Optimal Surgical Method and Timing for Low-birth-weight Esophageal Atresia Babies: Multi-institutional Observational Study.

AIM OF THE STUDY: Previous research has shown that low birth weight is one of the risk factors for esophageal atresia. However, there remains a paucity of evidence on the timing and the treatment method. METHOD: Data were collected using a multi-institutional observational study in 11 hospitals that performed surgeries on esophageal atresia babies whose birth weights were ≤1500 g from 2001 to 2020. RESULTS: Of the 46 patients analyzed, median birth weight was 1233 (IQR 1042-1412) g. Within 46 cases, 19 (41%) underwent definitive esophageal anastomosis at the median of age in 8 (IQR 2-101) days. Thirteen out of 19 experienced either closure of tracheoesophageal fistula, gastrostomy, or esophageal banding at the first operation, followed by esophageal anastomosis. Seven infants, including four cases of <1000 g, underwent anastomosis after one month of age to wait for weight gain (variously 2-3000 g). Twenty-one out of 27 infants (78%) who did not receive anastomosis died within one year of age, including 21 (78 %) with major cardiac anomalies and 24 (89%) with severe chromosomal anomalies (trisomy 18). Six survivors in this group, all with trisomy 18, lived with palliative surgical treatments. CONCLUSION: In our study, the definitive esophageal anastomosis was effective either at the first operation or as a later treatment after gaining weight. Although having severe anomalies, some infants receive palliative surgical treatments, and the next surgery was considered depending on their condition. EVIDENCE LEVEL: II.

Infantile macrocephaly and multiple subcutaneous lipomas diagnosed with PTEN hamartoma tumor syndrome: A case report.

A heterozygous loss-of-function mutation of the PTEN gene, one of the tumor suppressor genes, causes a wide variety of disorders, ranging from macrocephaly/autism syndrome to PTEN hamartoma tumor syndrome, including Cowden disease that causes thyroid and breast cancer mainly in the adolescence and young adult generation. An 8-month-old male infant with simple macrocephaly developed a café-au-lait spot and two subcutaneous tumors at the age of 1 year. One of the tumors developed rapidly was resected at the age of 1 year and 9 months and identified as benign lipoma. From the age of 2 years, the patient often threw a tantrum. At the age of 2 years and 9 months, a pathogenic germline mutation was identified in the PTEN gene (NM_000314.7), c.195C>A, p.Y65* in the form of a heterozygous germline variant. Developmental delay was noted but no tumors were found in the thyroid gland and breasts. Immunohistochemistry for PTEN in the resected lipoma demonstrated that the PTEN expression pattern was similar to that in a subcutaneous adipose tissue from a normal subject, suggesting that two-hit was not likely involved in the rapid growth of this lipoma. At the age of 5 years, the patient was diagnosed with autism spectrum disorders with moderate developmental delay. A long-term follow-up is underway to examine developmental changes in psychomotor disorders and possible tumor formation.

Anterior-posterior cricoid split combined with silastic T-tube stenting for subglottic stenosis in children: a single surgeon's experience.

PURPOSE: Treatment strategies and clinical outcomes of subglottic stenosis (SGS) in children are varied due to the degree and range of stenotic lesions. The optimal surgical procedure for SGS in children is still under debate. The aim of this study was to evaluate the clinical outcomes of our anterior-posterior cricoid (APC) split technique combined with long-term T-tube stenting for grade II or III SGS in children. METHODS: A retrospective chart review of children with SGS between January 2011 and December 2016 was conducted. APC split was performed via open procedure under rigid bronchoscopy. After splitting, a silastic T-tube was inserted as a stent and removed 6 months postoperatively. RESULTS: Seven children underwent APC split during the period. All children had undergone previous tracheostomy, and APC split was performed when the children were 3-9 years old without any intraoperative complications. Median duration of T-tube stenting was 11 months, and all children were decannulated successfully. There were T-tube-related complications, including two tube-tip granulation that required intervention and one accidental T-tube removal. CONCLUSION: APC split is a technically simple and reproducible procedure, and it could be employed as an optimal procedure for SGS in children.

Long-term T-tube stenting as definitive treatment of severe acquired subglottic stenosis in children.

PURPOSE: We analyzed our results of long-term T-tube stenting for severe acquired subglottic stenosis secondary to prolonged endotracheal intubation in a neonatal period. MATERIALS AND METHODS: Twenty children treated with T-tube stenting since 1999 were retrospectively analyzed. T-tube stenting consisted of anterior cricoid split and placing silicon T-tube as a stent for expanded subglottic lumen. RESULTS: Fourteen of 20 patients were treated as an initial operation and 6 patients as an additional operation after other failed procedures. Decannulation was achieved in 8 (57.1%) of 14 children in the initial operation group and in 4 (66.7%) of 6 patients in the additional operation group. The average duration of T-tube stenting was 16.1 months in the initial group and 65.8 months in the additional group. Four of 20 patients continued to require T-tube stenting. In the other 4 patients, T-tubes were switched to tracheostomy tubes. The voice quality improved gradually after decannulation. CONCLUSION: T-tube stenting for severe acquired subglottic stenosis is recommended as a best available treatment in postoperative quality of life. T-tube stenting should be performed as the initial treatment because the time to decannulation was shorter than as an additional operation.

Insignificant seasonal and geographical variation in incidence of biliary atresia in Japan: a regional survey of over 20 years.

PURPOSE: Biliary atresia (BA) is the leading cause of obstructive jaundice in the newborn and the major indication for liver transplantation in Japan. Viral infection has been implicated in its etiology because of seasonality and space clustering. However, this has been controversial among whites. The present study investigates space-time clustering of the incidence of BA in Japan. METHODS: Birth prevalence rates of BA were analyzed in infants born in Hyogo prefecture between 1985 and 2004 to assess time clustering among 3 seasons. The birth prevalence rates were also analyzed for space clustering between the northern (Hokkaido) and the southern islands (Kyushu) (difference of latitude, 10 degrees ) based on the Japanese Biliary Atresia Society Survey 1996-2004. We compared the prevalence rates between these groups using relative risks (RRs) calculated from 2 x 2 contingency tables. RESULTS: One hundred nineteen infants were born with BA in Hyogo (1.1/10000 live births). Seasonal clustering in April to July did not significantly differ from that of the reference period (RR, 1.45; 95% confidence interval, 0.93-2.27). Based on Japanese registry data, space clustering between the northern and southern islands did not significantly differ (RR, 1.24; 95% confidence interval, 0.83-1.86). CONCLUSIONS: Neither seasonal nor spatial clustering was statistically proven in Japan.

MCRA of an anastomotic stenosis after esophagoesophagostomy for long gap esophageal atresia: a case report.

A 25-month-old boy with long gap esophageal atresia developed severe esophageal stenosis refractory to balloon dilatations after definitive esophagoesophagostomy. At 33 months of age, the patient had a magnetic compression revision anastomosis, in which a pair of 2 cylindrical Samarium-cobalt rare-earth 320 mT (3200 G) magnets, 15 x 5 mm (diameter x thickness), were placed in the esophagus to compress the scar tissue of the anastomosis. The magnets were retrieved 34 days after the magnetic compression revision anastomosis procedure. For 3 months, the patient subsequently had balloon dilatations to prevent restenosis, and he has been able to eat normally by mouth without further balloon dilatations since then.

Development of acquired tracheal stenosis in premature infants due to prolonged endotracheal ventilation: etiological considerations and surgical management.

The aim of this study was to determine the etiology and appropriate surgical treatment for acquired tracheal stenosis that developed in patients who had undergone prolonged endotracheal mechanical ventilation as premature neonates. During the period 2000-2004, four patients aged 1-16 years were referred for tracheal stenosis characterized by stridor, choking, and recurrent pulmonary infection. All patients had undergone endotracheal mechanical ventilation for 2-5 months for respiratory distress related to prematurity (gestational age 25-29 weeks, birth weight 648-1,222 g). During this period, methicillin-resistant Staphylococcus aureus was predominantly cultured from the trachea. All patients exhibited a stenotic lesion encompassing 30-37% of the entire tracheal length on spiral CT. On palpation and inspection of the trachea during surgery, however, the stenotic segment appeared to encompass over 50% of the entire trachea. The carina was intact. Three patients underwent slide tracheoplasty with a tracheal resection and the other underwent resection and end-to-end anastomosis. Of the three patients treated by slide tracheoplasty, two are currently free of respiratory symptoms. However, one patient in this group required secondary resection of the remaining stenotic lesion with end-to-end anastomosis. This patient is currently asymptomatic. The remaining patient who underwent a resection and end-to-end anastomosis is doing well. The resected specimen showed fibrosis and degeneration of tracheal cartilage. A combination of prematurity, prolonged endotracheal mechanical ventilation and tracheal infection seem to be responsible for tracheal inflammation and stenosis. When considering surgical procedures for acquired tracheal stenosis, resection and end-to-end anastomosis are desirable. However, slide tracheoplasty with a partial tracheal resection is indicated for the treatment of stenosis involving a long tracheal segment.

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.