Shigeru Takamizawa

Esophageal atresia: Prognostic classification revisited

BACKGROUND Although the Spitz classification is the most widely used prognostic classification for esophageal atresia and/or tracheoesophageal fistula (EA), its discrimination ability remains unclear. We sought to develop a more accurate prognostic classification for EA. METHODS The records of 121 consecutive infants with EA (1980-2005) were reviewed. The independent variables included 6 clinical characteristics, and the dependent variables were survival and mortality. Stepwise logistic regression analysis was used to construct models predicting mortality and create a revised prognostic classification. The discrimination abilities of the revised classification and the Spitz classification were compared using receiver-operating characteristic (ROC) curves. RESULTS Birth weight and the presence of major cardiac anomalies were significant prognostic factors for mortality, and major cardiac anomalies affected mortality more than birth weight. The ROC curve for birth weight suggested that 2,000 g was an appropriate cutoff point. The Spitz classification was revised as follows: the revised class I (low-risk group) consisted of patients without major cardiac anomalies and birth weight >2,000 g; class II (moderate-risk group) consisted of patients without major cardiac abnormalities and birth weight <2,000 g; class III (relatively high-risk group) consisted of patients with major cardiac anomalies and birth weight >2,000 g; and class IV (high-risk group) consisted of patients with major cardiac anomalies and birth weight <2,000 g. The ROC comparisons showed that the revised classification provided a significant improvement (P = .049). CONCLUSION This revised classification can improve the stratification of EA patients and be a useful predictor of survival.

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.

Surgical intervention for esophageal atresia in patients with trisomy 18

Trisomy 18 is a common chromosomal aberration syndrome involving growth impairment, various malformations, poor prognosis, and severe developmental delay in survivors. Although esophageal atresia (EA) with tracheoesophageal fistula (TEF) is a potentially fatal complication that can only be rescued through surgical correction, no reports have addressed the efficacy of surgical intervention for EA in patients with trisomy 18. We reviewed detailed clinical information of 24 patients with trisomy 18 and EA who were admitted to two neonatal intensive care units in Japan and underwent intensive treatment including surgical interventions from 1982 to 2009. Nine patients underwent only palliative surgery, including six who underwent only gastrostomy or both gastrostomy and jejunostomy (Group 1) and three who underwent gastrostomy and TEF division (Group 2). The other 15 patients underwent radical surgery, including 10 who underwent single‐stage esophago‐esophagostomy with TEF division (Group 3) and five who underwent two‐stage operation (gastrostomy followed by esophago‐esophagostomy with TEF division) (Group 4). No intraoperative death or anesthetic complications were noted. Enteral feeding was accomplished in 17 patients, three of whom were fed orally. Three patients could be discharged home. The 1‐year survival rate was 17%: 27% in those receiving radical surgery (Groups 3 and 4); 0% in those receiving palliative surgery (Groups 1 and 2). Most causes of death were related to cardiac complications. EA is not an absolute poor prognostic factor in patients with trisomy 18 undergoing radical surgery for EA and intensive cardiac management.

Congenital tracheal stenosis: the prognostic significance of associated cardiovascular anomalies and the optimal timing of surgical treatment

BACKGROUND/PURPOSE Cardiovascular anomalies (CA) are frequently associated with congenital tracheal stenosis (CTS), but their prognostic impact on CTS and the optimal timing of surgical treatment remain uncertain. The aim of this study was to explore the prognostic factors and the optimal timing of surgical treatment in CTS patients with CA. METHODS After obtaining institutional review board approval, a retrospective review of 42 patients who underwent surgical repair of CTS between 1996 and 2006 was conducted. The patients were divided into 3 groups: CTS without CA (n = 10, group A), CTS with CA repaired simultaneously (n = 27, group B), and CTS with CA repaired in stages (n = 5, group C). Seven clinical characteristics, including gestational week and weight at birth, the age and body weight at operation, the length of tracheal stenosis (%), the duration of cardiopulmonary bypass (CPB) during surgery, and operation time were compared among the groups using analysis of variance, Fishers Exact test, and Students t test. RESULTS Although no operative mortalities occurred in groups A and C, there were 3 early deaths and 1 late death in group B. The deaths occurred in cases with associated complex CA (critical pulmonary stenosis, tetralogy of Fallot with an absent pulmonary valve, right ventricular outflow block, and cor triatrium). The duration of CPB was significantly different between groups A and B (P = .017), and furthermore, CPB time was significantly longer in early death cases than in surviving cases in group B (318.3 +/- .71.1 vs 204.0 +/- 67.8 minutes; P = .012). CONCLUSIONS Complex CA and long CPB duration would be prognostic factors for the outcome of surgical management for CTS and CA. Simultaneous reconstruction of CTS and simple CA appears to be a reasonable method of surgical intervention, but patients with long segment CTS with complex CA may still be difficult to cure using this strategy, and staged correction may be considered.

Communicating bronchopulmonary foregut malformation: particular emphasis on concomitant congenital tracheobronchial stenosis

A total of four patients with communicating bronchopulmonary foregut malformation were treated surgically at Kobe Children’s Hospital between 1993 and 2004. Of these, three patients displayed congenital tracheobronchial stenosis and developed life-threatening respiratory distress soon after birth. In each case, anomalous bronchi arose from the lower portion of the esophagus and connected to the lower part of the ipsilateral lung. This anomaly involved the right lung in two patients, and the left lung in one patient. Tracheobronchial stenosis extended from the inlet of the thorax to the carina in one patient, and to the contralateral main stem bronchus in two patients. Surgical treatment included division of the esophageal bronchus and anastomosis of bronchus to the trachea in one patient. In the other patient, the ipsilateral lung was resected and the stenotic tracheobronchus was stented. The remaining patient underwent pneumonectomy of the ipsilateral lung. Details of this fatal anomaly and a discussion of appropriate surgical management are described herein.

Pretransplant risk factors and optimal timing for living-related liver transplantation in biliary atresia : experience of one Japanese children's hospital and transplantation center

BACKGROUND/PURPOSE Although living-related liver transplantation (LRLT) is effective for patients with biliary atresia (BA) after a failed Kasai operation, the pretransplant factors affecting post-LRLT mortality and the optimal timing of the procedure remain unclear. METHOD A retrospective review of 27 patients with BA after a failed Kasai operation (median age, 22 months; range, 6-237 months) who received LRLT from 1994 to 2005 was done. The clinical characteristics at the time of the pre-LRLT assessment of those who did and did not survive were compared. A simple regression analysis and receiver operating characteristic analysis were done to correlate the clinical data. RESULTS Among the 27 patients, 4 patients died within 1 year post-LRLT. The significant factors affecting posttransplant death were hepatopulmonary syndrome (HPS), age at LRLT, and graft-to-recipient weight ratio. The arterial blood gas analysis of HPS patients showed that there was a significant negative correlation between the SaO(2) value on room air and the intrapulmonary shunt ratio. The receiver operating characteristic analysis of age at LRLT showed that the optimal cutoff point was 103 months of age. CONCLUSION Older children with HPS or a lower graft-to-recipient weight ratio are not ideal candidates for LRLT. The correlation between the shunt ratio and SaO(2) suggests that HPS could be detected early using pulse oximetry.

The preliminary study of modified Swenson procedure in Hirschsprung disease

PURPOSE We have been using the Swenson procedure for more than 3 decades for Hirschsprung disease (HD). Recently, we modified this procedure, leaving the anterior wall below the peritoneal reflection undissected (mSwen). In 2000, we introduced mSwen with laparoscopic guidance (LapmSwen). We hypothesized that (1) omitting anterior wall dissection would not affect postoperative anorectal function, and (2) reduced dissection with better visualization via laparoscopy would reduce operative risks. METHOD Charts of 89 patients with Hirschsprung disease operated on between 1990 through 2005 were retrospectively reviewed. Comparisons between Swen and mSwen, as well as between mSwen and LapmSwen, were analyzed in terms of operating time, blood loss, and complications. RESULTS Mean operating times (minutes) were 312 for Swen, 284 for mSwen (P = .152), and 302 for LapmSwen ( mSwen, P = .218). Mean blood loss (mL) were 64.8 for Swen, 60.3 for mSwen (P = .669), and 8.7 for LapmSwen (as compared to mSwen, P = .001). We noted leakage in 7 Swen, 2 mSwen, and no LapmSwen patients. There were no significant differences between Swen and mSwen, or between mSwen and LapmSwen, in the incidence of enterocolitis, constipation, and soiling. CONCLUSION LapmSwen appeared to be comparable to the standard and modified Swenson procedures in most measures. Operative blood loss may be reduced in the LapmSwen approach.

Population‐based study of esophageal and small intestinal atresia/stenosis

The aim of this study was to describe the prevalence of esophageal atresia/stenosis and small intestinal atresia/stenosis in Nagano, Japan, together with associated anomalies, prenatal diagnosis and survival.

Successful tracheobronchial reconstruction of communicating bronchopulmonary foregut malformation and long segment congenital tracheal stenosis: a case report

Communicating bronchopulmonary foregut malformation (CBPFM) and congenital tracheal stenosis (CTS) are difficult developmental disorders especially when they are presented simultaneously in a patient. The authors report a case of a newborn boy born at 37 weeks of gestation weighing 2356 g with CBPFM (right esophageal lung) and long segment CTS. Staged surgical repair (by-force endotracheal intubation for securing the airway followed by bronchotracheal anastomosis for CBPFM, tracheostomy with handmade, length-adjustable tracheostomy tube, and slide tracheoplasty) was performed. He has been healthy without tracheostomy for 25 months after slide tracheoplasty. This is the first report of a successful tracheobronchial reconstruction for a patient with a long segment CTS and CBPFM preserving the affected lung function.

Efficacy of urinary sulfated bile acids for diagnosis of bacterial cholangitis in biliary atresia

This study evaluated the efficacy of measuring urinary sulfated bile acids (USBA) for diagnosis of bacterial cholangitis in patients with biliary atresia. Eight infants with biliary atresia were recruited. The USBA level was measured when they were admitted to hospital with a fever of unknown origin. Clinical manifestations and laboratory data were reviewed. The standard USBA value for each patient (S-USBA) was defined as the level measured when they had no fever, and USBA ratio (R-USBA) was calculated as the USBA level during the febrile episode divided by the S-USBA. Then R-USBA values were compared between febrile episodes with and without cholangitis to assess the diagnostic ability of USBA. Twenty-three febrile episodes occurred in eight patients during a 15-month period. Nine episodes were diagnosed as being due to cholangitis, five were due to non-cholangitis, and nine were of undetermined origin. The R-USBA value ranged from 1.5 to 15.4 during cholangitis episodes and from 0.4 to 1.2 during non-cholangitis febrile episodes. When fever was of undetermined origin, R-USBA was found to be increased during some episodes and not in others. USBA increased immediately in patients with cholangitis. The measurement of USBA is a useful non-invasive test for cholangitis in patients with biliary atresia who had undergone Kasai’s operation.