David C. Gregg

Inflammatory myofibrohistiocytic proliferation simulating sarcoma in children

The term “inflammatory myofibrohistiocytic proliferation” (IMP) has been proposed to replace the conventional designations of plasma cell granuloma and inflammatory pseudotumor. Three cases of extrapulmonary IMP in children are reported, including an intracerebral lesion which has been formerly undescribed. In children, IMP may be associated with microcytic hypochromic anemia, hypergammaglobulinemia, and high erythrocyte sedimentation rate. In this clinical setting, differentiation of a rapidly growing but benign IMP from a bona fide sarcoma is of paramount importance.

Mycoplasma hominis and Ureaplasma species brain abscess in a neonate.

We report an infant with a mixed Mycoplasma and Ureaplasma brain abscess who was successfully treated with intravenous doxycycline and erythromycin. Therapeutic concentrations of doxycycline were demonstrated in cerebrospinal fluid. This report is evidence of the potential for Mycoplasma and Ureaplasma to produce focal central nervous system infection, as well as meningitis, in neonates and that use of doxycycline can be efficacious in the therapy of such infections.

THE NORMAL KIDNEY GROWTH RATE DURING YEAR 1 OF LIFE IS VARIABLE AND AGE DEPENDENT

AbstractPurpose: The concept of normal contralateral kidney growth rate was introduced as a parameter to consider in conjunction with others in the evaluation of unilateral hydronephrosis in asymptomatic neonates and infants. Historically measurements of renal size obtained at different times from different patients have been relied on to construct normal renal growth charts. We report normal kidney growth rates in year 1 of life derived from interval ultrasound measurements of renal size in the same newborns and infants longitudinally with time.Materials and Methods: We reviewed 333 normal renal ultrasound studies performed between 1991 and 1995 in 55 patients during year 1 of life. These longitudinal measurements were made bilaterally 2 to 8 times (median 3) per patient. Growth curve models that consider random patient effects were used to estimate the mean growth curve and ascertain its form. We also reviewed an additional 91 ultrasound studies in 10 patients with myelomeningocele without hydronephrosi...

Sonographic diagnosis of enlarged prostatic utricle.

The enlarged prostatic utricle or prostatic utricle cyst is a condition frequently associated with hypospadias and cryptorchidism.• This embryologic remnant is felt to represent a residual mullerian duct structure.2 We present a case of enlarged prostatic utricle identified as a cystic pelvic mass. The sonographic findings of this entity and the differential diagnosis of cystic pelvic lesions in the male infant are discussed briefly.

Lymphoscintigraphy. Chylous ascites and lymphocele demonstration.

A 12-year-old girl had a gradual onset of abdominal detention. A computed tomographic study of the abdomen showed a fluid-filted peritoneal cavity without masses. The fluid was chylous in nature. A lymphoscintigram was performed to evaluate for the site of obstruction or leakage. A positive contrast radiographic pedal lymphogram is shown for comparison. The value of lymphoscintigraphy is illustrated.

Ga-67 imaging of "cervical collar" sign in periarteritis nodosa.

A 30-month-old girl was evaluated with Ga-67 imaging to identify foci of abnormal inflammation. Biopsy of a cutaneous lesion demonstrated manifestations of periarteritis nodosa, which included polymorphic exanthemata-purpuric, urticarial, and multiform in character. There was also severe subcutaneous hemorrhage resulting from necrotizing arteritis. Ga-67 imaging shows the only abnormality located to the posterior neck muscles at sites of cutaneous involvement. Other areas were normal

Nail-patella syndrome. Image correlation.

The nail-patella syndrome is an autosomal dominant disorder characterized by deformities of a variety of mesodermal and ectodermal elements. Most affected patients exhibit hypoplasia, dysplasia, or absence of the fingernails and toenails. Other common sites of involvement include the skin, kidneys, pelvis, knees, and elbows. In many cases, the earliest radiographic manifestations of this disorder in infants and children is osteocartilage exostoses (iliac horns) of the iliac wings. These exostoses may be detected with skeletal scintigraphy, and should not be confused with other pathologic conditions

DISCUSSION: THE NORMAL KIDNEY GROWTH RATE DURING YEAR 1 OF LIFE IS VARIABLE AND AGE DEPENDENT

PURPOSE The concept of normal contralateral kidney growth rate was introduced as a parameter to consider in conjunction with others in the evaluation of unilateral hydronephrosis in asymptomatic neonates and infants. Historically measurements of renal size obtained at different times from different patients have been relied on to construct normal renal growth charts. We report normal kidney growth rates in year 1 of life derived from interval ultrasound measurements of renal size in the same newborns and infants longitudinally with time. MATERIALS AND METHODS We reviewed 333 normal renal ultrasound studies performed between 1991 and 1995 in 55 patients during year 1 of life. These longitudinal measurements were made bilaterally 2 to 8 times (median 3) per patient. Growth curve models that consider random patient effects were used to estimate the mean growth curve and ascertain its form. We also reviewed an additional 91 ultrasound studies in 10 patients with myelomeningocele without hydronephrosis or reflux. We analyzed the kidney growth rate in a similar manner and compared the results with those in the normal patients. RESULTS Analysis of the normal cases revealed a changing growth rate during year 1 of life estimated to be 3.1 mm. per month at birth, decreasing gradually during the first 7 months of life to 0.25 mm. per month and remaining constant thereafter (p = 0.0064). Mean kidney size at birth was estimated to be 42.8 mm. (95% confidence limits 27.0 to 58.5). At age 7 months mean size was 60.7 mm. (95% confidence limits 40.1 to 81.3). CONCLUSIONS These data demonstrate that normal kidney growth is age dependent. A rapid but slowly decreasing growth rate during the first 7 months of life is followed by a more constant and lower rate. The data also suggest that unusually rapid growth, such as that which may occur in the normal contralateral kidney in unilateral obstruction, may also be age dependent, exceeding 5.0 mm. per month during the first 7 months of life and 2.0 mm. per month thereafter.

Larsen Syndrome : Extraosseous uptake of bone imaging agent

Larsen syndrome is characterized by anomalies present at birth. These include a flat face, hypertelorism, cleft palate, cleft uvula, and laxity of joints with dislocations. In addition, there are central nervous system abnormal- ities, congenital heart defects, and neurological impairment due to cord compression. Not all anomalies may be present, and the condition is frequently under diagnosed. Our patient presented with right leg pain and limitation of motion of the right hip. The diagnosis of Larsen syndrome was not known. A radionuclide bone scan was performed to rule.out an acute injury. The examination demonstrated extraossoeous accumulation of tracer in the right thigh. An MRI scan showed edema involving the entire right sartorius muscle. This was secondary to repeated dislocations of the hip because of Larsen syndrome, which was established with genetic consultation.

Metastatic Wilm's tumor to the central nervous system : Extraosseous accumulation of bone seeking radiopharmaceutical

A 6-year-old boy was examined for left leg and arm weakness. The patient had a left Wilms tumor resected 6 years earlier. Treatment also consisted of chemotherapy and abdominal radiation. The child was asymptomatic until the present admission. A radionuclide bone scan was performed as part of the evaluation for metastatic bone disease. The examination demonstrated several regions in the skeleton with metastatic deposits. In addition, an area of abnormal extraosseous accumulation was identified in the right parietal region. A computed tomograph examination confirmed metastatic cerebral metastases. The case illustrates necessity to carefully evaluate the extraosseous components of the radionuclide bone images.