Unilateral ovarian agenesis and fallopian tube maldescent

Abstract

Unilateral ovarian agenesis (UOA) and fallopian descent problems are very rare congenital defects. We present an unusual case of UOA associated with fallopian-tube maldescent discovered incidentally during a laparotomy in a female infant for a persistent heterogeneous right ovarian cyst refractory to needle aspiration. A necrotic, hemorrhagic paratubal cyst was found associated with a normal right adnexa. The left ovary was absent and a rudimentary left fallopian tube was found tightly stretched over the sigmoid colon to the left retroperitoneum. This previously unreported constellation of anomalies may carry significant potential risks for bowel obstruction and later fertility.

Four Cases with Congenital Unilateral Absence of Ovary and Fallopian Tube: Review of the Literature

“…Torsion most likely manifests itself with acute abdominal pain, nausea, and vomiting, but it can also be asymptomatic and therefore diagnosed only in operations performed for other reasons. In the cases reported by Sivanesaratnam [1], Dueck et al [23] and Guvenc et al [24], they were also asymptomatic, suggesting that the event had occurred during the fetal stage. Laparoscopy provides a definitive diagnosis of unilateral ovarian and/or tubal absence.…”

Ovarian maldescent

“…As in case 1, all the reported cases presented an elongated fallopian tube whose fimbriated end had a normal relationship with the undescended ovary [2,[4][5][6][7]. This fact suggests that a migration disorder in the müllerian duct is associated with the undescended ovary and that congenital uterine anomalies are not only due to an abnormal fusion of the müllerian ducts, but we can hypothesize that an abnormal migration could affect their normal fusion [4].…”

Conservative Management of a Large Neonatal Ovarian Cyst: A Case Report

“…During maturation of gonads, the ovary descends in the neonatal pelvis. The position of the NOC is usually high in the abdomen attached to the pelvis by a thin stalk, resembling the pattern of ovarian development suggesting that these cyst are actually dysgenic gonads which are still in their initial position and have not descended to the pelvis [3]. Another theory suggest role of hormonal imbalances in utero for cyst formation, related to excess gonoadotropin, placental hCG or placental oestrogen release [4].…”