Management of VACTERL association with uterine anomaly: complex acute on chronic 10-year pelvic pain with haematometra

This case report presents a woman in her 20s with VACTERL association who presented with acute on chronic 10-year pelvic pain and haematometra. Initially, diagnostic imaging suspected a didelphic uterus with a dilated right horn on CT imaging. However, after physical examination and further imaging, further diagnosis of unicornuate uterus with non-communicating horn was noted. After years of expectant management, the patient began to experience severe malnutrition and metabolic derangements. After significant patient-centred discussions regarding pain relief, the patient was refractory to oral contraceptive pills and later started on GnRH antagonists. After the patient underwent an abdominal hysterectomy and right salpingectomy with resolution of pain symptoms. The case highlights the complexities of managing patients with multiple congenital anomalies, particularly VACTERL, and while not in the constellation of syndromes, gynaecological complications may arise due to common embryological origins. This case emphasises the importance of a multidisciplinary approach in challenging scenarios.

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Publication Details

Journal
BMJ Case Reports
Published
2025-05-01
DOI
https://doi.org/10.1136/bcr-2024-262730
Primary Topic
Hernia repair and management
Type
article
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article

Management of VACTERL association with uterine anomaly: complex acute on chronic 10-year pelvic pain with haematometra

Urvashi Gupta, Richard Hsu, Bria Elrod, Patrice Harold
BMJ Case Reports
Hernia repair and management
article

Management of VACTERL association with uterine anomaly: complex acute on chronic 10-year pelvic pain with haematometra

Urvashi Gupta, Richard Hsu, Bria Elrod, Patrice Harold
article en

Abstract

This case report presents a woman in her 20s with VACTERL association who presented with acute on chronic 10-year pelvic pain and haematometra. Initially, diagnostic imaging suspected a didelphic uterus with a dilated right horn on CT imaging. However, after physical examination and further imaging, further diagnosis of unicornuate uterus with non-communicating horn was noted. After years of expectant management, the patient began to experience severe malnutrition and metabolic derangements. After significant patient-centred discussions regarding pain relief, the patient was refractory to oral contraceptive pills and later started on GnRH antagonists. After the patient underwent an abdominal hysterectomy and right salpingectomy with resolution of pain symptoms. The case highlights the complexities of managing patients with multiple congenital anomalies, particularly VACTERL, and while not in the constellation of syndromes, gynaecological complications may arise due to common embryological origins. This case emphasises the importance of a multidisciplinary approach in challenging scenarios.

BMJ Case ReportsVol. 18(5)
Wayne State University (US), Detroit Medical Center (US)
Zero hunger
Openalex Percentile: Top 84%
Hernia repair and management
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Management of VACTERL association with uterine anomaly: complex acute on chronic 10-year pelvic pain with haematometra — Urvashi Gupta, Richard Hsu, et al. · BMJ Case Reports (2025) | TGRS Research Map | TGRS