A case of cerebrocostomandibular syndrome with congenital heart disease

Cerebrocostomandibular syndrome is characterized by micrognathia, cleft palate, multiple thorax deformity and frequently, mental deficiency. Respiratory compromise is a common cause of death. We experienced a case of cerebrocostomandibular syndrome with congenital heart disease in a 2 day old female baby with the chief complaint of cyanosis and respiratory difficulty since birth. She was delivered by cesarian section due to delayed labor at IUP 42 weeks. The diagnosis was made on the basis of clinical features, radiologic findings and echocardiogram, which showed micrognathia, deformity of 3rd finger Lt, hemivertebrae of T1-T6 Lt., absence of 1-6th rib Lt., dextrocardia, tetralogy of fallot, atrial septal defect secondum. We reported this case and reviewed related litertures briefly.

Foreign body in the upper gastrointestinal tract in infants and children

The accidental swallowing of foreign bodies by infants and children is a common problem. The proper methods of therapy must be carefully selected according to the age of the patient, the type of forei gn body, the location of impaction, the duration of ingestion, and the available medical resources and skills. This report reviews 41 cases of ingested foreign bodies in the upper GI tract at the Department of Pediatrics, Yonsei University College of Medicine between January 1981 and June 1991. The results were as follows: 1) The ages ranged from 3 months to 13 years with 34 cases less than 5 years of age, 5 cases between 5 to 10 years of age, and 2 cases greater than 10 years of age. 2) The male to female ratio was 1.9:1 with 27 male and 14 female patients. 3) The types of ingested foreign bodies were coins in 15 cases(36.6%), pins in 9 cases(22.0%), rings in 3 cases, food in 3 cases, nails in 2 cases, and others. 4) presenting symptoms were varable with vomiting in 8 cases(19.5%), coughing in 4 cascs(9.8%), dysphagia in 4 cases(9.8%), poor oral intake in 2 cases(4.9%), diarrhea in 2 cases(4.9%), and others. 5) Ingested foreign bodies were located in the esophagus in 14 cases(34.2%), stomach in 24 cases(58.5%), duodenum in 1 cases(2.4%)and uncertain locations in 2 cases. 6) Methods for the removal of ingested foreign bodies included 15 cases of endoscopic removal(53.6%), 1 surgical removal (3.6%), and 12 spontancous removals(42.8%). 10cndoscopic removals were carried out in 12 esophageal cases(83.3%), but 10 out of 16 stomach cases were removed spontaneously (62.5%). 7) Duration from ingestion to removal of foreign bodies renged from 19 hours to 2 years. All 12 spontaneous removal cases were within 2 weeks, and 1 surgical case was performed 2 years after ingestion. 8) Endoscopic findings of patients with esophageal foreign bodies included 3 normal cases(30.0%)and 7 moderate to severe abnormal cases(70.0%), and those patients with gastric foreign bodies included 3 normal cases(60.0%)and 2 mild superficial mucosal lesion cases.