Ann Pediatr Endocrinol Metab.  2014 Sep;19(3):164-168. 10.6065/apem.2014.19.3.164.

Long-term follow-up on Cushing disease patient after transsphenoidal surgery

• Jeong I
• Oh M
• Kim JH
• Cho JH
• Choi JH
• Yoo HW

• KMID: 2134085
• DOI: http://doi.org/10.6065/apem.2014.19.3.164

Abstract

Cushing disease is caused by excessive adrenocorticotropic hormone (ACTH) production by the pituitary adenoma. Transsphenoidal surgery is its first-line treatment. The incidence of Cushing disease in children and adolescents is so rare that long-term prognoses have yet to be made in most cases. We followed-up on a 16-year-old male Cushing disease patient who presented with rapid weight gain and growth retardation. The laboratory findings showed increased 24-hour urine free cortisol and lack of overnight cortisol suppression by low-dose dexamethasone test. The serum cortisol and 24-hour urine free cortisol, by high-dose dexamethasone test, also showed a lack of suppression, and a bilateral inferior petrosal sinus sampling suggested lateralization of ACTH secretion from the right-side pituitary gland. However, after a right hemihypophysectomy by the transsphenoidal approach, the 24-hour urine free cortisol levels were persistently high. Thus the patient underwent a total hypophysectomy, since which time he has been treated with hydrocortisone, levothyroxine, recombinant human growth hormone, and testosterone enanthate. Intravenous bisphosphonate for osteoporosis had been administered for three years. At his current age of 26 years, his final height had attained the target level range; his bone mineral density was normal, and his pubic hair was Tanner stage 4. This report describes the long-term treatment course of a Cushing disease patient according to growth profile, pubertal status, and responses to hormone replacement therapy. The clinical results serve to emphasize the importance of growth optimization, puberty, and bone health in the treatment management of Cushing disease patients who have undergone transsphenoidal surgery.

Keyword

Pituitary ACTH hypersecretion; Petrosal sinus sampling; Hypopituitarism

MeSH Terms

Adolescent
Adrenocorticotropic Hormone
Bone Density
Child
Dexamethasone
Follow-Up Studies*
Hair
Hormone Replacement Therapy
Human Growth Hormone
Humans
Hydrocortisone
Hypophysectomy
Hypopituitarism
Incidence
Male
Osteoporosis
Petrosal Sinus Sampling
Pituitary ACTH Hypersecretion*
Pituitary Gland
Pituitary Neoplasms
Prognosis
Puberty
Testosterone
Thyroxine
Weight Gain
Adrenocorticotropic Hormone
Dexamethasone
Human Growth Hormone
Hydrocortisone
Testosterone
Thyroxine

Figure

• 

  Fig. 1 Simple radiographic findings on thoracolumbar spine. The anteroposterior (A) and lateral (B) views show generalized osteopenia and rugger-jersey spine, both indicative of osteoporosis.

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  Fig. 2 Growth curve of Cushing disease patient. The arrow indicates the initiation of the recombinant human growth hormone (GH), pamidronate, and testosterone enanthate. After GH therapy, his final adult height attained the midparental level.

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