Introduction
Hypomelanosis of Ito is a multisystemic disorder affecting skin, eyes, central nervous system, extremities and heart. Skin manifestations include linear or macular hypopigmented lesions along with Blaschko’s line arising from low-count of melanocytes.The most common complication of this disorder is assosciated with central nervous system such as convulsions and mental retardation. Cardiovascular findings are very rare.
Case
A Three years old girl who admitted to public hospital with complaints of vomiting and diarrhea was referred to our center with the diagnosis of hypertension. There was a psychotropic drug exposure in her prenatal history. She was born with cesarean birth from a multiple pregnancy, birth weight was 3450 gr. She had been operated four times due to an anal atresia in the first year of life. According to the past history motor and mental development, speaking and walking ability, was found obviously delayed than her peers. At the first physical examination, body weight was 10 p, height and head circumference were 75-90 p. A closure defect at the right eyelid, some linear hypopigmented lesions on the left side of the trunk and the left arm were found. Additionally, right sided hemihypertrophy and scar tissue belong to last operation on the left abdominal wall were also seen. Eye examination also revealed that the right coloboma of iris was exist. Dermatological examination made by an expert dermatologist was resulted as hypomelanosis of ito. Right upper extremity blood pressure was measured as 190/80 mmHg, lower extremity blood pressure was 110/80 mmHg. Femoral arterial pulses were too weak. With these findings, a coarctation of the aorta was considered and echocardiographic examination was performed but no coarctation was found. Thereon, a catheter angiography was performed. In the angiography, an abdominal aortic coarctation (middle aortic syndrome) and assosciated collateral vascular structures were diagnosed. She was referred to surgery after starting a beta-blocking agent.
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