title: Annular pancreas in two consecutive siblings: an extremely rare case.
abstract: 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.
authors.affiliation: 1st Pediatric Surgery Department, Aghia Sophia Children's Hospital, Athens, Greece.
authors.forename: N
authors.initials: N
authors.lastname: Lainakis
authors.forename: S
authors.initials: S
authors.lastname: Antypas
authors.forename: A
authors.initials: A
authors.lastname: Panagidis
authors.forename: I
authors.initials: I
authors.lastname: Alexandrou
authors.forename: K
authors.initials: K
authors.lastname: Kambouri
authors.forename: C
authors.initials: C
authors.lastname: Kyriazis
authors.forename: T
authors.initials: T
authors.lastname: Dolatzas
mesh_terms: D000714:Anastomosis, Surgical; D004065:Digestive System Abnormalities / Q000235:genetics; D004380:Duodenal Obstruction / Q000151:congenital* / Q000000981:diagnostic imaging / Q000601:surgery; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D040582:Inheritance Patterns; D007409:Intestinal Atresia / Q000601:surgery; D008297:Male; D010179:Pancreas / Q000002:abnormalities*; D011247:Pregnancy; D035781:Siblings; D016216:Ultrasonography, Prenatal