Imaging in Van Wyk Grumbach syndrome: An uncommon presentation of hypothyroidism

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Abstract

Isosexual precocious puberty with ovarian masses in long-standing juvenile hypothyroidism is well described in the literature as Van Wyk Grumbach syndrome (VWGS). The present case reports this rare entity in a 4-year-old girl who was referred for imaging to evaluate the cause of non-traumatic bleeding per vagina. Antecedent history, clinical features and thyroid function tests were consistent with long-standing juvenile hypothyroidism with documented clinical response to thyroxine replacement therapy.

Contribution

Typical clinical and radiological features of the syndrome are reported, which helps in the early diagnosis and management, henceforth avoiding the associated complications.

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Most cited references 16

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Congenital hypothyroidism

Maynika V Rastogi, Stephen H LaFranchi (2010)

Congenital hypothyroidism (CH) occurs in approximately 1:2,000 to 1:4,000 newborns. The clinical manifestations are often subtle or not present at birth. This likely is due to trans-placental passage of some maternal thyroid hormone, while many infants have some thyroid production of their own. Common symptoms include decreased activity and increased sleep, feeding difficulty, constipation, and prolonged jaundice. On examination, common signs include myxedematous facies, large fontanels, macroglossia, a distended abdomen with umbilical hernia, and hypotonia. CH is classified into permanent and transient forms, which in turn can be divided into primary, secondary, or peripheral etiologies. Thyroid dysgenesis accounts for 85% of permanent, primary CH, while inborn errors of thyroid hormone biosynthesis (dyshormonogeneses) account for 10-15% of cases. Secondary or central CH may occur with isolated TSH deficiency, but more commonly it is associated with congenital hypopitiutarism. Transient CH most commonly occurs in preterm infants born in areas of endemic iodine deficiency. In countries with newborn screening programs in place, infants with CH are diagnosed after detection by screening tests. The diagnosis should be confirmed by finding an elevated serum TSH and low T4 or free T4 level. Other diagnostic tests, such as thyroid radionuclide uptake and scan, thyroid sonography, or serum thyroglobulin determination may help pinpoint the underlying etiology, although treatment may be started without these tests. Levothyroxine is the treatment of choice; the recommended starting dose is 10 to 15 mcg/kg/day. The immediate goals of treatment are to rapidly raise the serum T4 above 130 nmol/L (10 ug/dL) and normalize serum TSH levels. Frequent laboratory monitoring in infancy is essential to ensure optimal neurocognitive outcome. Serum TSH and free T4 should be measured every 1-2 months in the first 6 months of life and every 3-4 months thereafter. In general, the prognosis of infants detected by screening and started on treatment early is excellent, with IQs similar to sibling or classmate controls. Studies show that a lower neurocognitive outcome may occur in those infants started at a later age (> 30 days of age), on lower l-thyroxine doses than currently recommended, and in those infants with more severe hypothyroidism.

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Syndrome of precocious menstruation and galactorrhea in juvenile hypothyroidism: an example of hormonal overlap in pituitary feedback

Judson J. Van Wyk, Melvin M. Grumbach (1960)

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Thyroid hormone acts directly on growth plate chondrocytes to promote hypertrophic differentiation and inhibit clonal expansion and cell proliferation.

H. Robson MacDonald, Richard D. Stevens, Bryan Williams … (2000)

T3 is an important regulator of endochondral bone formation in epiphyseal growth plates. Growth arrest in juvenile hypothyroidism results from disorganization of growth plate chondrocytes and their failure to undergo hypertrophic differentiation, but it is unclear how T3 acts directly on chondrocytes or whether its actions involve other pathways. To address this issue, we investigated whether thyroid hormone receptors (TR) were localized to discrete regions of the unfused epiphysis by immunohistochemistry performed in tibial growth plates from 21-day-old rats and examined the effects of T3 on growth plate chondrocytes in agarose suspension cultures in vitro. TRalpha1, -alpha2, and -beta1 were expressed in reserve and proliferating zone chondrocytes, but not in hypertrophic cells, suggesting that progenitor cells and immature chondrocytes are the major T3 target cells in the growth plate. Chondrocytes in suspension culture expressed TRalpha1, -alpha2, and -beta1 messenger RNAs and matured by an ordered process of clonal expansion, colony formation, and terminal hypertrophic differentiation. Clonal expansion and proliferation of chondrocytes were inhibited by T3, which also induced alkaline phosphatase activity, expression of collagen X messenger RNA, and secretion of an alcian blue-positive matrix as early as 7 days after hormone stimulation. Thus, T3 inhibited chondrocyte clonal expansion and cell proliferation while simultaneously promoting hypertrophic chondrocyte differentiation. These data indicate that thyroid hormones concurrently and reciprocally regulate chondrocyte cell growth and differentiation in the endochondral growth plate.

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Author and article information

Journal

Journal ID (nlm-ta): SA J Radiol

Journal ID (iso-abbrev): SA J Radiol

Journal ID (publisher-id): SAJR

Title: SA Journal of Radiology

Publisher: AOSIS

ISSN (Print): 1027-202X

ISSN (Electronic): 2078-6778

Publication date (Electronic): 15 March 2023

Publication date Collection: 2023

Volume: 27

Issue: 1

Electronic Location Identifier: 2572

Affiliations

[1 ]Department of Radiodiagnosis, All India Institute of Medical Sciences Rishikesh, Rishikesh, India

[2 ]Department of Paediatrics, All India Institute of Medical Sciences Rishikesh, Rishikesh, India

Author notes

Corresponding author: Poonam Sherwani, sherwanipoonam@ 123456gmail.com

Author information

Poonam Sherwani https://orcid.org/0000-0002-6816-5018

Khanak K. Nandolia https://orcid.org/0000-0002-8627-2992

Kirti Joshi http://orcid.org/0000-0003-2403-0186

Radhapyari Lourembam https://orcid.org/0000-0001-7803-3118

Article

Publisher ID: SAJR-27-2572

DOI: 10.4102/sajr.v27i1.2572

PMC ID: 10091148

SO-VID: 341aac16-3ffa-485a-95f4-74c0734c7d1a

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Licensee: AOSIS. This work is licensed under the Creative Commons Attribution License.

Imaging in Van Wyk Grumbach syndrome: An uncommon presentation of hypothyroidism

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Most cited references 16

Congenital hypothyroidism

Syndrome of precocious menstruation and galactorrhea in juvenile hypothyroidism: an example of hormonal overlap in pituitary feedback

Thyroid hormone acts directly on growth plate chondrocytes to promote hypertrophic differentiation and inhibit clonal expansion and cell proliferation.

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