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Congenital esophageal stenosis (CES) is rare, and the available clinical data are limited. We explored the current diagnosis, treatment and outcomes of CES. A questionnaire survey was performed using medical records at pediatric surgical centers in the Kyushu area. Over 10 years, 40 patients (24 males) had CES. The incidence of associated anomalies was 52.5% (21/40), and that of esophageal atresia was 20.0% (8/40). The mean age at the diagnosis was 12.0 months (range, 1 day–8.8 years). Seven (17.5%) patients were diagnosed in the neonatal period. Ten (25.0%) developed CES due to tracheobronchial remnants, 27 (67.5%) due to fibromuscular stenosis (FMS) and 1 (2.5%) due to membranous stenosis + FMS. Thirty-six (90.0%) were treated by balloon dilatation (mean, 3 times; range, 1-20). Perforation at dilatation occurred in 7 (17.5%) patients, and all were diagnosed with FMS. Eighteen (45.0%) patients underwent radical operation (3 primary, 15 secondary to dilatation). Our study clarified the characteristics and outcomes of CES, including neonatal diagnoses. CES occurred in 1 in every 33,000 births in the Kyushu area. Careful attention should be paid, even in cases of dilatation for FMS. CES requires long-term follow-up for symptom persistence after adequate and repeated treatment.

Treatment for a large abdominal wall defect remains challenging. The aim of this study was to optimize tissue engineering therapy of muscle constructs using a rat model. Experimental abdominal wall defects were created in Wister rats. The animal model was divided into three groups: collagen sponge (CS), hybrid scaffold (HS) and hybrid scaffold containing bone marrow liquid (HSBM). Hybrid scaffolds comprised collagen sponge and poly L-lactide (PLLA) sheets. Abdominal wall defects were covered by three kinds of sheets. Thereafter, the bone marrow liquid was spread onto the sheets. Rats were killed at 4, 8, and 16 weeks. Pathological examinations were performed using hematoxylin–eosin and desmin antibody staining. The CS group showed abdominal hernia, whereas the HS and HSBM groups did not. Vascular formation was confirmed in all groups. Muscle tissue was recognized at the marginal area of the sheet only in the HSBM group. The HS and HSBM groups show a greater intensity than the CS group. Muscle tissue regeneration is solely recognized in the HSBM group. Our experimental data suggest that the triad of scaffold, cell, and growth factor is fundamental for ideal biomaterials. The HSBM may be useful for reconstruction of abdominal wall defects.

While the diagnosis and outcomes of esophageal atresia (EA) have improved, associated anomalies, the management of late complications and growth remain major issues. We analyzed factors that affected the prognosis, late complications and growth. We retrospectively reviewed EA patients treated at two centers from 1984 to 2016. Patient characteristics, complications (gastroesophageal reflux [GER], anastomotic stenosis, tracheomalacia, dysphagia) and growth were evaluated. Seventy-three EA patients were treated (overall survival rate:80.8%). The mean birth weight was 2514 ± 509 g in the surviving group, and 2453 ± 567 g in the fatal group excluded chromosomal abnormality (p = 0.76). Cardiac and chromosomal anomalies significantly affected mortality. Postoperative GER and anastomotic stenosis each occurred in 39% of the patients. Only GER was significantly affected by the Gross classification. The standard deviation (SD) values of the EA patients’ growth were all lower than in the normal population. The SD of body weight was significantly lower in patients with extremity anomalies. Associated cardiac and chromosomal anomalies significantly affected the prognosis. GER and anastomotic stenosis were the most common late complications. The growth of the surviving cases was insufficient. These factors will help optimize the therapeutic strategies and postoperative management for EA.

To determine the most appropriate treatment strategy for neonates with coexisting congenital heart diseases (CHD) requiring surgery, 408 neonates treated in our department between 2002 and 2009 were retrospectively reviewed. Of the 408 neonates, 37 (9.1%) had coexisting CHD. Among the pediatric diseases requiring surgery, esophageal atresia (46.7%), anorectal malformation (24.1%), and tracheobronchial anomalies (75.0%) were frequently associated with CHD, and the survival rate among patients with coexisting CDH was 24.3%, about 2-fold higher than among those without coexisting CHD. Clinical observations in the present series suggested that fatal conditions such as tracheoesophageal fistula should be repaired immediately after birth, followed by palliative cardiac surgery to stabilize the circulatory status if necessary. Thereafter, radical repair of cardiac and pediatric anomalies should be performed, taking into account the balance between surgical stress and cardiac reserve. For this purpose, proper assessment of surgical stress and cardiac function and collaboration among specialists in relevant fields are essential during the perinatal period to determine the treatment strategy for patients with multiple-organ anomalies.