Surgical Management of an Obstructive Müllerian Anomaly in a Patient with VACTERL Association: A Case Report

Lavoie C, Melanie Au, Syed H, Christine Do, Baker Z and Vasquez E
 

Division of Urology, Children’s Hospital Los Angeles, CA, USA
California University of Science and Medicine, CA, USA
Arizona State University, Knowledge Enterprise, Office of the Vice President of Research, USA
Keck School of Medicine, University of Southern California, CA, USA

^*Correspondance to: Evalynn Vasquez 

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Abstract:

Background: The incidence of VACTERL association alone has been estimated at around 1 in 10,000 to 1 in 40,000 live born infants. Upon review of existing literature, few if any other cases of VACTERL association in combination with vaginal atresia, uterine anomalies, and cervical agenesis in a patient of pubescent age have been discussed despite a likely association between VACTERL and vaginal anomalies. Case Report: A 10-year-old female with a history of VACTERL association initially presented for management of nephrolithiasis and pyelonephritis. Following successful treatment, she represented six months later with recurrent cyclical abdominal pain. Workup demonstrated proximal vaginal atresia with right hematometrocolpos, hematosalpinx, and uterine didelphys. A complex robotic assisted vaginoplasty was successfully performed. Two years later, the patient re-presented due to recurrent cyclical monthly pelvic pain, nausea, and vomiting and was found to have a left hematometra, hematosalpinx, and ovarian cysts due to cervical agenesis of the left hemi-uterus. Patient underwent left laparoscopic hysterectomy, drainage of the hematosalpinx, and left ovarian cystectomy which led to symptom resolution. Conclusion: This is a rare case of VACTERL association which presented in combination with distal vaginal atresia, right hematometrocolpos, hematosalpinx, and uterine didelphys, later complicated by left hematometra, hematosalpinx, and ovarian cysts secondary to agenesis of the left hemi-uterus demonstrates an exceedingly complex case of interdisciplinary medical and surgical management. The array of symptoms which were present in a single patient and the numerous surgical interventions which were utilized to improve the patient’s quality of life and symptomatology represent a unique case of medical presentation and management.

Keywords:

Mullerian Anomaly; Uterine didelphys; Vaginal atresia; VACTERL

Cite the Article:

Lavoie C, Melanie Au, Syed H, Christine Do, Baker Z, Vasquez E. Surgical Management of an Obstructive Müllerian Anomaly in a Patient with VACTERL Association: A Case Report. Clin Case Rep Int. 2024; 8: 1668.