Complete Labial Fusion Causing Urinary Retention and Pyo-Hematocolpos in a Female With Turner Syndrome

TL;DR

A woman with Turner syndrome developed severe labial fusion leading to life-threatening complications, which were successfully treated with surgery and antibiotics.

Contribution

This case highlights the rare but severe complications of labial fusion in Turner syndrome and emphasizes the importance of early intervention.

Findings

Complete labial fusion in Turner syndrome can cause urinary retention, sepsis, and pyo-hematocolpos.

Emergency surgical separation and estrogen therapy effectively resolved the complications in this case.

Early recognition and treatment are crucial to prevent life-threatening outcomes in Turner syndrome patients.

Abstract

Turner syndrome, the most common chromosomal disorder in females, is associated with hypoestrogenism, which predisposes patients to genital tract atrophy and complications such as labial fusion. Although often asymptomatic, complete fusion may cause urinary obstruction, infection, and, in rare cases, life-threatening sepsis. We report a case of a 58-year-old female with Turner syndrome who presented with urinary retention, fever, and systemic signs of sepsis. Examination revealed complete labial fusion, obscuring the vaginal introitus, with imaging confirming hydro-pyo-hematocolpos. Laboratory findings were consistent with systemic inflammation and impaired renal function. Emergency surgical separation of the fused labia led to the drainage of purulent and hemorrhagic fluid, restoration of normal genital anatomy, and insertion of a Foley catheter. The patient was managed with broad-spectrum antibiotics, inotropes, intravenous fluids, and local estrogen therapy, achieving full recovery and discharge after 14 days.

Labial fusion in Turner syndrome results from chronic hypoestrogenism, recurrent inflammation, and mechanical irritation. While typically underdiagnosed, advanced cases can cause urinary retention, ascending infections, and pyocolpos. Surgical separation is an effective treatment modality, particularly in severe cases complicated by obstruction or infection, with postoperative estrogen therapy minimizing recurrence. This report highlights the need for heightened clinical vigilance for labial fusion in Turner syndrome patients. Early recognition and timely surgical intervention are essential to prevent severe complications, including hemato-pyocolpos and sepsis, and to improve quality of life.

Figures