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Purpose Lingual thyroglossal duct cysts (L-TGDCs) are rare and sometimes lethal owing to their association with asphyxia. We aimed to analyze our single institutional experience with L-TGDCs. Methods Twelve L-TGDC cases treated at our institution between January 2010 and December 2017 were investigated. Results The male/female ratio was 6/6. The age at the diagnosis was 2 ± 1.4 months (7 days to 6 months), and 3 patients were diagnosed in the neonatal period. The patients presented with stridor (n = 12; 100%), growth retardation (n = 5; 42%), apnea (n = 3; 25%), and vomiting (n = 1; 8.3%). Lateral X-rays were obtained in 8 cases (66.7%); a lingual mass was suspected in 7 (87.5%). Transoral marsupialization of the cyst was performed under direct vision in all cases. All cases were nasally and orally intubated using a laryngoscope, bronchoscope, or airway scope. The mean operative time was 18 ± 2.9 min. The mean cyst size was 10.5 ± 1.8 mm. No recurrence was observed during the follow-up period (37.5 ± 18 months). Conclusion L-TGDC requires a precise diagnosis and rapid intervention because of the risk of asphyxia resulting in sudden death. Transoral marsupialization under direct vision is an effective and secure approach. L-TGDC should be considered when patients younger than six months of age present with respiratory distress. Type of study Retrospective Study. Level of evidence Level IV.

We herein demonstrate a case of a gastric-type duplication cyst of the pancreas with a bifid tail in a 1-year-old girl, who presented as relapsing pancreatitis with pseudocyst formation. We reviewed the literature concerning pancreatic enteric duplication cysts with and without pancreatitis to search for causes of pancreatitis. The following new information was obtained: 1) The presence of a communication between the pancreatic duct system and enteric duplication cysts is the major cause, but it does not always lead to pancreatitis. 2) Enteric duplication cysts are occasionally connected to abnormal pancreatic lobes or ducts. We suggest that a duplication cyst associated with bifid pancreas is in the same category as the association of enteric cyst formation and pancreatic lobe maldevelopment. Since an aberrant lobe associated with an enteric duplication cyst often evokes pancreatitis, its surgical resection should be considered.

We report a rare case of coexisting pulmonary artery sling, congenital tracheal stenosis, and dextrocardia caused by right lung hypoplasia. Successful treatment of severe postoperative tracheomalacia was achieved by aortopexy, aiming displacement of the aortic arch across orthogonally in front of the trachea due to dextrocardia. The aim of this surgery was different from the usual aortopexy for tracheomalacia, which lifts the tracheal wall with the aorta. The three-dimensional evaluation considering the patient's associated malformations led to a successful result.

Background/Purpose: The incidence of gastroschisis has increased over the past 3 decades in a number of countries. To elucidate the Japanese status of anterior abdominal wall defects, the Japanese Society of Pediatric Surgeons conducted a national survey in Japan. Methods: Information was obtained by sending out a questionnaire to 192 University Hospitals, Children's hospitals, and general hospitals that each had more than 200 beds. The characteristics of the patients including the birth date, birth weight, gestations, rate of associated anomalies, rate of antenatal diagnosis and prognosis, maternal age, gravidity, history of smoking, and drug use were analyzed. Results: The authors obtained answers from 149 institutions, including 1,785 cases of omphalocele and 970 cases of gastroschisis, which were treated between 1975 to 1997. There was a significant increase in the incidence of gastroschisis, from 0.131 in 1975 to 1980, 0.269 in 1981 to 1985, 0.337 in 1986 to 1990, 0.461 in 1991 to 1995 to 0.467 per 10,000 births in 1996 to 1997. The incidence of omphalocele was 0.322 in 1975 to 1980, 0.567 in 1981 to 1985, 0.657 in 1986 to 1990, 0.741 in 1991 to 1995 to 0.626 per 10,000 births in 1996 to 1997, respectively. In the omphalocele group, 43.1% of the mothers were between 25 to 29 years of age, whereas in the gastroschisis group 42.6% of the mothers were 20 to 24 years of age. In the gastroschisis group, the number of primipara mothers was larger than that of multipara mothers. In the omphalocele group, approximately 10% of the mothers smoked during each period, whereas in the gastroschisis group, the percentage of smoking mothers increased chronologically from 12.9% in 1981 to 1985, 18.7% in 1986 to 1990, 23.5% in 1991 to 1995 and 29.3% in 1996 to 1997. A history of drug use by the mother was approximately 10% for both the omphalocele and gastroschisis groups. In the omphalocele group, 55.9% had associated anomalies against 21.8% in the gastroschisis group. Approximately 10% in the omphalocele group and less than 3% in the gastroschisis group showed chromosomal abnormalities. From 1986, a significant number of cases detected antenatally has been observed. Conclusions: There have been substantial changes in the incidence of anterior abdominal wall defects, particularly regarding gastroschisis in Japan. The reasons for such changes are most likely multifactorial, further epidemiological monitoring is thus called for. J Pediatr Surg 35:66-72. Copyright © 2000 by W.B. Saunders Company.

Although the etiology of Hirschsprung's disease and neuronal intestinal dysplasia remains obscure, both have histological abnormalities involving ganglion cells and neuronal elements. Searching for a common pathway that may inhibit normal maturation of neurogenic precursors, we examined the possible role of an immune mechanism in the maldevelopment of the enteric neural network. Six patients with Hirschsprung's disease were studied by comparing biopsy specimens from diseased colon with ones taken from proximal ganglionic colon in the same patients. These were similarly compared with colonic biopsy specimens from patients studied with chronic constipation or bowel removed at the time of operation for other disorders. Biopsies were taken from four other patients with neuronal intestinal dysplasia. Each was examined by hematoxylin & eosin staining, acetylcholinesterase histochemistry, and immuno-histochemistry of major histocompatibility complex (MHC) class I and class II antigens. All rectal samples from Hirschsprung's disease patients exhibited elevated acetylcholinesterase histochemistry and absent ganglia to confirm the diagnosis. These findings were correlated with marked elevation of class II MHC in the aganglionic area, whereas the proximal normal ganglionic segments showed no elevation. Rectal biopsy specimens from patients with chronic constipation exhibited no such elevation. A similar elevation of class II MHC was detected in the mucosa and submucosa of all four patients with the rare neuronal intestinal dysplasia disorder whose diagnosis was confirmed by giant ganglia in Auerbach's plexuses, aberrant Meissner's ganglia in the lamina propria mucosa, and giant neurofibrils in the mucosa and submucosa. The correlation of elevated class II MHC in these two neuronal dysfunction disorders may indicate an underlying autoimmune mechanism as is seen in thyroiditis and insulin dependent diabetes mellitus. These observations confirm those found in a small number of Hirschsprung's disease patients reported earlier, and extend the observation to other neuronal dysfunction disorders. These findings provoke a reexamination of the etiology of Hirschsprung's disease and may predict alternate therapies.