Shinkichi Kamata

Anomalies of the tracheobronchial tree in patients with esophageal atresia

To examine associated anatomic anomalies of the tracheobronchial tree and structural abnormalities of the trachea, and their clinical consequences, bronchoscopic examination and follow-up studies were performed in 32 patients with esophageal atresia (EA) and tracheoesophageal fistula (TEF) over a 5-year period. To evaluate structural abnormalities of trachea, the ratio of circumferential length of cartilaginous trachea to circumferential length of membranous trachea (C:M ratio) was determined before surgical repair. In patients examined before and after surgical repair, the motion of the posterior tracheal wall was observed under spontaneous respiration. Fifteen of the 32 patients (47%) had abnormalities of the tracheobronchial tree such as ectopic right upper bronchus (12), congenital bronchial stenosis (4), and absence of the right upper bronchus (2). The C:M ratio of the 27 patients with EA (2.0 +/- 0.6) was significantly lower than that of controls (3.0 +/- 0.3); the ratio for the 10 cases with ectopic right upper bronchus (1.7 +/- 0.4) was significantly lower than that of other cases (2.2 +/- 0.5). The C:M ratio for the 12 patients who had atelectasis (1.7 +/- 0.3) was significantly lower than that of the 15 who did not have atelectasis (2.2 +/- 0.6). Of 24 infants, nine had bulging of the posterior tracheal wall synchronized with expiration, and their C:M ratio (1.8 +/- 0.2) was significantly lower than that of the 15 patients without bulging (2.2 +/- 0.6). These data show an association between anatomic anomalies of the tracheobronchial tree and structural abnormalities of the trachea in patients with EA. Because these abnormalities may be related to the occurrence of respiratory disorders such as tracheomalacia and atelectasis, early bronchoscopic examination, including measurement of the C:M ratio and observation of the motion of the posterior tracheal wall under spontaneous respiration, may be useful in the care of patients with EA.

The Dipeptide Alanyl-Glutamine Prevents Intestinal Mucosal Atrophy in Parenterally Fed Rats

This study was performed to determine whether the addition of alanyl-glutamine (Ala-Gln) can prevent intestinal mucosal atrophy induced by standard solution of total parenteral nutrition (S-TPN). Forty-one male Sprague-Dawley rats weighing 250 g were randomly divided into four groups: group I was killed after overnight fasting; group II received S-TPN. The other groups received S-TPN supplemented with amino acids other than glutamine (group III) or supplemented with Ala-Gln 2 g/100 mL (group IV); both solutions were isocaloric and isonitrogenous. After 1 week of TPN the rats were killed, and the duodenum, proximal jejunum, mid-small bowel, and distal ileum were obtained for morphologic and functional analysis. Weight gain did not differ significantly among these four groups, and there was no difference in nitrogen balance between groups III and IV. Serum glutamine in group IV (102.8 +/- 13.3 mumol/dL) was significantly increased (p less than .05) compared with groups I, II, and III (66.2 +/- 3.9, 55.7 +/- 7.8, and 61.3 +/- 10.8 mumol/dL, respectively). Mucosal wet weight, protein, RNA, sucrase, and maltase of group IV were significantly increased (p less than .05) compared with groups II and III. Villus height was significantly increased (p less than .05) in the jejunum of group IV rats compared with groups II and III, but not in any other segments of the intestine. No significant changes were observed in crypt depth among all groups. Diamine oxidase in groups II, III, and IV was significantly decreased (p less than .05) compared with group I in all segments except for the ileum.(ABSTRACT TRUNCATED AT 250 WORDS)

Esophageal atresia in Osaka: A review of 39 years' experience☆

BACKGROUND One hundred fifty-nine patients who had esophageal atresia with or without tracheoesophageal fistula have been treated at Osaka University Medical School and its affiliated hospitals since the initial (Japanese) experience of Dr T. Ueda in 1957. METHODS These cases were divided chronologically into three groups. With earlier recognition of surgical neonates and the development of perinatal care, the long-term survival of these patients has steadily improved over 39 years from 28% in the first period (1957 to 1967) to 80% in the third period (1980 to 1995). Of 141 patients treated in the second and third periods (1968 to 1995), 92 (65.2%) had associated anomalies. Cardiovascular and gastrointestinal malformations were the most frequently seen major anomalies. VATER or VACTER association was seen in 12.8% (18 of 141) of these patients. Survival of these cases according to Waterston risk factors was 100% for group A, 100% for group B, and 50% for group C, whereas the new classification proposed by Spitz showed survival of 92% for group 1, 50% for group 2, and 0% for group 3, showing better differentiation among the three groups. RESULTS There was a long gap between the proximal and distal esophageal ends in seven patients (type A), in all of whom primary anastomosis was possible after 28 to 128 days of elongation by bouginage. Although the survival of esophageal atresia patients dramatically improved in recent years, there is still a high incidence of early and long-term postoperative complications, ie, anastomotic leakage (26.5%), recurrent fistula (7.2%), anastomotic stricture (49.1%), postoperative pneumonia or atelectasis (57.0%), tracheomalacia (25.8%), and gastroesophageal reflux (52.0%). CONCLUSIONS Recently, there have been changing patterns in the occurrence of complications, which are mainly attributed to technical improvement, better perinatal care and early recognition of pathophysiologic conditions such as tracheomalacia and gastroesophageal reflux.

Total parenteral nutrition decreases luminal mucous gel and increases permeability of small intestine.

The distribution of fluorescein isothiocyanate dextran 70,000 (FITC-dextran) and mucous gel across the lumen of small intestine was observed as an investigation into the role of mucous gel on permeability in total parenteral nutrition (TPN). Thirty-two rats were randomly divided into two groups fed with either TPN or oral rat food. On day 4 or 7, FITC-dextran (750 mg/kg body weight) was given through the gastroduodenal tube. After 1 hour, blood samples were taken by aortic puncture to analyze plasma FITC-dextran by fluorescence spectrometry. Samples of small intestine with luminal contents were frozen and sectioned in a cryostat for fluorescence microscopy; the same sections were placed in a 0.2% celloidin solution for 3 minutes to preserve mucous gel and stained by periodic acid-Schiff reaction for light microscopy. The plasma level of FITC-dextran after 1 hour of this marker injection showed a significant increase (p < .01) in the TPN group compared with the rat food group on days 4 and 7. Morphologic findings on days 4 and 7 were similar in both the jejunum and ileum: The mucous gel filled the spaces between villi and FITC-dextran centered in the lumen in the rat food group, whereas the mucous gel decreased and FITC-dextran filled the spaces between villi in the TPN group. FITC-dextran and mucous gel showed complementary distributions in both groups. These data suggest that TPN decreases luminal mucous gel and increases permeability of small intestine in rats.

The effect of metronidazole on TPN-associated liver dysfunction in neonates

The effect of metronidazole (MNZ) on hepatic dysfunction associated with total parenteral nutrition (TPN) in neonates was investigated. Neonates receiving TPN for more than 2 weeks were divided into three groups. In group 1, TPN was given alone, in group 2, 25 mg/kg/d of MNZ was administered intravenously for the first 2 weeks of TPN, and in group 3, 50 mg/kg/d of MNZ was given for the first 3 weeks of TPN. Several parameters of liver function tests (LFTs) during the first 4 weeks of TPN were compared among these three groups. There was no significant difference of these parameters between group 1 and group 2. Although there was no significant difference of alkaline phosphatase, gamma-glutamyl transpeptidase, direct bilirubin, and total bile acid between groups 1 and 3, transaminase (glutamic oxaloacetic, glutamic pyruvic) of group 3 remained significantly lower than those of group 1. In conclusion, the administration of MNZ 50 mg/kg/d for 3 weeks, at least, prevented the elevation of transaminase during TPN in neonates, suggesting the possible involvement of intestinal anaerobic flora in the pathogenesis of TPN-associated liver dysfunction.

Anterior sagittal anorectoplasty for rectovestibular and anovestibular fistula

Rectovestibular (or anovestibular) fistula is the most common form of anorectal anomaly in female infants. In the surgical repair of these malformations, anal transplantation has been in widespread conventional use. Based on the idea of posterior sagittal anorectoplasty, we devised a new approach, anterior sagittal anorectoplasty, in which, in the lithotomy position, the anterior portion of sphincter muscles are cut through a median perineal skin incision and then the rectum is pulled through the center of these muscles to make its opening at the normal position. The operation has been performed in 12 patients with satisfactory results.

Prenatal diagnosis of congenital diaphragmatic hernia and perinatal care: assessment of lung hypoplasia

To assess the severity of lung hypoplasia, we have attempted to measure the lung thorax transverse area ratio (L/T) by using ultrasonic echography and to select immediate surgery after delivery by caesarean section. The evaluation of L/T for an index of lung hypoplasia was made by arterial blood gas data and clinical courses. Of 14 fetuses diagnosed, 13 had left sided CDH and one right sided case, from 11 to 38 weeks of pregnancy, 10 cases survived. The L/T in 14 fetuses with CDH was from 0.08 to 0.36 (mean 0.2 +/- 0.073) and was significantly lower than that of the controls. L/T was correlated best with data of arterial pH, PCO2, preductal A-aDO2 before operation and the duration of mechanical ventilation in survivors. Although L/T was also significantly low in the cases with severe grade, diaphragmatic patch closure and ECMO therapy, no significant differences were noted in L/T between survivors and non-survivors. These results indicated that L/T may predict the severity of lung hypoplasia in CDH and that the combination of perinatal management bases on prenatal diagnosis of CDH and ECMO support may improve the outcome of fatal CDH with severe lung hypoplasia.

Experience in tracheobronchial reconstruction with a costal cartilage graft for congenital tracheal stenosis

Although successful surgical management of congenital tracheal stenosis has been reported, it is still controversial as to the best operative procedure. Eleven infants with congenital tracheal stenosis were evaluated to confirm the efficacy of tracheobronchial reconstruction with costal cartilage graft. Symptoms ranged from recurrent respiratory infection to severe respiratory failure. All infants had other congenital anomalies in addition to tracheal stenosis. Notably, five infants had pulmonary artery sling and four infants had patent ductus arteriosus. Definitive diagnosis was made by bronchoscopy, results of which showed complete tracheal rings in all patients with severely compromised tracheobronchial lumens. Five infants had elongated stenosis involving nearly the whole length of the trachea, and five infants had segmental stenosis involving nearly one half the length of the trachea. One infant had bilateral stenosis of the main bronchi. Early experience included two deaths from problems related to the repair. The involvement of the carina and the distal portion of the trachea was associated with increased complications and a higher mortality rate. Currently, our preferred technique facilitated by extracorporeal membrane oxygenation (ECMO) includes carinal reconstruction with a thin-wall intraluminal stent. Bronchoscopy is essential for accurate intraoperative incision of the trachea, post-operative airway management for several weeks, and removal of the intraluminal stent.

Prenatal diagnosis of abdominal wall defects and their prognosis

To determine whether associated anatomic features may be useful in predicting fetal prognosis, 43 fetuses who had ultrasonographic evaluation for abdominal wall defects during a 13-year period were reviewed. Thirty-one fetuses had omphalocele and were classified into three groups according to fetal ultrasonography results: ruptured omphalocele with exposed liver, giant omphalocele with exposed liver, and small omphalocele without liver herniation. Twelve fetuses had gastroschisis. Ten of the 12 fetuses with gastroschisis survived; one died in utero. Nine of the 12 with a small omphalocele survived. Ten of 12 fetuses with giant omphalocele survived, but six of the seven with ruptured omphalocele died of pulmonary hypoplasia and respiratory insufficiency. Ruptured omphalocele recognized in utero was accompanied by intrauterine growth retardation and liver herniation and frequently was associated with deformity of the spine, diaphragmatic defects, vesicointestinal fissure, and meningocele. These results indicated that, together with the deformity of the spine, rupture and absence of the covering membrane with an exposed liver (noted through fetal ultrasonography) may suggest a poor prognosis because of pulmonary hypoplasia.

Influence of glutamine-supplemented parenteral nutrition on intestinal amino acid metabolism in rats after small bowel resection.

Glutamine (Gln)-supplemented total parenteral nutrition (TPN) has been shown to improve mucosal adaptation after massive small bowel resection (SBR); however, its influences on intestinal amino acid metabolism remain unknown. In this study, intestinal amino acid flux, circulating plasma aminogram, mucosal glutaminase activity and protein, and DNA content were measured 7 days after massive SBR in rats receiving either standard (Std) or Gln-supplemented TPN. Sham-operated rats and rats fed chow after enterectomy served as controls. The uptake of Gln and the release of citrulline (Cit) by the remaining intestine was significantly decreased, with reduced mucosal glutaminase activity after SBR in the Chow and Std-TPN groups. Glutamine supplementation resulted in significantly increased gut Gln uptake compared with Std-TPN (P<0.01). Mucosal glutaminase activity, mucosal protein, and DNA content was also increased by Gln; however, the gut release of Cit remained unchanged (P>0.05). The subsequent decrease in circulating arginine (Arg) in the Gln-TPN group compared with the Std-TPN group (P<0.05) was attributed to an insufficient exogenous supply. These findings show that Gln-supplemented TPN improves mucosal growth and gut Gln uptake after SBR. However, the intestinal production of Cit, which remained low in both TPN groups, may lead to an insufficiency of endogenous Arg synthesis. Thus, both Gln and Arg may be essential amino acids after SBR.