A case of esophageal atresia with the bronchial-like lower esophagus which originates from the left lower lobe bronchus.

BACKGROUND: Esophageal atresia with or without a trachea-esophageal fistula occurs due to the failure of separation or incomplete development of the foregut. Therefore, esophageal atresia is often associated with various forms of tracheobronchial anomalies. We report an extremely rare case of esophageal atresia. CASE PRESENTATION: A female infant was born at 37 weeks of gestation and weighed 2596 g. A diagnosis of esophageal atresia and total anomalous pulmonary vein return type III were confirmed. The infant had respiratory distress that required tracheal intubation and ventilatory support soon after birth. Temporary banding of the gastroesophageal junction and gastrostomy were performed on the second day of life. However, her respiratory condition deteriorated due to atelectasis of the left lung and compensatory hyperinflation of the right lung. Preoperative examinations showed the unilobe and atelectatic left lung. The trachea was trifurcated in three directions, and the branch that was expected to be the left main bronchus was blind-ended. The dorsal branch was cartilaginous and bifurcated into the left lower lobe bronchus and lower esophagus approximately 1 cm distal from the tracheal trifurcation. The cartilaginous tissue continued to the lower esophagus. The diagnosis of esophageal atresia with the lower esophagus which originated from the left lower lobe bronchus was made. Esophageal atresia repair was performed when the patient was 4 months of age. The esophagus was dissected distally to the bifurcation of the left lower lobe bronchus via right thoracotomy. The lower esophagus was bronchial-like in appearance, transitioning to the normal esophageal wall approximately 7 mm distal to the transected edge. The cartilage tissue was completely resected during surgery, and a primary end-to-end anastomosis of the esophagus was successfully performed. Histopathological findings revealed that the extracted specimen was surrounded by tracheal cartilage and that the inner surface was covered by stratified squamous epithelium that originated from the esophagus. CONCLUSIONS: In cases of esophageal atresia with an atypical clinical presentation, there may be unique structural abnormalities of the foregut. We emphasize the importance of a preoperative surgical planning since an inadequate operation can lead to fatal complications.

Elimination of MYCN-Amplified Neuroblastoma Cells by Telomerase-Targeted Oncolytic Virus via MYCN Suppression.

Neuroblastoma (NB) is a primary malignant tumor of the peripheral sympathetic nervous system. High-risk NB is characterized by MYCN amplification and human telomerase reverse transcriptase (hTERT) rearrangement, contributing to hTERT activation and a poor outcome. For targeting hTERT-activated tumors, we developed two oncolytic adenoviruses, OBP-301 and tumor suppressor p53-armed OBP-702, in which the hTERT promoter drives expression of the viral E1 gene for tumor-specific virus replication. In this study, we demonstrate the therapeutic potential of the hTERT-driven oncolytic adenoviruses OBP-301 and OBP-702 using four human MYCN-amplified NB cell lines (IMR-32, CHP-134, NB-1, LA-N-5) exhibiting high hTERT expression. OBP-301 and OBP-702 exhibited a strong antitumor effect in association with autophagy in NB cells. Virus-mediated activation of E2F1 protein suppressed MYCN expression. OBP-301 and OBP-702 significantly suppressed the growth of subcutaneous CHP-134 tumors. Thus, these hTERT-driven oncolytic adenoviruses are promising antitumor agents for eliminating MYCN-amplified NB cells via E2F1-mediated suppression of MYCN protein.

Overgrowth of costal cartilage is not the etiology of pectus excavatum.

PURPOSE: The etiology of pectus excavatum (PE) has not been clarified. In 1944, Sweet (Sweet RH. Pectus excavatum. Ann Surg 1944;119:922-934) mentioned about the possibility of the overgrowth of costal cartilage being involved. However, no additional report that supports his hypothesis is available. In this study, we investigated whether the overgrowth of costal cartilage was an actual cause of PE through measurement of the costal cartilage length in PE patients and healthy controls. MATERIALS AND METHODS: We investigated the length of the fifth and sixth costal cartilages and ribs in PE patients from reconstructed images of 3-dimensional computed tomography. To examine the relative costal cartilage length, we calculated the C/R ratio, defined as the quotient of the costal cartilage length divided by the adjacent rib length, and compared it between PE patients and healthy controls. RESULTS: In PE patients, the C/R ratios were not larger than in healthy controls at any level. At the left sixth, the C/R ratio was significantly smaller in patients than in the healthy control group. DISCUSSION: The results revealed that, in PE patients, relative costal cartilage lengths were not longer than in healthy controls. We conclude that the overgrowth of costal cartilage is not the etiology of PE.

Umbilical center insertion method for initial trocar placement in pediatric laparoscopic surgery.

BACKGROUND: For laparoscopic surgery in pediatric patients, the initial trocar insertion is usually made employing open laparotomy to avoid injury to the viscera. It is safe but somewhat time-consuming for establishing a pneumoperitoneum, and requires additional sutures for anchoring or preventing air leakage. We devised a new initial trocar insertion technique employing an umbilical center incision to shorten the surgical duration and improve the esthetic appearance. PATIENTS AND METHODS: Four hundred and thirty-one pediatric patients were indicated for this umbilical center insertion method (UCM) at 2 pediatric surgical departments. Patients suspected of having umbilical lesions (except for umbilical hernia) or extensive bowel adhesions were excluded. RESULTS: There was no complication associated with UCM. In almost all patients, a pneumoperitoneum was established within 1 minute. The wound was completely within the umbilical ring, and very satisfactory esthetically. DISCUSSION: We consider UCM to be a safe and easy procedure, and acceptable method of initial trocar insertion for pediatric laparoscopic surgery.

Does overgrowth of costal cartilage cause pectus excavatum? A study on the lengths of ribs and costal cartilages in asymmetric patients.

PURPOSE: The cause of pectus excavatum has been hypothesized to be overgrowth of the costal cartilage. According to this theory, the length of costal cartilages must be longer in the side of deep depression in asymmetric patients. To challenge this hypothesis, we measured the lengths of ribs and costal cartilages and investigated lateral differences. SUBJECTS AND METHODS: Twenty-four adolescent and adult patients with asymmetric pectus excavatum (14-30 years of age) with no history of surgery were investigated in this study. The fifth and sixth ribs and costal cartilages were individually traced to measure their full lengths on 3-dimensional computed tomographic (CT) images. As an index of asymmetry, sternal rotation angle was measured in the chest CT images. Patients with a 21 degrees or greater angle of sternal twist were designated as an asymmetric group and those with an angle of smaller than 20 degrees as a symmetric group. Lateral differences in the fifth and sixth costal and costal cartilage lengths were compared between the groups. RESULTS: On comparison of the costal and costal cartilage lengths in the asymmetric group, the right fifth ribs and costal cartilages were significantly shorter than the left (P = .02 and .03, respectively), and right sixth ribs were also significantly shorter than the left (P = .004), but right sixth costal cartilages were not (P = .31). In the symmetric group, the lengths of the left and right fifth ribs and costal cartilages were showing no significant difference (P = .20 and P = .80, respectively), and those of the sixth ribs and costal cartilage were also showing no significant difference (P = .97 and P = .64, respectively). DISCUSSION: The ribs and costal cartilages on the right side with severer depression were significantly shorter or not different than those on the contralateral side. Based on these findings, the theory of costal cartilage overgrowth is contradictory. The etiology of asymmetric chest deformity should be reevaluated.

Successful reconstruction of communicating bronchopulmonary foregut malformation associated with laryngotracheoesophageal cleft.

A full-term newborn male infant presented with dyspnea and cleft lip and palate. He was thought to have esophageal atresia with tracheoesophageal fistula. He underwent bronchoscopy before operation that showed a laryngotracheoesophageal cleft (LTEC) type III. The left main bronchus originated from the lower esophagus. His diagnosis was communicating bronchopulmonary foregut malformation (CBPFM) type IA associated with LTEC type III. Enhanced chest computed tomographic scan showed the left pulmonary artery originated from the descending aorta. Staged operations were indicated. At first, reconstruction of the left pulmonary artery was done at 3 months of age. Then at 6 months of age, operations for LTEC (tracheoplasty and esophagostomy) and CBPFM left bronchoplasty were performed. Reconstruction of esophagus was performed at age of 1 year. He is now 3 years old and doing well with a mild degree of bronchomalacia. This is the first report of total reconstruction of CBPFM type IA associated with LTEC.