Toshihiro Muraji

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.

Diamond-shaped anastomosis for duodenal atresia: An experience with 44 patients over 15 years

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 transanastomotic 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.

Maternal Microchimerism in Underlying Pathogenesis of Biliary Atresia: Quantification and Phenotypes of Maternal Cells in the Liver

OBJECTIVE. The goal was to examine whether microchimerism plays a crucial role in the pathogenesis of biliary atresia; we analyzed the localization of maternal microchimeric cells and their phenotypes. METHODS. Liver biopsy specimens from 8 male infants with biliary atresia and 6 control subjects with other liver diseases were investigated for maternal chimeric cells and their phenotypes through double-staining fluorescence in situ hybridization and immunohistochemical analyses. RESULTS. Significantly larger numbers of maternal XX+ cells were found in the portal area and sinusoids of patients with biliary atresia, in comparison with control patients. In phenotypic analyses of XX+ cells, CD8+ T cells, CD45+ cells, and cytokeratin-positive cells were found, and the numbers and proportions among total CD8+ T cells were significantly higher than those in control patients. CONCLUSIONS. Significantly more maternal chimeric CD8+ T cells in the livers of patients with biliary atresia suggest that maternal immunologic insults represent the underlying pathogenesis in biliary atresia. The findings support the recently postulated mechanisms of alloautoimmune and/or autoalloimmune responses.

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

Abstract 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.

A shape memory airway stent for tracheobronchomalacia in children: An experimental and clinical study☆

The authors have designed a coil airway stent using a thermal shape-memory titanium-nickel alloy (SMA) to relieve airway collapse in children. A characteristic of alloy allowed the stent to be enclosed in the thin introducer tube and to position it bronchoscopically in the collapsed airway. When the stent is warmed to 37 to 40 degrees C, it expands to the memorized diameter and stents the airway. In eight rabbits, an experimental model of potentially fatal tracheomalacia was created by fracturing the tracheal cartilages. The stents of 6 mm in diameter and 15 mm in length were placed, and then the stents were recovered to their original shape within 1 minute. All rabbits except one showed no respiratory symptoms during the follow-up period. Results of bronchoscopy performed 6 and 10 months after implantation showed satisfactory patency of the trachea. The rabbits were killed for histological evaluation 6 to 28 months after implantation. The specimens showed little proliferation of granulation and no dislodgement of the stents. This procedure was attempted in two children who had severe bronchomalacia. One 5-year-old patient underwent implantation with a stent of 5 mm in diameter and 25 mm in length into the left main bronchus. The patient was relieved from apneic attacks. The stent was removed 2 years after implantation after a remarkable improvement of ventilation. The other patient with left bronchomalacia, age 1 year 2 months, underwent implantation with a 5-mm x 20-mm stent. The animal experiment and clinical experience indicated that (1) this stent can be easily inserted and removed bronchoscopically, (2) the stent has good tissue compatibility and little interference of mucociliary function, and (3) the SMA stent is a promising therapeutic adjunct in the management of children with severe tracheobronchomalacia.

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.

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.

Laryngotracheal Separation for Intractable Aspiration Pneumonia in Neurologically Impaired Children: Experience With 11 Cases

PURPOSE Laryngotracheal separation (LTS) with or without end-to-side laryngoesophagostomy was performed as an antiaspiration procedure for intractable aspiration pneumonia in 11 children. The effectiveness of LTS for preventing aspiration was investigated. METHODS Eleven children aged from 9 months to 16 years with intractable aspiration pneumonia underwent LTS with (n = 8) or without (n = 3) laryngoesophagostomy at our institution over the last 2 years. Of these 11 patients, 7 underwent fundoplication with or without gastrostomy for gastroesophageal reflux (GER) before LTS (n = 5) or concurrently with LTS (n = 2). The effectiveness of LTS was evaluated by chart review and follow-up phone questionnaires. RESULTS LTS decreased the frequency of performing suction from an average of once every 30 minutes to once every 4.5 hours in all patients. In 5 patients who underwent fundoplication with gastrostomy before LTS, aspiration pneumonia remained unless they underwent LTS. Two patients who underwent LTS with or without laryngoesophagostomy tolerated oral feeding postoperatively. All parents rated LTS as excellent or good in terms of improving the quality of life. CONCLUSIONS LTS can be recommended for neurologically impaired children with intractable aspiration as a primary surgical intervention. If patients show impaired swallowing and GER, LTS could be performed simultaneously with fundoplication and gastrostomy.