Epignathus is an extremely rare congenital oropharyngeal teratoma. Here, we report a case of epignathus without intracranial extension in a fetus. The mass was first found by ultrasonography at 22 gestational weeks. Serial ultrasound examinations and magnetic resonance imaging confirmed that the fetus had neither central nervous system involvement nor abnormal nose or tooth. The baby was delivered at 37 weeks and six days of gestation via cesarean section set up for ex-utero intrapartum treatment. The postnatal pathologic examination confirmed the presence of mature tissues predominantly containing ectopic central nervous tissue, osseous tissue, and bronchial mucosal tissue. Most cases of epignathus are associated with malformation and death. Ultrasound and magnetic resonance imaging prenatal assessments are very important to facilitate counseling and understand prognosis. In conclusion, the ex-utero intrapartum treatment procedure is a good approach to improve the survival of infants with epignathus.

Epignathus is an extremely rare congenital oropharyngeal teratoma. Here, we report a case of epignathus without intracranial extension in a fetus. The mass was first found by ultrasonography at 22 gestational weeks. Serial ultrasound examinations and magnetic resonance imaging confirmed that the fetus had neither central nervous system involvement nor abnormal nose or tooth. The baby was delivered at 37 weeks and six days of gestation via cesarean section set up for ex-utero intrapartum treatment. The postnatal pathologic examination confirmed the presence of mature tissues predominantly containing ectopic central nervous tissue, osseous tissue, and bronchial mucosal tissue. Most cases of epignathus are associated with malformation and death. Ultrasound and magnetic resonance imaging prenatal assessments are very important to facilitate counseling and understand prognosis. In conclusion, the ex-utero intrapartum treatment procedure is a good approach to improve the survival of infants with epignathus.

Objective:To explore the application of home nutrition support in children with intestinal failure.Methods:Children with intestinal failure admitted to Division of Pediatric Gastroenterology and Nutrition and Department of Pediatric Surgery in Xinhua Hospital were retrospectively enrolled since January 2009. The details of home nutrition support, nutritional status and home parenteral nutrition (HPN) associated complications were collected.Results:A total of 10 children received HPN support, 7 of whom were with short bowel syndrome (SBS) and the other 3 with pediatric intestinal pseudo-obstruction. The average length of remnant small bowel in 7 SBS children was (36.7±32.4) cm. The average age at HPN onset was (5.4±4.7) years. The average duration of follow-up was (3.1±2.1) years. The average duration of HPN was (619.5±669.1) days after (391.8±340.1) days of parenteral nutrition support in our hospital. All 10 cases started home enteral nutrition (HEN) with tube feeding (3 cases transited to oral feeding during treatment). The average duration of HEN was (536.1±429.6) days. Daily calorie intake was 104.0%±39.0% of the recommended intake according to the guideline, with 46.5%±21.3% via HPN and 57.5%±29.2% via HEN. During follow-up, 3 cases were found with severe malnutrition, 5 with moderate malnutrition and 2 with mild malnutrition. Four children suffered from catheter-related thrombosis and five children were identified with catheter-related blood stream infection. No intestinal failure associated liver disease was observed.Conclusions:HPN is feasible but needs the support of national medical insurance policy. At present, there are still frequent nutritional deficiencies and complications in HPN. Nutrition support team (NST) should provide guidance for more scientific nutrition screening and nutrition management.