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- How to evaluate a child for comorbidities associated with Cushing ’ s
syndrome?
A detailed clinical examination including height, weight, and blood pressure
should be performed in all children with Cushing’s syndrome. These children
should also be examined for cutaneous/systemic signs of infection. Biochemical
investigations include fasting and postprandial plasma glucose, lipid profi le,
serum potassium, and assessment of anterior pituitary hormones. X-ray of dor-
solumbar spine and DXA scan should also be performed. - What are the diffi culties in transsphenoidal surgery in children with Cushing ’ s
syndrome?
Transsphenoidal surgery (TSS) is the preferred treatment modality in children
with Cushing’s disease; however, TSS in children is technically challenging
and requires greater expertise as compared to adults. The small size of adenoma
(commonly <5 mm) and non-pneumatization of sphenoid sinus also make TSS
a diffi cult surgery in children. - What is the role of radiotherapy in children with Cushing ’ s disease?
Pituitary radiotherapy is an effective second-line modality for residual/recurrent
disease after transsphenoidal surgery (TSS). The cure rate after external beam
radiotherapy (EBRT) is better in children (92 %) as compared to adults (60–70 %).
In addition, the mean time to response is also faster in children (0.8–1 year) than
in adults (1.5–4 years). Growth hormone defi ciency is the most common anterior
pituitary hormone defi ciency after pituitary irradiation in children, while other
pituitary hormones are usually preserved. The data regarding the use of stereotac-
tic radiotherapy in children is limited; however, it seems to be equally effective.- How to optimize linear growth in a child with Cushing ’ s disease after curative
surgery?
Children with Cushing’s disease may not have optimal catch-up growth even
after curative therapy. This is due to suppressive effect of long-standing hyper-
cortisolemia on GH–IGF1 axis and growth plate or as a result of insult to
somatotropes after TSS. Children with suboptimal catch-up growth after cura-
tive TSS should be subjected to GH dynamic tests after 3 months of surgery and
if found to have GH defi ciency, they should be supplemented with
rhGH. Addition of GnRH analogues may be considered in children who are in
peripubertal age and have compromised predicted adult height. In addition,
children who had undergone radiotherapy should be treated with ketoconazole/
metyrapone during the interim period. GH dynamics should be performed once
eucortisolemia is attained and treated with rhGH, if found to be GH defi cient.
Other hormone defi ciencies, if present, should be adequately replaced. If the
child is on glucocorticoid supplementation, overdosage should be avoided.
4 Childhood Cushing’s Syndrome