Objective: To describe the presentation and outcome of a patient with an unusual association of VATER/VACTERL syndrome (vertebra/anus/cardiac/trachea/esophogus/radius/renal/limb) and noncommunicating functioning uterine horn and a unicornuate uterus.
Design: Descriptive case report.
Setting: A foundation trust in the United Kingdom. MATERIALS AND METHOD(S): A patient known to have VATER/VACTERL syndrome was diagnosed with a hematometra of a noncommunicating uterine horn and hematosalpinx. She underwent multidisciplinary surgery to remove the functioning uterine horn, after which she had a full recovery.
Conclusion(s): This case documents the association of VATER/VACTERL syndrome with a unicornuate uterus and noncommunicating horn.
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