Herlyn-Werner-Wunderlinch: An unusual presentation in a patient with Prader-Willi syndrome.
Endocrinol Diabetes Nutr (Engl Ed)
; 71(4): 171-176, 2024 Apr.
Article
em En
| MEDLINE
| ID: mdl-38735678
ABSTRACT
Herlyn-Werner-Wunderlich syndrome is an uncommon urogenital anomaly defined by uterus didelphys, obstructed hemi-vagina and unilateral renal anomalies. The most common clinical presentation is dysmenorrhoea following menarche, but it can also present as pain and an abdominal mass. Prader-Willi syndrome is a rare neuroendocrine genetic syndrome. Hypothalamic dysfunction is common and pituitary hormone deficiencies including hypogonadism are prevalent. We report the case of a 33-year-old female with Prader-Willi syndrome who was referred to the Gynaecology clinic due to vaginal bleeding and abdominal pain. Abdominal ultrasound revealed a haematometra and haematocolpos and computed tomography showed a uterus malformation and a right uterine cavity occupation (hematometra) as well as right kidney agenesis. Vaginoscopy and hysteroscopy were performed under general anaesthesia, finding a right bulging vaginal septum and a normal left cervix and hemiuterus. Septotomy was performed with complete haematometrocolpos drainage. The association of the two syndromes remains unclear.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Síndrome de Prader-Willi
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Útero
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Vagina
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Rim
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Nefropatias
Limite:
Adult
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Female
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Humans
Idioma:
En
Revista:
Endocrinol Diabetes Nutr (Engl Ed)
Ano de publicação:
2024
Tipo de documento:
Article