PMC4551381_01
Photographic progression demonstrating the dramatic weight gain of our patient over 18 months. a) 18 months prior to presentation, b) Just prior to presentation with her mother and c) At admission while in the ICU
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Fig1
Her rapid weight gain was documented in photographs provided by the family (Fig. 1).
Height and Weight curve over 18 months for our patient, showing a dramatic increase in weight from the 95th percentile to well beyond, yet a negligible height velocity
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Fig2
Physical examination revealed poor growth (height of 98.2 cm, unchanged in 7 months, Fig. 2), central obesity (weight, 40.6 kg; BMI >99th percentile, Fig. 2), moon facies, dorsocervical fat pad, and prominent skin folds on all extremities.
a CT angiogram of the abdomen demonstrating thickened limbs of the adrenal glands with nodular contour. Arrows point to adrenal glands. b Magnetic resonance imaging (MRI) of the abdomen confirmed the presence of thickened nodular adrenal glands with no evidence of a dominant nodule to suggest adenoma or adrenal cortical carcinoma. Arrows point to the two adrenal glands
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Fig3
MRI was performed in an attempt to get a more discriminating view of the adrenal glands. (Fig. 3).
a Adrenal gland on cross sectioning shows several discrete, deeply red-brown nodules, consistent with PPNAD. b Golden brown pigment with the cytoplasm of cortical cells from the adrenal gland are seen on microscopic examination, imparting the typical pigment seen on gross examination with PPNAD (H&E stain, original magnification 400x)
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Fig4
Both adrenal glands demonstrated multiple cortical-based, deeply red-brown pigmented nodules, with intervening atrophic adrenal cortex (Fig. 4a). Microscopic examination of both the right and left adrenal glands showed features consistent with primary pigmented nodular adrenocortical disease (Fig. 4b).