Annular Pancreas in Two Consecutive Siblings: An Extremely Rare Case
| Autor: | C. Kyriazis, N. Lainakis, Theodoros Dolatzas, Antonios Panagidis, Katerina Kambouri, S. Antypas, I. Alexandrou |
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| Rok vydání: | 2005 |
| Předmět: |
Male
Pediatrics medicine.medical_specialty Inheritance Patterns Intestinal Atresia Prenatal diagnosis Anastomosis Ultrasonography Prenatal Pregnancy medicine Humans Ascending colon Pancreas business.industry Siblings Anastomosis Surgical Infant Newborn Annular pancreas medicine.disease Stenosis medicine.anatomical_structure Atresia Pediatrics Perinatology and Child Health Female Surgery Duodenal Obstruction Presentation (obstetrics) business Digestive System Abnormalities |
| Zdroj: | European Journal of Pediatric Surgery. 15:364-368 |
| ISSN: | 1439-359X 0939-7248 |
| DOI: | 10.1055/s-2005-865838 |
| Popis: | Annular pancreas is the rare congenital anomaly where the pancreas forms a full or incomplete ring around the second segment of the duodenum, causing various degrees of stenosis or atresia. It is estimated that it appears in 1 out of 12 000-15 000 births of living neonates and until now, in the literature, only 6 cases have been reported among individuals of the same family. We present the case of two siblings, a boy and a girl, with annular pancreas from consecutive pregnancies of the same couple. Both neonates had a prenatal diagnosis of duodenal obstruction and they underwent duodenoduodenal, proximal transverse to distal longitudinal anastomosis. Furthermore, the girl had a mobile ascending colon. Their postoperative condition was perfect. The case we are reporting is an addition to the other 6 cases of familial presentation of annular pancreas and is similar to one of them. In these families, a total of 16 persons present this congenital anomaly while 14 are seemingly healthy. Twelve of the affected persons are female and 4 male. In conclusion, it can be stated that female individuals seem to have a greater propensity to transmit the disease to their descendants, compared to males, suggesting the possible action of an autosomal recessive sex-influenced gene. The recording of such rare family cases should be encouraged, in order to fully recognize a possible type of inherited transmission. |
| Databáze: | OpenAIRE |
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