Renal ultrasound abnormalities in children with end-stage liver disease--reversal by liver transplantation.

Ultrasound (US) imaging of the kidneys was performed in 43 pediatric patients with end-stage liver disease evaluated for orthotopic liver transplantation. Renal size was increased in 8 patients (19%) and echogenicity of the kidneys was increased in 12 patients (28%). In 12 patients studied after liver transplantation, US revealed normal renal size in all and increased echogenicity in only 2 patients shortly after transplantation. Normalization of renal US findings was also found in 2 patients studied before and after liver transplantation. The glomerular filtration rate varied from 65 to 225 ml/min per 1.73 m2 in 5 patients with abnormal US, and from 74 to 116 ml/min per 1.73 m2 in 3 patients with normal US. Nephromegaly and increased echogenicity on renal US are frequent in children with end-stage liver disease and appear to be mostly reversible by liver transplantation.

Infantile hepatic hemangiomas. Clinical features, radiologic investigations, and treatment of 20 patients.

The clinical features, radiologic investigation, and treatment of 20 infants with hepatic hemangiomas are presented. Palpable abdominal mass (n = 18) and cardiac failure (n = 11) were the common presenting features. Nine patients had hyperconsumptive coagulopathy. Seven patients had other hemangiomas. Ultrasound (n = 15) showed the number and distribution of the hemangiomas within the liver. Hypoechoic and hyperechoic elements were present in addition to prominent vascular channels and diminished caliber of the distal aorta. Radionuclide sulfur colloid (n = 12) and labeled red blood cell (n = 7) studies showed the distribution and vascularity of the hemangiomas. Computed tomography (n = 8) revealed central hypointensity with marked peripheral enhancement after contrast. Arteriography now performed only as a prelude to therapeutic embolization demonstrated hypervascularity in each patient, contrast pooling in six and early draining veins in five. Magnetic resonance scanning (n = 3) showed decreased signal intensity on T1 images and high intensity signal on T2. In two patients, there was resolution or improvement of the hemangiomas without therapy. Four patients had surgery (lobectomy [2], trisegmentectomy [1], and surgical evacuation of a central hematoma [1]). Steroids and radiation were given to seven patients, and one patient also required therapeutic embolization. Steroids were the initial therapy in five patients, one of whom later required therapeutic embolization and another cyclophosphamide. Two patients were treated initially with radiation therapy, one of whom also needed emergency hepatic artery ligation. Seventeen of the 20 patients are alive and well from 6 months to 14 years after diagnosis.

Scoliosis after thoracotomy for esophageal atresia.

Progressive scoliosis was seen in eight children after thoracotomy for esophageal atresia. Postoperatively, all had had severe mediastinitis and empyema secondary to dehiscence of the esophageal anastomosis and required reoperation. Healing was accompanied by marked scarring and rib fusion; with growth, a scoliosis developed with the concavity toward the thoracotomy site. Most spinal curvatures appeared years after the thoracotomy and progressed rapidly at the time of adolescent growth spurt. Excision of fused ribs, spinal fusion, and instrumentation led to improvement in four patients.