Journal of Pediatric Surgery Case Reports 65 (2021) 101749

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Journal of Pediatric Surgery Case Reports

Cushing's syndrome in a child

Kalyan Dutta a, *, Hemonta Dutta b, **
a Department of Surgery, Assam Medical College & Hospital, Dibrugarh, India
b Department of Pediatric Surgery, Assam Medical College & Hospital, Dibrugarh, Assam, India

ARTICLE INFO ABSTRACT

Keywords: Cushing's syndrome is a rare entity in children. Adrenal tumour is the common cause of this syndrome in young
Cushing's syndrome children, whereas, iatrogenic causes are more common among older children. We report a 4 year old male child
Adrenal adenoma
diagnosed with Cushing syndrome due to a right adrenal adenoma; the child presented with obesity and increase
Adrenalectomy
distribution of body hair. After thorough investigation and control of hypertension and dyselectrolytemia, right
adrenalectomy was performed. The patient had good clinical recovery with weight loss and biochemical resolu-
tion of Cushing's syndrome.

1. Introduction
Cushing's syndrome (CS) is rarely encountered in children. The
overall incidence of Cushing syndrome is approximately 2–5 new cases
per million people per year. Only approximately 10% of the new cases
each year occur in children [1]. Unlike in adults, a male-to-female pre-
dominance have been observed in infants and young toddlers [1–3].
Athough iatrogenic causes are common in children above seven years of
age, adrenal causes (adenoma, carcinoma or hyperplasia) are common
in children of younger age [4]. We report a 4 year old boy diagnosed
with Cushing syndrome caused by a right adrenal adenoma, who had
presented with obesity and increase distribution of body hair. Right
adrenalectomy was performed and clinical stabilization resulted in
weight loss and biochemical resolution of Cushing's syndrome. (see Fig.
5)
2. Case report
A 4 years old boy presented with complaints of excessive weight
gain of 5 months duration and increase frequency of micturition and
appearance of body hair for 4 months. There was no history of any
other illness, medication or steroid intake. The child was first born at
term by normal vaginal delivery and birth weight of 3 kg. Physical ex-
amination revealed a chubby boy with moon face, buffalo hump, pro-
truding abdomen, increase body hair and appearance of coarse pubic
hair (Fig. 1). His intelligent quotient (IQ) was appropriate for his age
and sex. His younger sibling was in good health and other family mem-
bers did not have any metabolic or similar problems.
The patient's body length was 92cm (between -2SD to -3SD), weight
20kg (between 1 SD and 2 SD), weight for height >3SD, and BMI was
23.6 (BMI for age >3 SD). His blood pressure on right arm in lying
position was 138/76 mm Hg (above 99th percentile for height and
age).
Investigations: Morning 8am serum cortisol level - 27.3 μg/dl
(normal: 6–23 μg/dl).
with a concurrent plasma ACTH level of < 5 pg/ml (n
value < 46 pg/ml).
His serum cortisol following low dose dexamethasone suppression
test (1mg dexamethasone at 11pm) at 8 am next morning was
22.1 μug/dl and his 24 hours urine catecholamine fraction was
within normal limit.
HB % -- 10.3 gm/dl; LDDST -- 25 μg/dl; FBS -- 106 mg/dl.
Serum Na+ - 140.6mmol/l; K+ - 2.83mmol/l; Ca+ - 8.7 mg/dl.
S. Creatinine −0.3 mg/dl.
Ultrasonography of abdomen revealed a heterogenous predomi-
nantly hypoechoic right supra renal mass. Contrast enhanced CT ab-
domen revealed well defined soft tissue density lesion (size
−5.2 cm × 5.2 cm x 5.7cm) in right adrenal gland with calcifications
and fat attenuations showing mild attenuation on post contrast study
(Fig. 2).
The child was started on oral amlodipine 2.5mg 12hourly; after
5days blood pressure became normal. For hypokalemia oral potassium
was given @20 meq 8 hourly and serum potassium value became nor-

* Corresponding author.
** Corresponding authior.
E-mail addresses: kalyandutta918@gmail.com (K. Dutta), hemontdut@gmail.com (H. Dutta).

https://doi.org/10.1016/j.epsc.2020.101749
Received 27 November 2020; Accepted 11 December 2020
Available online 18 December 2020
2213-5766/© 2020 Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
K. Dutta and H. Dutta
Fig. 1. The child with moon face, protruded abdomen and coarse body hair.
Fig. 2. CECT shows right adrenal mass with calcification and mild attenuation on post-contrast study.
mal after 4 days. Right laparoscopic adrenalectomy was planned. but
due to intra operative technical problems it was converted to an open
adrenalectomy with right subcostal incision. A lobulated mass of size
9 cm × 5 cm x 4 cm with intact capsule was excised. The tumour
weighed 230 gm. There was no adhesion with adjacent organs, three re-
gional nodes were enlarged but without any tumour tissue. Inferior
vena cava was spared. Histopathology report was consistent with
adrenal adenoma (Fig. 3) (see Fig. 4).
Post operative management: during post operative period hy-
pokalemia and flaxuating blood sugar level was managed with oral
potassium and oral glucose supplement. patient developed mild cough
and respiratory distress on post op day 2, it was managed with salbuta-
mol nebulization and respiratory physio therapy. Patient developed mi-
Fig. 3. Cut section of tumour shows fleshy mass with fatty tissue.
2
Journal of Pediatric Surgery Case Reports 65 (2021) 101749
nor ssi and discharged on 10 th post operative day with oral pred-
nisolone supplementation.
Follow up: the patient was followed up 2week after discharge and
then every monthly, the oral prednisolone was gradually tapered and
completely withdrawn on 2nd month after surgery.The patient experi-
enced no post-surgical complications. After 4 months of surgery he re-
duces 6 kgs of his body weight with BMI of 16.5 (between median
and 1SD) & BP 100/74 mm hg (within normal range), the moon face,
buffalo hump, central obesity disappeared, morning 8am serum corti-
sol level was found within normal range 14 μg/dl (n value 6–23 μg/
dl).
3. Discussion
Cushing's syndrome is caused by prolonged exposure to supraphysi-
ological levels of circulating glucocorticoids, which may be endoge-
nously or exogenously derived. During infancy, CS is usually associated
with McCune-Albright syndrome; adrenocortical tumours most com-
monly occur in children under four years of age and Cushing's disease
(ACTH dependent) is the commonest cause of CS after five years of age
[5]. Primary adrenocortical tumours (ACTs) account for only 0.3–0.4%
of all childhood neoplasms. Almost a third of these tumours manifests
as Cushing syndrome and over 70% of the unilateral tumours in young
children are often malignant [2,3,6,7]. There seems to be a bimodal in-
cidence of these tumours, with one peak at under 5 years of age and the
second one in the fourth or fifth decades of life. ACTs may be associated
with other syndromes, such as, Li-Fraumeni syndrome, Beckwith-
wiedemann syndrome, isolated hemihypertrophy, or even a germline
point mutation of P53 tumour suppressor gene as reported in a series
from Brazil [8]. In comparison to adult CS, growth failure with associ-
K. Dutta and H. Dutta Journal of Pediatric Surgery Case Reports 65 (2021) 101749

Fig. 4. Microphotograph (100 × 10) showing intact capsule and adrenal tumour cells, which are larger in size with nuclear pleomorphism, inconspicuous nucle-
oli, cytoplasm of the tumour cells are abundant, eosinophilic and vacuolated.

Fig. 5. Physical appearance 4 months after adrenalectomy.

ated weight gain is one of the most reliable indicators of hypercortiso- 4. Conclusion
laemia in pediatric CS. The parents often fail to notice facial changes
and growth failure and hence the diagnosis is often delayed. In one Pediatric adrenocortical tumours (ACTs) are most commonly en-
study, the mean time from appearing symptoms to diagnosis in 33 chil- countered in females and in children less than four years. But our case
dren with Cushing's disease was 2.5 years [5]. More recently the com- being an 4-year-old boy forms a rare presentation of endogenous Cush-
parison of height and BMI SDS measurements provided a sensitive diag- ing's syndrome due to adrenal adenoma. Cushing's syndrome in this
nostic discriminator in pediatric patients with CD and those with simple child was controlled after right adrenalectomy.
obesity [9]. In the present case, the parents observed noticeable
changes in his face and presence of body hair, which made them to Patient consent
bring the child to medical attention. A review of 254 children on the In-
ternational Pediatric Adrenocortical Tumour Registry identified viril- Informed written consent was taken.
ization as the most common manifestation [10]. About 10% of the tu-
mours can be non-functional at presentation, and approximately one Funding
third of pediatric patients present with hypertension. Majority of pa-
tients (192/254) in the Registry had localized disease and metastatic No funding or grant support.
disease was found in less than 5% of cases. Older children with CS or
mixed androgen and cortisol secreting adrenocortical tumours had a Authorship
worse prognosis compared to younger children [10]. The present case
had mild hypertension as well as dyselectrolytemia at presentation, All authors attest that they meet the current ICMJE criteria for au-
which could be controlled with medication. He had a single adenoma thorship.
confined to the adrenal gland and there was no evidence of malignancy.
After surgical excision of the tumour and the right adrenal gland, the Declaration of competing interest
patient made rapid improvement in clinical condition and has been on
follow up for last 7 months. The authors declare that they have no known competing financial
interests or personal relationships that could have appeared to influ-
ence the work reported in this paper.

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K. Dutta and H. Dutta Journal of Pediatric Surgery Case Reports 65 (2021) 101749

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