Van Wyk-Grumbach Syndrome: A Rare Triad of Precocious Puberty, Pituitary Pseudomacroadenoma, and Multicystic Ovaries in an Eight-Year-Old Girl

Van Wyk-Grumbach syndrome (VWGS) is a rare manifestation of chronic, severe primary hypothyroidism in children.It is classically characterized by delayed bone age, short stature, and isosexual precocious puberty, often accompanied by multicystic ovaries and pituitary hyperplasia.Because of its presentation, it is frequently misdiagnosed as an ovarian or pituitary neoplasm, leading to unnecessary surgical interventions.We report the case of an eight-year-old female child who presented with a three-day history of per vaginal bleeding.Physical examination revealed severe disproportionate short stature.Imaging initially suggested neoplastic etiologies, revealing bilateral complex cystic ovarian lesions on abdominal magnetic resonance imaging (MRI) and a sellar/suprasellar mass on brain MRI suggestive of a pituitary macroadenoma.However, a severely delayed bone age (3.5-4 years) and a thyroid profile confirming autoimmune primary hypothyroidism led to the diagnosis of VWGS.The patient was managed purely medically with cautious levothyroxine replacement therapy.This case highlights the critical importance of recognizing VWGS to avoid devastating and unnecessary ovarian or pituitary surgeries.

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Publication Details

Journal
Cureus
Published
2026-09-15
DOI
https://doi.org/10.7759/cureus.116300
Primary Topic
Hypertrophic osteoarthropathy and related conditions
Type
article
Field-Weighted Citation Impact
0.00
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article

Van Wyk-Grumbach Syndrome: A Rare Triad of Precocious Puberty, Pituitary Pseudomacroadenoma, and Multicystic Ovaries in an Eight-Year-Old Girl

Prashant Bhosale, Rubanbalaji G., Ripdaman Kaur, Anita Meena et al.
Cureus
Hypertrophic osteoarthropathy and related conditions
article

Van Wyk-Grumbach Syndrome: A Rare Triad of Precocious Puberty, Pituitary Pseudomacroadenoma, and Multicystic Ovaries in an Eight-Year-Old Girl

Prashant Bhosale, Rubanbalaji G., Ripdaman Kaur, Anita Meena, Yogendra N Maurya
article en

Abstract

Van Wyk-Grumbach syndrome (VWGS) is a rare manifestation of chronic, severe primary hypothyroidism in children.It is classically characterized by delayed bone age, short stature, and isosexual precocious puberty, often accompanied by multicystic ovaries and pituitary hyperplasia.Because of its presentation, it is frequently misdiagnosed as an ovarian or pituitary neoplasm, leading to unnecessary surgical interventions.We report the case of an eight-year-old female child who presented with a three-day history of per vaginal bleeding.Physical examination revealed severe disproportionate short stature.Imaging initially suggested neoplastic etiologies, revealing bilateral complex cystic ovarian lesions on abdominal magnetic resonance imaging (MRI) and a sellar/suprasellar mass on brain MRI suggestive of a pituitary macroadenoma.However, a severely delayed bone age (3.5-4 years) and a thyroid profile confirming autoimmune primary hypothyroidism led to the diagnosis of VWGS.The patient was managed purely medically with cautious levothyroxine replacement therapy.This case highlights the critical importance of recognizing VWGS to avoid devastating and unnecessary ovarian or pituitary surgeries.

Cureus
Indraprastha Apollo Hospitals (IN)
Gender equality, Zero hunger
Openalex Percentile: Top 10%
Hypertrophic osteoarthropathy and related conditions
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