Disturbances of bone growth and development

"What is growth anyway? Can one talk about positive growth in childhood, neutral growth in maturity, and negative growth in old age?" Our goal is to help promote normal positive growth in infants and children. To achieve this, we must be cognizant of the morphologic changes of both normal and abnormal bone formation as they are reflected in the radiographic image of the skeleton. The knowledge of the various causes and the pathophysiologic mechanisms of the disturbances of bone growth and development allows us to recognize the early radiographic manifestations. Endocrine and metabolic disorders affect the whole skeleton, but the early changes are best seen in the distal ends of the femurs, where growth rate is most rapid. In skeletal infections and in some vascular injuries two-or three-phase bone scintigraphy supercedes radiography early in the course of the disease. MRI has proved to be very helpful in the early detection of avascular bone necrosis, osteomyelitis, and tumor. Some benign bone tumors and many bone dysplasias have distinct and diagnostic radiographic findings that may preclude further studies. In constitutional diseases of bone, including chromosomal aberrations, skeletal surveys of the patient and all family members together with biochemical and cytogenetic studies are essential for both diagnosis and genetic counseling. Our role is to perform the least invasive and most informative diagnostic imaging modalities that corroborate the biochemical and histologic findings to establish the definitive diagnosis. Unrecognized, misdiagnosed, or improperly treated disturbance of bone growth can result in permanent deformity usually associated with disability.     

Otitis media and hearing loss in Turner syndrome

Twenty-two phenotypic females with Turner syndrome underwent prospective otologic evaluation including a standard history, physical examination, audiogram, and tympanogram. Eight of these patients had computed tomography of the temporal bones. Eighty-two percent of the patients had a history of chronic or recurrent ear infections. Eleven patients (50%) had previous myringotomy and tube placement and 4 (16%) had undergone tympanoplasty or tympanomastoidectomy for sequelae of otitis media. Ten patients (45%) had middle ear effusions evident on examination. Sixteen patients (73%) had hearing loss in at least one ear at the time of examination. Sensorineural losses were evident in 37% of patients. No malformations of the otic capsule were noted on computed tomography. The high prevalence of both hearing loss and otitis media in Turner syndrome warrants otologic and audiologic assessment of patients with this chromosomal anomaly.     

Respiratory syncytial virus infections in pediatric liver transplant recipients.

Respiratory syncytial virus (RSV) is the most important cause of lower respiratory tract infection in infants and young children. The charts of 17 children found to have RSV among 493 children who underwent liver transplantation between February 1985 and February 1991 were reviewed. The median age at diagnosis was 20 months. Median time of diagnosis was 24 days after transplantation. Thirteen patients developed nosocomial infections while convalescing from their transplant. Common symptoms included tachypnea, cough, fever, and congestion. Acute radiographic changes were seen in 12 patients. Two deaths were associated with progressive pulmonary disease and occurred in children with infection early in the postoperative period who were intubated before the onset of symptoms. RSV in children after liver transplantation has a clinical spectrum similar to that in normal children. Early onset of infection (less than 20 days) after transplantation and preexisting lung disease may predict more severe disease.     

The cerebro-hepato-renal (Zellweger''s) syndrome: report of four cases

Four cases of cerebro-hepato-renal syndrome are described. Radiographic findings of scimitar-shaped calcifications of the patellae and stippled cartilage califications have previously been described. We noted calcifications in the area of the greater trochanters in 2 of our patients, and bell-shaped thoraces in all patients. One of our patients is the longest survivor of the children affected by this syndrome.     

Pediatric liver transplantation. Part II. Diagnostic imaging in postoperative management

The postoperative diagnostic imaging examinations of 44 children who underwent 59 orthotopic liver transplantations were reviewed. The imaging modalities used for the evaluation of suspected complications include plain roentgenography, ultrasonography (US), computed tomography (CT), nuclear scintigraphy, arteriography, percutaneous and operative cholangiography, and endoscopic retrograde cholangiopancreatography. The main postoperative complications included ischemia, thrombosis (hepatic artery and portal vein), infarction, obstruction or leakage of the biliary anastomosis, hepatic and perihepatic infection, and allograft rejection. US, the most frequently used abdominal imaging modality, was best suited for detection of biliary duct dilatation, fluid collections in or around the transplanted liver, and hepatic arterial, inferior vena caval, and portal vein thrombosis. CT was especially helpful in corroborating findings of infection and in locating abscesses. Technetium 99m sulfur colloid (early- and late-phase imaging) provided a sensitive, although nonspecific, means of assessing allograft vascularization and morphology. Angiography showed vascularity most clearly, and cholangiography was the most useful in the assessment of bile duct patency. A diagnostic imaging algorithm is proposed for evaluation of suspected complications.     

Radiographic manifestations of anomalies of the limbs

Limb anomalies and their commonly associated organ malformations are increasingly recognized in fetal life because of the use of high resolution real-time sonography. In most instances plain radiography establishes the diagnosis of limb anomalies shortly after birth. In some neonates the diagnosis is tentative until full skeletal maturity is attained. When evaluation of the soft tissues and unmineralized cartilage or ossification center is a prerequisite to early definitive therapy, computed tomography and magnetic resonance imaging are the procedures of choice.     

Radiographic manifestations of anomalies of the gastrointestinal tract

Congenital anomalies of the gastrointestinal tract can pose serious threats to the health of newborn infants and children. Perhaps nowhere has pediatric surgery had as dramatic an impact as in the care and treatment of these conditions. The pediatric radiologist works closely with the surgeon in evaluating these anomalies in young children. Plain radiographic films and contrast studies have been and remain the first step in studying these anomalies. Newer imaging modalities, however, also have made contributions to the continuing importance of the role of the radiologist in the diagnosis and care of children with these anomalies.     

Abnormal paranasal sinuses in patients with cystic fibrosis of the pancreas

Radiographic examinations of the paranasal sinuses and chests of 187 patients with C.F. from five months to 27 years of age were reviewed. One hundred eighty-five patients consistently had opaque maxillary and ethmoidal sinuses. Forty-five patients had normal chest radiographs. These observations show that the paranasal sinuses are almost always affected in children with C.F. Although opaque paranasal sinuses do not indicate the diagnosis of C.F., clear paranasal sinuses exclude this disease with reasonable certainty.