A child with neurofibromatosis and hypertension also demonstrated renal artery stenosis, the most common cause of hypertension in children with neurofibromatosis; abdominal coarctation, which has previously been described; and thoracic coarctation, which, to our knowledge, has not been previously reported. Rib notching may appear in patients with uncomplicated nueorfibromatosis, but the possibility of associated thoracic coarctation must also be considered in these patients. The role of neurofibromatosis in children with hypertension is now well established. The presence of neurocutaneous syndromes in associa¬ tion with pheochromocytoma has been long known.1·2 There is a more recent awareness of renal artery stenosis as (Dr. Rowen). the most frequent cause of hyperten¬ sion in patients with neurofibroma¬ tosis.315 A few of these patients also have associated abdominal aortic coarctation,111419 but to date no case of thoracic coarctation has been de¬ scribed. The case presented here dem¬ onstrated both thoracic and abdomi¬ nal coarctation as well as patent ductus arteriosus, superior mesenteric artery stenosis, and minimal right re¬ nal artery stenosis. A 17-year-old girl exhibited congenital neurofibromatosis with extensive involve¬ ment of the right eye, face, neck, and scalp at birth. She had repeated surgical proce¬ dures to remove neurofibromatous tumors.A heart murmur was first noted at birth, but she was not seen by a cardiologist until 9 years of age. At that time blood pres¬ sure was 118/80 in the right arm, 128/64 in the left arm, and 108/62 mm Hg in the right leg, with radial pulses noted to be good and femoral pulses fair in intensity.A third sound was present at the apex. The S2 was normally split. A grade 3 harsh sys¬ tolic ejection murmur was maximal at the second interspace in the left sternal border and well heard over the upper left part of the back.Blood pressure at 12 years of age was 130/80 in the right arm, 144/76 in the left arm, and 92/76 mm Hg in the right leg, and the femoral pulses were now weak.Cardiac catheterization at that time showed pressures of 180/0 in the left ventricle, 180/90 in the ascending aorta, 135/80 in the descending aorta, and 105/75 mm Hg in the abdominal aorta. She was admitted to Childrens Hospital of Orange County at 14 years of age for thoracic spinal fusion for scoliosis, fol¬ lowed six weeks later by cervicothoracic fusion with a Harrington rod.Cardiac catheterization was repeated at 15 years of age, again showing thoracic and abdominal coarctations with severe stenosis of the superior mesenteric artery, possible right renal artery stenosis, dupli¬ cated left renal arteries, and a small pa¬ tent ductus arteriosus (Fig 1 and 2). Pres¬ sures at that time were 210/0 in the left ventricle, 205/100 in the ascending arch of the aorta, 180/0 in the descending thoracic aorta, and 150/95 mm Hg in the abdominal aorta. At operation, an aorticoaortic graft from the distal part of the thoracic aorta to the infrarenal aorta was performed. Direct Downloaded From: http://archpe...
