranged from 140 to 485 cm(3) (mean, 340 cm(3)). Standardized NEVS ratio ranged from 2.21 to 7.22 (mean, 4.25). The platelet counts with standardized NEVS ratio below 5.0 (group I) and above 5.0 (group II) were 15.1 x 10(4)/mm(3) and 7.2 x 10(4)/mm(3) at 6-month follow-up, respectively. CONCLUSIONS: (1) Nonembolized volumetric evaluation is useful in predicting the functional outcome of PSE. (2) Reembolization is indicated for the patients with standardized NEVS above 5.0.

Multistaged extrathoracic esophageal elongation procedure for long gap esophageal atresia: Experience with 12 patients.

PURPOSE: This study evaluates the multistaged extrathoracic esophageal elongation procedure performed on 12 babies with long gap esophageal atresia over 15 years. METHODS: Eight babies had pure esophageal atresia, 2 had proximal tracheoesophageal fistula (TEF), and 2 had distal TEF. The gaps ranged between 2 and 7 vertebral bodies. Proximal esophagostomy, TEF ligation, and gastrostomy were performed initially. The proximal esophagus is elongated 2 to 3 cm each time by translocating the esophagostomy distally along the anterior chest wall at 2- to 3-month intervals. Sham-fed milk is collected in an ostomy bag and refed via the gastrostomy. The definitive esophageal reconstruction is performed at 5 to 24 months of age. RESULTS: Only one elongation was required in 4 babies, 2 were needed in 5, 3 in 2, and 5 in 1 patient. All patients tolerated sham feeding well. After esophageal restoration, 3 patients had minor leakage. All (12 of 12) patients had anastomotic stenosis requiring multiple dilatations, of which, 3 needed resection of stricture. Eleven patients had gastroesophageal reflux that required fundoplication. Follow-up was possible in 11 patients for 4 months to 14 years after esophageal restoration. Seven early patients are eating normally. CONCLUSION: Multistaged extrathoracic esophageal elongation is effective in stretching the proximal esophagus to bridge 2 to 7 vertebral bodies.

Posterior sagittal anorectoplasty for failed imperforate anus surgery: lessons learned from secondary repairs.

PURPOSE: The aim of this study was to evaluate secondary operations using a posterior sagittal approach in patients with fecal incontinence and impaction after primary repair of anorectal malformations. METHODS: Twenty patients (14 boys, 6 girls) who had previous failed surgery for imperforate anus underwent secondary operations. The indications for surgery included fecal incontinence (n = 16) and fecal impaction (n = 4). Patients ranged in age from 2 to 30 years (mean, 11 years), with 4 over the age of 20 years. The primary procedures included abdominosacroperineal (n = 7), sacroperineal (n = 10), and perineal (n = 3) pull-throughs. At surgery, none of the patients underwent a diverting colostomy. The rectum was mobilized from the surrounding structures through a posterior sagittal approach. The surgical findings included anteriorly displaced anus (n = 17), laterally displaced anus (n = 3), mesenteric fat surrounding the rectum (n = 4), mega-rectosigmoid (n = 2), and others (n = 3). The rectum underwent reconstruction, which involved relocation of the rectum and anus to surround them with the muscle complex. RESULTS: Patients underwent follow-up for periods ranging from 8 months to 6 years after surgery (mean, 3 years). To evaluate the functional results, fecal continence scores (Templeton and Ditesheim) were calculated for incontinent patients. Of the 16 incontinent patients, 12 achieved continence and 4 some improvement. Of the 4 patients with fecal impaction, 2 achieved daily voluntary bowel movement, whereas the other 2 have mild constipation and need occasional enemas. CONCLUSIONS: Our study suggests that (1) a secondary operation through a posterior sagittal approach can restore fecal continence and is efficacious even in adolescents and adults and (2) a posterior sagittal procedure can be safely performed without a diverting colostomy.

The evaluation of meconium disease by distribution of cathepsin D in intestinal ganglion cells.

Meconium disease (MD) results in intestinal obstruction in the neonate where tenacious meconium is found in the distal ileum and proximal colon. The obstructive symptoms improve at several days of age after some of the meconium is passed. We observed premature infants with MD who underwent ileostomy for intestinal obstruction due to tenacious meconium. Afterward, meconium was passed well and the clinical symptoms improved. After closing the ileostomy, growth and defecation became normal. The MD in our cases was documented by histologic changes in the maturation of ganglion cells observed at the time of ileostomy creation and closure. For an objective evaluation of the maturation of intestinal ganglion cells (IGC), we attempted to distinguish immature from mature cells by the expression of cathepsin D. We examined the distribution of cathepsin D in IGC in patients with MD to test the hypothesis that ganglion-cell immaturity might be related to MD. In ganglion cells at the time of ileostomy, cathepsin D was detected in the perinuclear cytoplasm (immature staining pattern), while at the time of ileostomy closure it was detected in intense granules throughout the cytoplasm (mature staining pattern). We propose that it would be possible to evaluate the maturation of IGC by the intracellular distribution of cathepsin D in MD and suggest that immaturity of IGC might be the cause of MD.

Changing profile of abdominal wall defects in Japan: results of a national survey.

BACKGROUND/PURPOSE: The incidence of gastroschisis has increased over the past 3 decades in a number of countries. To elucidate the Japanese status of anterior abdominal wall defects, the Japanese Society of Pediatric Surgeons conducted a national survey in Japan. METHODS: Information was obtained by sending out a questionnaire to 192 University Hospitals, Children's hospitals, and general hospitals that each had more than 200 beds. The characteristics of the patients including the birth date, birth weight, gestations, rate of associated anomalies, rate of antenatal diagnosis and prognosis, maternal age, gravidity, history of smoking, and drug use were analyzed. RESULTS: The authors obtained answers from 149 institutions, including 1,785 cases of omphalocele and 970 cases of gastroschisis, which were treated between 1975 to 1997. There was a significant increase in the incidence of gastroschisis, from 0.131 in 1975 to 1980, 0.269 in 1981 to 1985, 0.337 in 1986 to 1990, 0.461 in 1991 to 1995 to 0.467 per 10,000 births in 1996 to 1997. The incidence of omphalocele was 0.322 in 1975 to 1980, 0.567 in 1981 to 1985, 0.657 in 1986 to 1990, 0.741 in 1991 to 1995 to 0.626 per 10,000 births in 1996 to 1997, respectively. In the omphalocele group, 43.1% of the mothers were between 25 to 29 years of age, whereas in the gastroschisis group 42.6% of the mothers were 20 to 24 years of age. In the gastroschisis group, the number of primipara mothers was larger than that of multipara mothers. In the omphalocele group, approximately 10% of the mothers smoked during each period, whereas in the gastroschisis group, the percentage of smoking mothers increased chronologically from 12.9% in 1981 to 1985, 18.7% in 1986 to 1990, 23.5% in 1991 to 1995 and 29.3% in 1996 to 1997. A history of drug use by the mother was approximately 10% for both the omphalocele and gastroschisis groups. In the omphalocele group, 55.9% had associated anomalies against 21.8% in the gastroschisis group. Approximately 10% in the omphalocele group and less than 3% in the gastroschisis group showed chromosomal abnormalities. From 1986, a significant number of cases detected antenatally has been observed. CONCLUSIONS: There have been substantial changes in the incidence of anterior abdominal wall defects, particularly regarding gastroschisis in Japan. The reasons for such changes are most likely multifactorial, further epidemiological monitoring is thus called for.

Ingested ring-pull causing bronchoesophageal fistula and transection of the left main bronchus: successful salvage of the left lung and esophagus five years after injury.

A 6-year-old girl with a history of ingestion of a ring-pull of a can and a transient episode of stridor had been asymptomatic 3 years before admission when left lung atelectasis with severe respiratory distress developed. Fluoroscopy and 3-dimensional computed tomography scan showed bronchoesophageal fistula and the ring-pull around the left main bronchus. At operation, the ring-pull, which transected the left main bronchus, was extracted. The left main bronchus was reconstructed by end-to-end anastomosis in spite of insufficient inflation of the collapsed left lung. The esophageal defect was repaired. The patient's respiratory distress gradually disappeared, and the x-ray films 3 months after operation showed complete expansion of the left lung. This case shows the risk of the long-term retained esophageal foreign body and the possibility of pulmonary salvage after long-term total atelectasis of the lung.

Surgical repair of rectovestibular fistula with normal anus.

PURPOSE: The aim of this study was to evaluate the authors' surgical approach and technique in patients with congenital rectovestibular fistula with a normal anus (CRF). METHODS: During the period between 1981 and 1995, 19 girls from 2 months to 13 years of age were treated surgically for CRF by a primary perineal approach. After appropriate bowel preparation, the patient was placed in a lithotomy position. A probing catheter was placed in the fistula. A perineal transverse skin incision was made on the midpoint between the posterior commissure and the anus, and the underlying tissue was dissected. The fistula was divided, and the both ends were closed by interrupted sutures. The external sphincter muscle was mobilized to interpose between the vestibular and rectal stumps of the fistula. Postoperative feeding was begun on day 6. RESULTS: A protecting colostomy was created in the early 4 patients. Fifteen patients underwent a primary fistula division without colostomy. In those without colostomy, 1 patient had a reopening of the fistula 6 days after the primary repair. In this patient, colostomy was created, and the fistula was divided 6 months later by the same approach. After a follow-up of 3 to 17 years, all patients have normal bowel habit. CONCLUSION: A primary perineal approach is appropriate for the treatment of CRF.

Fusion of intussusceptum and intussuscipiens in intrauterine intussusception: a rare type of intestinal atresia.

Intrauterine intussusception is well known as one of the rare causes of intestinal atresia. Although the polypoid intussusceptum is usually observed at the obstructed end on the distal side, a few cases with the polypoid lesion located apart from the blind end have been reported. To elucidate the etiology of separated polypoid intussusceptum, we reviewed 42 surgical cases of jejunal or ileal atresia over the last 12 years at Kobe Children's Hospital, Kobe, Japan. Of the 42 cases, 11 were intrauterine intussusception. Two of the 11 cases were associated with polypoid intussusceptums separated from the obstructed ends; the intestinal portion between the polypoid intussusceptum and the obstructed end showed a partial two-fold proper muscle wall and a mesenteric structure invaginated between the two walls. Another case showed linear ulcers facing each other on both the intussusceptum and intussuscipiens. Linear ulceration and subsequent fusion of the intussusceptum and intussuscipiens are suggested to be the pathogenesis of the first two cases.

The mechanism of focal intestinal perforations in neonates with low birth weight.

Among 36 neonates with intestinal perforations (IP) between 1975 and 1996, 5 had necrotizing enterocolitis (NEC IP) and 10 had focal IPs (FIP). A histologic review of the bowel near the perforations was made to see if there was any difference between cases of NEC IP and FIP. In 1 case of NEC IP, a defect in the musculature was found in addition to disappearance of the mucosal villi and dilated vessels or hemorrhage in the submucosa. Thinning or absence of the intestinal musculature and short villi in the mucosa was observed in 3 cases of FIP, but the acute ischemic changes in FIP were much less than in NEC IP. Hypothesizing that the defective musculature in FIP may be acquired by a vascular accident either before or after birth, we examined the histology of the latest consecutive infants diagnosed as having meconium peritonitis (MP) due to in-utero volvulus and perforation. In the tissue near the perforation, there was an identical focus of thinning and interruption of the musculature while the acute ischemic changes were minimal. We speculate that thinning or absence of the intestinal musculature in FIP may be a result of a transient ischemic event occurring in-utero and that FIP may develop in the damaged intestine after birth when it is fully dilated.

Jejunoileal atresia: a 27-year experience.

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.

Slide tracheoplasty: a case report of successful concomitant reconstruction of extensive congenital tracheal stenosis and pulmonary artery sling.

An 8-month-old infant presented with an extensive congenital tracheal stenosis with an aberrant left pulmonary artery. The patient was treated successfully by relocation of the left pulmonary artery and tracheal reconstruction with slide tracheoplasty. This patient is the first survivor cited in the literature after concomitant repair using slide tracheoplasty.

Reappraisal of lateral cervical cysts in neonates: pyriform sinus cysts as an anatomy-based nomenclature.

BACKGROUND/PURPOSE: Cervical cysts in neonates are recognized as rare branchial remnants. This term includes many different derivatives of the branchial cleft and sinus. METHODS: A retrospective review was performed in seven neonates with lateral cervical cysts seen at a single institution between 1975 and 1996. Two patients underwent simple excision of the cyst before 1987. Since 1987, five underwent complete excision of the sinus with or without esophagoscopic assistance to identify the orifice of the pyriform sinus. RESULTS: All of the cysts were located on the left side of the neck, anterior to the sternocleidomastoid muscle. Five had large cysts containing air. In four patients, a large orifice of the pyriform sinus was identified by the esophagoscopy. In six of seven patients, the histological examination showed thyroid or thymic tissue in the wall of the cysts, which is compatible with a clinical entity of "pyriform sinus fistula" commonly seen in older children. CONCLUSION: We propose that this anomaly be called a pyriform sinus cyst, which is an anatomic term based on their draining orifice.

[Pulmonary artery sling with tracheal stenosis--primary repair in infancy].

Between 1984 and 1996 five infants underwent surgical repair of pulmonary artery sling associated with severe congenital tracheal stenosis. All infants had symptoms of severe respiratory distress and three of them required ventilator support preoperatively ages ranged from 2 to 11 months (mean age 6 months). Complete tracheal rings were present in all patients as an associated lesion and right upper lobe tracheal bronchus in 3 patients. The length of tracheal stenosis ranged from 18 to 45 mm (median 40 mm). Three had associated intracardiac anomalies (Scimitar syndrome (1), VSD (1), double-outlet right ventricle with VSD (1), double-outlet right ventricle with pulmonary hypertension (1)). Surgical intervention was carried out through a right thoracotomy (1) or median sternotomy (4). Cardiopulmonary bypass (CPB) was used in 3 patients and extracorporeal membrane oxgenator (ECMO). In 1. All infants had reimplantation of the left pulmonary artery into the main pulmonary artery left anterior to the trachea. Four patients underwent simultaneous tracheoplasty using costal cartilage grafts and one had complete resection of obstructed trachea between the right upper lobe tracheal bronchus and carina. The length of resected trachea was about 30% of the entire length of the trachea. Three infants underwent simultaneous intracardiac repair. There was no hospital death. All were weaned from ventilatory support and extubated on 1 to 16 months (mean 4, 5 months) postoperatively. AS an additional procedure, aortopexy, removal of granulation tissue or balloon dilatation of the trachea were carried out in one patient each following tracheoplasty using cartilage grafts. There was one late death at 1 year postoperatively. Three of 4 survivors are doing well with no stridor. We adonostridor. We adovocate 1) early aggressive primary repair of pulmonary artery sling with tracheal stenosis, 2) concomitant repair of tracheal lesion and intracardiac anomalies whenever possible, 3) application of CPB or ECMO to avoid cumbersome intubation technique, and 4) utmost effort to perform tracheal resection and end-to-end anastomosis.

The colon patch graft procedure for extensive aganglionosis: long-term follow-up.

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) bowel 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.

Postoperative intussusception after surgery for malrotation and appendicectomy in a newborn.

Postoperative intussusception in the newborn is an infrequent condition. A 17-day-old female with duodenal stenosis and malrotation underwent excision of the membrane in the duodenum and incidental appendicectomy. Postoperatively, a ceco-colic type of intussusception occurred, necessitating a right hemicolectomy. We speculate that the causative factors are twofolds: the embedded appendiceal stump, a polyp-like protrusion that became a lead point, and the non-fixation of the ileocecal mesentery, which facilitated a ceco-colic type of invagination.

Congenital true diverticula of the esophagus: a case report.

Pseudodiverticulosis secondary to gastroesophageal reflux is a common disease in adults, but true esophageal diverticula are rare in infants and children. A 5-year-old boy was well until the age of 1 1/2 years when he started vomiting. An upper gastrointestinal series showed two diverticula bulging from the posterior right side of the middle esophagus associated with slight hiatal hernia and short esophagus. Diverticulectomy, the Collis-Nissen antireflux procedure, and pyloroplasty were performed simultaneously through a left thoracoabdominal incision. Histological examination of the diverticula showed that the wall of each diverticulum consisted of a full-thickness of esophageal wall. Because there was no tracheal remnant in the diverticula, this lesion is more likely to be a true diverticulum than a duplication.

Diaphragmatic eventration in infants and children: is conservative treatment justified?

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.

Biliary atresia: current management and outcome.

Between 1986 and 1994, 42 patients with BA were treated at the Kobe Children's Hospital. These patients underwent a wider excision of the hilar fibrous remnant with Roux-Y reconstruction (with or without intussuscepted valve) without stoma. Corticosteroids were used postoperatively when the stool was acholic or unsteadily cholic. The daily dose was reduced from 20 mg/day by half down. The patients were divided into two groups; in Group I (n = 17, before October 1990), a single course of corticosteroid therapy was employed. In Group II (n = 25, from November 1990 on), this regimen was repeated whenever the stool appeared less cholic. The bile flow improved significantly (excellent in 29% and 60%, and poor in 71% and 32% in Groups I and II, respectively.) Corticosteroids were used in 15 Group I patients with good response in 10 and in 21 Group II patients, 15 of whom had multiple courses. Sixteen of the 21 Group II patients had a good response. The incidence of the cholangitis was not significantly different between the 19 patients with valve and the 23 patients without valve. A 5 year survival significantly improved from 70% in Group I to 96% in Group II. In both groups, the survival rate significantly increased, when compared with the survival rate figured out with an assumption of OLT survivors as dead. On the same assumption, the survival rate of Group II is significantly more than that of the Group II. These suggest a positive contribution of liver transplantation and an aggressive corticosteroid therapy on better survival of Group II.