Sivasubramanian Dhiran, Senthilkumar Virushnee, Aravind Smrti, Rajasekar Aswin Ram, Prasaanth Sharan, Sanil Sathwik
Department of Critical Care Medicine, Christian Medical College, Vellore, India.
Coimbatore Medical College and Hospital, Coimbatore, India.
Int J Surg Case Rep. 2025 Feb;127:110975. doi: 10.1016/j.ijscr.2025.110975. Epub 2025 Jan 28.
Van Wyk-Grumbach Syndrome (VWGS) is a rare pediatric endocrinological disorder characterized by hypothyroidism, delayed bone age, enlarged multicystic ovaries, and precocious puberty. It results from prolonged hypothyroidism, affecting the hypothalamic-pituitary-gonadal axis. This report describes a 7-year-old girl presenting with vaginal bleeding and abdominal pain, leading to a diagnosis of VWGS with bilateral inguinal hernia and requiring surgical intervention.
A 7-year-old girl presented with a single episode of vaginal bleeding, abdominal pain, and growth retardation. Clinical examination revealed delayed growth parameters, Tanner stage II breast development, and bilateral inguinal hernias. Abdominal examination identified a cystic mass in the right iliac fossa. Laboratory tests showed hypothyroidism. Imaging revealed a multiloculated right ovarian cyst. The patient underwent exploratory laparotomy with right salpingo-oophorectomy, marsupialization of the left ovarian cyst, and bilateral hernia repair. Histopathology confirmed ovarian hemorrhagic infarction. Postoperatively, she was started on levothyroxine therapy, leading to symptom resolution, height improvement, and cyst regression during follow-up.
VWGS manifests due to thyroid dysfunction-induced gonadal stimulation, causing ovarian enlargement and precocious puberty. Prompt initiation of levothyroxine can prevent complications and avoid surgical intervention. The bilateral hernias in this case represent a unique presentation, potentially linked to hypothyroidism-induced muscle weakness.
This case underscores the necessity of routine thyroid evaluation in pediatric patients with ovarian cysts and precocious puberty. Early diagnosis and levothyroxine therapy can resolve symptoms and prevent invasive interventions, emphasizing the critical role of endocrinological assessment.
范·怀克 - 格伦巴赫综合征（VWGS）是一种罕见的儿科内分泌疾病，其特征为甲状腺功能减退、骨龄延迟、多囊卵巢增大和性早熟。它由长期甲状腺功能减退引起，影响下丘脑 - 垂体 - 性腺轴。本报告描述了一名7岁女孩，出现阴道出血和腹痛，诊断为VWGS并伴有双侧腹股沟疝，需要手术干预。
一名7岁女孩出现单次阴道出血、腹痛和生长发育迟缓。临床检查发现生长参数延迟、坦纳II期乳房发育和双侧腹股沟疝。腹部检查在右髂窝发现一个囊性肿块。实验室检查显示甲状腺功能减退。影像学检查发现右侧卵巢有一个多房性囊肿。患者接受了剖腹探查术，切除右侧输卵管卵巢，对左侧卵巢囊肿进行袋形缝合，并进行双侧疝修补。组织病理学证实为卵巢出血性梗死。术后，她开始接受左甲状腺素治疗，随访期间症状缓解、身高增加且囊肿消退。
VWGS由于甲状腺功能障碍引起性腺刺激而表现出来，导致卵巢增大和性早熟。及时开始使用左甲状腺素可以预防并发症并避免手术干预。该病例中的双侧疝是一种独特的表现，可能与甲状腺功能减退引起的肌肉无力有关。
本病例强调了对患有卵巢囊肿和性早熟的儿科患者进行常规甲状腺评估的必要性。早期诊断和左甲状腺素治疗可以缓解症状并防止侵入性干预，强调了内分泌评估的关键作用。
