[MIM 160 980, 605 244, 608 837]

(PPNAD acronym for Primary Pigmented Nodular Adrenocortical Disease)

Rare, less than 1% of the causes of Cushing's syndrome. This is one of the causes of  hypercortisolism insensitive to ACTH, in the child and young adult. It is caused by a mutation of gene PRKAR1A (17q22-24) which codes for the regulatory subunit R1A the PKA. 

Variable association of:

-          micronodular pigmented dysplasia  of the adrenal glands (25-45%) with cyclic hypercortisolism resulting in Cushing syndrome

-         lentiginosis (62%) around the natural orifices

-         a cardiac (30-70%) or cutaneous (60%) or mammary myxoma, sometimes perinervous (schwannomas)

-         tumors: testicle (56%, bilateral), thyroid (10%)

-         ovarian cysts (67%)

-         acromegaly (10%)

Anesthetic implications: 

sequelae of the hypercortisolism:

-         high blood pressure

-         important proteolysis, particularly in the postoperative period (risk of wound dehiscence);

-         hyperglycemia resistant to insulin ;

-         fragility of the vascular walls (difficulty to place venous lines, important bruises);

-         decreased resistance to infection (antibioprophylaxis);

-         brittle bones (osteoporosis)

-  difficult intubation in case of acromegaly

In case of bilateral surrenalectomy, substitutive steroid opotherapy.

References : 

• Groussin L, Cazabat L, René-Corail F, Jullian E, Bertherat J. 
  Le complexe de Carney : du syndrome de Cushing à la génétique. 
  Métabolismes Hormones Diabètes et Nutrition 2005 ; 9 : 38-43.
• Staudt G, Caman W.
  Successful pregnancy and delivery in a patient with Carney Complex.
  A &A Case Reports 2015 ; 4 : 12-3.

Updated: December 2016