Ken Kimura

Tracheoplasty for congenital stenosis of the entire trachea

Congenital stenosis involving the entire length of the trachea has generally been regarded as a fatal disease. Tracheoplasty using costal cartilage grafts to enlarge the lumen was successfully employed in such a case, and the technique is described. A 12-mo-old female was referred with recurrent severe respiratory distress since birth. By tracheoscopy and bronchography, the entire trachea was seen to be stenotic. The left bronchus was of normal caliber by bronchogram and the left lung was over inflated, while the right lung was aplastic. Through a midsternal thoracotomy, the left bronchus was incised and cannulated for ventilation. Longitudinal incision of the entire length of the anterior wall of the trachea permitted the advance of a nasotracheal tube along the inner surface of the divided trachea to the carina. Two pieces of costal cartilage were used to fill the defect in the anterior wall of the trachea. The grafts were attached to the tracheal edges by interrupted 5-0 Dexon sutures. The endotracheal tube was successfully removed two months later. The subsequent course of the patient has been satisfactory.

A new surgical approach to extensive aganglionosis.

A new surgical technique is described that has been employed in three infants with extensive aganglionosis (1979–1980). Several weeks after the initial ileostomy, an extensive longitudinal ileocolostomy was performed electively, with the distal end of this segment replacing the original ileostomy. The ascending and transverse colon is attached to the ileum by a side-to-side anastomosis (10–25 cm long) and functions actively almost at once in absorbing water from intestinal contents. This results in passage of formed stools, weight gain, and permits management at home. When the definitive procedure was carried out at 1–1.5 yr of age, the mesocolon attached to the ileocolostomy segment employed in this side-to-side anastomosis was severed, so that this segment could be mobilized (attached only by the ileal mesentery) as far as the distal pelvis, where a Swenson-type procedure was carried out. The colonic segment had acquired a vascular supply from the ileal mesentery, sufficient to retain viability when the mesocolon was divided. This segment of colon looks normal by sigmoidoscopy, retains its ability to absorb water, and prevents diarrhea after the pull-through procedure. When employed in managing infants with extensive aganglionosis, this technique has the following advantages: (a) it permits home care because of earlier development of formed stools; and (b) it provides the same advantages in the ultimate intestinal reconstruction.

Congenital stenosis involving a long segment of the trachea: Further experience in reconstructive surgery

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.

Gastroschisis: A 17-year experience

From 1970 to 1987, 23 neonates with gastroschisis were treated at Kobe Childrens 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.

Extensive aganglionosis: Further experience with the colonic patch graft procedure and long-term results*

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.

Foramen of Morgagni hernia in identical twins : is this an inheritable defect ?

Twins with Downs syndrome, foramen of Morgagni hernias, and similar cardiac anomalies are described. While diaphragmatic hernias are not uncommon, the occurrence of this congenital defect in twins with very similar congenital anomalies raises the possibility that diaphragmatic hernias may result from an inheritable defect.

Aortosternopexy for tracheomalacia following repair of esophageal atresia: Evaluation by cine-CT and technical refinement

During the period of 1 year (1988 to 1989), five infants, aged 3 weeks to 10 months, presented with recurrent respiratory distress following repair of esophageal atresia with tracheoesphageal fistula (EA/TEF). These patients had associated congenital anomalies, including right aortic arch (1), biliary atresia (1), and a long gap that required esophageal elongation by spiral myotomy (1). The patients were evaluated for tracheomalacia using cinecomputed tomography (cine-CT; C-100 Scanner, Imatron, San Francisco, CA), which provides images of eight levels (8-mm interval) simultaneously with 0.7 second time intervals of cine-CT. Dynamic studies of the trachea by cine-CT showed tracheal collapse that was most significant during expiration in the segment immediately above and at the aortic arch. These patients underwent aortosternopexy. Via a right second intercostal approach, the ascending aorta and aortic arch were lifted anteriorly using two to four sutures of 5-0 Tevdek on pledgets placed between the tunica media and adventitia of the side walls of the aorta and the adjacent sternum. Respiratory distress was significantly improved postoperatively. Preliminary experience with these patients allows us to conclude that (1) cine-CT is a useful technique for diagnosing tracheomalacia; (2) it provides objective indication for its correction by aortosternopexy; and (3) the refined technique in placing sutures on the aorta may reduce the surgical risks of aortosternopexy.

A new approach for the salvage of unsuccessful esophageal atresia repair: a spiral myotomy and delayed definitive operation.

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.

Percutaneus transhepatic cholangiodrainage after hepatic portoenterostomy for biliary atresia

During the past 12 mo, percutaneous transhepatic cholangiodrainage (PTCD) was carried out in 9 infants (12 procedures) who had undergone hepatic portoenterostomy (8) and hepatic portocholecystostomy (1) for biliary atresia. Bile excretion following surgery was observed in all infants. In all patients, cholangiogram at the initiation of PTCD was successful in visualizing the intrahepatic biliary system. PTCD was achieved in 5 infants (9 procedures). In infants with cholangitis, cultures of the intrahepatic bile were positive for enteric flora. Direct administration of antibiotics via the PTCD catheter into the bile ducts was transiently effective in the management of cholangitis. In one patient, it was observed that continuous PTCD over three weeks was effective in reducing serum bilirubin by relief of cholestasis. This technique is useful for: (1) demonstration of the reconstructed biliary system, (2) collection of bile from the intrahepatic biliary system for biochemical and bacteriologic studies, (3) direct administration of antibiotics to the bile ducts for cholangitis and (4) decompression of the intrahepatic system and relief of biliary stasis.

Multiple intestinal atresia and amyoplasia congenita in four unrelated infants: A new association

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.