Management and Obtaining Informed Consent Complicated by Intellectual Disability in a Patient With Mrkh Syndrome - a Case Report
Year: 2026
Type: article
Study design: Case Report
Abstract: Introduction The coexistence of Mayer Rokitansky Küster Hauser syndrome and fetal alcohol syndrome in a minor patient with intellectual disability represents a rare clinical and ethical legal situation. Particular complexity arises from the simultaneous presence of two aspects, the clinical justification for surgical intervention in the presence of functioning endometrium within rudimentary uterine structures, and the process of obtaining informed consent taking into account the patient’s cognitive limitations and the involvement of a legal representative. According to a search of the PubMed and Google Scholar databases using keywords related to MRKH, fetal alcohol syndrome, intellectual disability, and informed consent, no publications addressing these aspects jointly were identified. Aim To analyze a clinical case of Mayer Rokitansky Küster Hauser syndrome in a patient with fetal alcohol syndrome, with a focus on the justification of surgical management and the specifics of obtaining informed consent. Methods A clinical case of a 17-year-old patient with fetal alcohol syndrome, attention deficit hyperactivity disorder, and intellectual disability, evaluated for primary amenorrhea and cyclical lower abdominal pain, is presented. The diagnostic process was based on clinical assessment and imaging methods with karyotype confirmation. Decision making regarding treatment was accompanied by adaptation of communication, assessment of the patient’s decision-making capacity, and the involvement of a legal representative. Results Two non-communicating rudimentary uterine horns with signs of functioning endometrium were identified, which caused cyclical pelvic pain and constituted an indication for surgical treatment. Informed consent was obtained from both the patient and her foster mother following step by step explanation of the diagnosis and treatment options. Laparoscopic removal of the rudimentary uterine structures with bilateral salpingectomy was performed. Postoperatively, resolution of pain and improvement in quality of life were observed. Conclusions The presented clinical case demonstrates the need for coordinated consideration of clinical indications for surgical treatment in MRKH with functioning rudimentary structures and assessment of a minor patient’s capacity to provide informed consent with the involvement of a legal representative. The case illustrates the clinical rationale for intervention and the decision-making process in the context of intellectual disability.
Source: Archiv Euromedica
Institution: Medical University of Lublin
Language: English
Field: Medicine
Domain: Health Sciences
Open Access status: bronze
OpenAlex ID: W7160093923