Presentation
Abdominal pain. Signs of precocious puberty.
Patient Data
The ultrasound images of the pelvis demonstrate the signs of precocious puberty:
- enlarged uterus, the thickness of the corporeal region is higher than that of the cervical region on sagittal images
- both ovaries are enlarged (right ovary = 4.1 x 2.2 x 1.9 cm, volume = 8.6 cm3), and (left ovary = 6.1 x 5.2 x 3.3 cm, volume = 52.3 cm3), containing numerous cysts of various sizes up to 1.8 cm on the right and 5.2 cm on the left.
The thyroid gland is small in size (right lobe = 3.93 x 1.57 x 1.21 cm, volume = 3.92 ml), (left lobe = 3.28 x 1.14 x 0.94 cm, volume = 1.84 ml) with inhomogeneous hypoechoic echotexture. The power Doppler shows increased internal vascularity.
According to Greulich and Pyle technique the bone age is around 6 years ( while the chronological age was exactly 7 years 11 months).
The pituitary gland is enlarged with suprasellar extension filling the suprasellar cistern with a convex shape of the sellar diaphragm. It displays a homogeneous signal and enhancement.
Case Discussion
The patient's chronological age was 7 years 11 months. The hormonal laboratory investigations revealed:
- TSH > 100 μIU/mL (0.35 - 4.94)
- FT4 < 2.05 pmol/l (9.74 - 17.1)
- FT3 = 1.31mol/l (4.1 - 7.9)
- prolactin level = 140.90 ng/mL (2.8 - 29.2)
- estradiol (E2) = 82.34 pg/mL (6 - 27)
- FSH = 3.83 mUI/mL (0.3 - 2.8)
- LH < 0.01 mUI/mL (0.1 - 6.0)
This case shows the characteristic features and hormonal profile seen in Van Wyk-Grumbach syndrome:
- chronic hypothyroidism (usually due to autoimmune thyroiditis) with a very high TSH level
- precocious puberty
- delayed bone age
The Van Wyk-Grumbach syndrome is considered as the only entity in which precocious puberty is associated with a delayed bone age, usually due to long-standing hypothyroidism.1.
The hyperprolactinemia is due to 1:
- thyrotrophic hyperplasia of the pituitary gland (as in this case), compressing the pituitary stalk with a secondary disruption of the hypothalamic inhibition of the prolactin
- or direct stimulation by TRH of the prolactin release
The precocious puberty may be related to the high levels of the circulating TSH which acts directly on the FSH receptor resulting in elevation of the estrogen production, which is responsible for the development of secondary sexual characteristics 2.
Additional contributor: ZE. Boudiaf, MD; CHU Constantine, Algeria
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