In 1978, your journal published the first report of dominant inheritance of annular pancreas [Jackson and Apostolides, 19781. To our knowledge, no other reports have appeared subsequently. We now provide brief details of a mother and son, both of whom presented in the neonatal period with gastrointestinal obstruction. The mother, the third child of healthy unrelated parents, 41 and 38 years old, underwent laparotomy at age 3 days. The descending duodenum was noted to "disappear," and the surgeon who performed the operation attributed this to an annular pancreas. No other abnormalities were noted. A duodenojejunostomy was performed and the infant made a full and uneventful recovery.
This woman and her healthy unrelated husband recently had their first child, a boy, who also underwent laparotomy at age 3 days. The descending duodenum was found to be stenotic in association with an annular pancreas. No other abnormality was noted. A duodenoduodenostomy was performed, and the baby recovered rapidly.
On recent examination at age 6 months, he was entirely normal.
This is the third familial report of annual pancreas. Montgomery et a1 I19711 described an affected sib pair, but, since annular pancreas may be silent or present only in adult life [Kiernan et al, 19801, it would be unwise to assume that affected sibs necessarily represent recessive inheritance. The family of Jackson and Apostolides [ 19781 included an affected mother, son, and two daughters. Our family consists of an affected mother and son. Thus, as yet, no male-to-male transmission has been documented, but clearly dominant inheritance, either autosomal or sex-linked, may account for at least a small proportion of cases of annular pancreas, a point worthy of note when assessing patients with this condition at the genetics clinic.