Moyamoya disease in the United States.

The epidemiology and radiological features of Moyamoya disease (MMD) in the US were investigated. This study encompassed 98 cases; 26 were newly collected from eight US institutions and 72 were previously reported in the US literature. The patients ranged in age from 6 months to 67 years with age peaks in the first, third and fourth decades. MMD was seen in various ethnic groups and females were more commonly involved (71%) than males. A specific etiology could not be determined in most cases but arteriosclerosis and use of oral contraceptives were occasional associations. On angiography and/or magnetic resonance angiography (MRA), carotid arterial stenosis or occlusion was seen bilaterally in 95 cases (97%) and unilaterally in three. On MR or MRA, internal carotid steno-occlusive lesions were well demonstrated in all cases but Moyamoya collateral vessels (MMVs) were visualized in only 65% of the patients. MMVs in the basal ganglia and thalami were best demonstrated on T1 weighted images. Parenchymal lesions were seen in all patients and were often bilateral. With advances in MR techniques and increasing awareness of diagnostic guidelines, MMD will be diagnosed more frequently than before in the US.

Open trial of intravenous immune serum globulin for chronic sinusitis in children.

BACKGROUND: Chronic sinusitis in children is a complex clinical problem. Some patients do not improve with medical therapy and some fail surgery as well. OBJECTIVE: A therapeutic trial of intravenous immune serum globulin (IVIG) was given to children whose sinus disease was recalcitrant to the usual therapeutic modalities. The objective of IVIG administration was to modulate the inflammatory process contributing to the chronicity of the sinusitis. METHODS: Six patients were given a 12-month trial of monthly (400 mg/kg) IVIG infusions. Entry criteria included persistence of sinusitis after 3 months of full course antibiotics, or two episodes of sinusitis within a 3-month period while on prophylactic antibiotics. All patients had abnormal sinus CT (computerized tomography) scans at entry. Three of the six patients remained symptomatic despite prior sinus surgery. Patients with primary immune deficiencies were excluded. Each patient served as his own control based on their previous 12-month history and clinical course. Four of the 6 patients were atopic as demonstrated by prick skin testing; however, all patients had nasal eosinophilia. RESULTS: Full course antibiotic use decreased in five of the six patients (183 to 84 days); correspondingly, the episodes of sinusitis decreased (average 9 to 4 per year). In addition, sinus CT scans showed significant improvement. CONCLUSION: This preliminary open-trial of IVIG suggests its usefulness as adjunct therapy to medical management in selected patients with chronic sinus disease. The mechanism(s) by which IVIG may be helpful is probably not based on the concept of replacement therapy, but more likely as an immune or inflammatory modulating agent.

The eye.

In recent years, the growing application of computed tomography (CT) and magnetic resonance (MR) imaging evaluation of the orbit and globe have dramatically increased the role of imaging and, hence, that of the radiologist in assessing ophthalmologic disorders. This article discusses magnetic resonance imaging features of the most common and less common ocular pathologies, with particular emphasis on the potential of magnetic resonance imaging in the field of ophthalmology.

Vesicoureteral reflux in children with and without a history of urinary tract infection: a comparative analysis.

Eighty-eight children who were discovered to have vesicoureteral reflux were prospectively studied over a two-year period. Two groups were compared: 49 who presented with a urinary tract infection and 39 who were identified through prospective screening. Based on the distribution of grade and the presence of parenchymal scarring, the severity of vesicoureteral reflux was comparable in the two groups. Of note, more males with reflux were identified in the noninfected group, whereas sex distribution was almost equal in the infected group. Ultrasound alone was found to be highly inaccurate in identifying reflux or parenchymal changes. In conclusion, screening of at-risk groups of children without a history of urinary tract infection may identify patients with clinically significant vesicoureteral reflux.

Common causes of low back pain in children.

Low back pain in children and adolescents is often caused by a serious problem. The diagnostic investigation should begin with a complete patient history, complete physical examination, laboratory testing, and plain radiography. These results should be used to direct further imaging studies. Although most of the painful injuries that children sustain in recreational activities are mild, back pain that lasts for extended periods may be due to various disorders, including spondylolysis and spondylolisthesis, disk herniation, Scheuermann disease, or neoplasms. Low back pain can also be caused by diskitis and osteomyelitis, most commonly found in children younger than 10 years old. Primary osseous neoplasms of the lumbar spine are uncommon, with Ewing sarcoma, aneurysmal bone cyst, benign osteoblastoma, and osteoid osteoma being the most common followed by primary lymphoma. These lesions occur more often between the ages of 5 and 20 years. Other causes of low back pain include spinal cord tumors (eg, ependymoma), congenital disorders of the spine (eg, scoliosis), and systemic disease (eg, sickle cell disease).

Ultrafast computerized tomography of the chest in cystic fibrosis: a new scoring system.

Assessing the presence and severity of bronchiectasis (BR) and mucoid impaction (MI) of the airways in cystic fibrosis (CF) is difficult by non-invasive methods. We hypothesized that scoring ultrafast computerized tomograms (UFCT) of the chest for BR and MI could be useful in detecting early lung changes in 28 patients with CF. To do this, UFCT scores were compared to established clinical and chest X-ray (CXR) scores. Results showed that UFCT scores correlated highly with clinical (r = 0.88) and CXR (r = 0.93) scores, and several pulmonary function tests. Regression analysis indicated that BR influenced clinical and CXR scores more than MI. Both BR and MI were found in all areas of the lungs without a major propensity for one region, although there was significantly more BR in the right upper lobe than the right lower lobe. In patients with CXR score greater than or equal to 21 BR was present in 8/9 and MI in 3/9, while in those with CXR score less than 21 UFCT showed BR in 19/19 and MI in 17/19. We conclude: 1) scored UFCTs correlate well with CXR and clinical scores; 2) BR influences clinical and CXR scores more than MI; 3) UFCTs detect BR and MI in CF patients with minimal evidence of pulmonary disease; and 4) CXR scores less than 21 reflect the presence of both BR and MI.

Radiographic features of craniometadiaphyseal dysplasia, wormian bone type.

We describe the radiographic findings in two siblings with a previously unrecognized craniotubular bone dysplasia. We call this condition craniometadiaphyseal dysplasia, wormian bone type. Because the parents of the siblings are consanguineous, this is probably a genetically determined condition with an autosomal recessive type of transmission. The findings in the siblings are compared with those of a woman with the same condition, previously reported as an example of craniometaphyseal dysplasia. The combination of findings in these patients seems diagnostic: characteristic skull changes including multiple wormian bones; wide long tubular bones without normal metaphyseal flaring; wide short tubular bones without normal diaphyseal constriction and sometimes actual diaphyseal expansion; and wide ribs and clavicles.

Chronic pancreatitis with diffuse fibrosis in early childhood.

A 2-year old girl is described in which biliary obstruction was the initial presentation of chronic pancreatitis. A pancreatic biopsy done at laparotomy showed extensive fibrosis not explained by her short clinical course. Chronic pancreatitis with fibrosis in childhood is reviewed.

Omphalocele with pyloroduodenal obstruction by extrinsic hepatic compression: a case report.

The diagnosis of gastroduodenal obstruction by hepatic compression following omphalocele closure must be suspected in the presence of persistent nonbilious vomiting. We feel that computed tomography (CT) is the appropriate imaging method to define the liver abnormality. Nonsurgical management is the best approach with nutritional support maintained by total parenteral nutrition until physiological resolution of the obstruction occurs.

CT evaluation of severe renal inflammatory disease in children.

We have performed CT scans on 15 children and 2 young adults with severe renal inflammatory disease. Most children with urinary tract infections do not require such evaluation. We have, however, found CT helpful in defining the nature of renal abnormality and in defining the extent of disease in selected patients who either presented as diagnostic dilemmas or who did not respond initially to proper medical treatment. We therefore use CT scanning as our initial examination in such problem patients.

Presentation of congenital diaphragmatic hernia past the neonatal period.

Congenital diaphragmatic hernias (CDHs) presenting beyond the neonatal period are a rare and unusual problem; they occurred in 11 of 83 children at our institution. Two discrete clinical groups were apparent: (1) younger children, with mainly respiratory symptoms; and (2) older children with gastrointestinal (GI) complaints. Chest roentgenograms suggested CDHs, but GI contrast studies were necessary for confirmation in eight patients. The diagnosis was made preoperatively in ten of 11 children. At operation, no peritoneal sacs were found, the hernial contents were viable in all patients, and malrotation was present in six of 11 patients. None had evidence of pulmonary hypoplasia. Congenital diaphragmatic hernias do present beyond the neonatal age group in a significant number of cases (13% in our series), and a diagnosis of CDH should be considered in any child with persistent GI or respiratory problems and abnormal chest x-ray film findings. The mortality rate in these patients is small, but morbidity may be significant.

Gastrointestinal tract angiography in infants and children.

In the past 10 years since the development of newer imaging modalities, the method of evaluation of gastrointestinal diseases has changed to less invasive examinations. Angiography of the gastrointestinal tract and its accessory organs, which once was one of the primary procedures for hepatic tumors especially, is now considered nonessential except for occasional demonstration of vascular distribution prior to attempted surgery. As for gastrointestinal bleeding, although the incidence of ulcer disease is not as common in children as in adults, a child with bleeding who is at high risk for surgery will benefit occasionally from intra-arterial infusion of vasopression. As the major cause of gastrointestinal bleeding in children is from esophageal varices, though great anatomical detail of portal circulation can be seen in computed tomography and ultrasound, the coronal mapping of vascular anatomy of portal circulation is of utmost benefit prior to any attempt for surgery. Intra-arterial portography and splenoportography can be very helpful to delineate the anatomy and hemodynamics of portal hypertension, as well as for evaluation of suspected shunt thrombosis. Briefly, an update of information on digital subtraction angiography in gastrointestinal pathology will be given though the pediatric application has not been as popular as in adults.

Esophageal duplication in children: a report of three cases evaluated by computed tomography.

Primary tumors of the esophagus are extremely rare in children. A common periesophageal mass in children is duplication. It constitutes only 0.5 to 2.5% of all esophageal lesions usually diagnosed in infancy or early childhood. Duplication of the esophagus may be diagnosed on examination by the emergency department (ED) physician when it produces symptoms or when it is inadvertently found on a chest radiograph. The duplication may produce acute gastrointestinal symptoms such as vomiting and dysphagia or respiratory symptoms such as cough, wheezing, or dyspnea. Currently the examination of choice for evaluation of esophageal duplication is a barium swallow. However, computed tomography (CT) has the advantage over conventional diagnostic procedures, since it demonstrates the cystic nature of the mass and its relationship to adjacent structures in a noninvasive manner. An esophageal duplication has a well-marginated spherical mass contiguous with the esophagus, with preserved surrounding fat planes, and numbers of 15 to 30 Hausenfield units (HU) on CT. We are reporting three cases of this anomaly as demonstrated by CT.

Renal artery stenosis and hypertension after abdominal irradiation for Hodgkin disease. Successful treatment with nephrectomy.

Hypertension secondary to stenosis of the left renal artery developed in a thirteen-year-old male six years after completion of inverted Y irradiation (3,600 rad) for abdominal Hodgkin disease. Surgical treatment with nephrectomy resulted in control of the hypertension without the use of antihypertensive agents. We review the literature for this unusual complication of abdominal irradiation, and recommend that a 99mTc-DMSA renal scan, selective renal vein sampling for renin determinations, and renal arteriography be performed on any patient in whom hypertension develops following abdominal irradiation in childhood.

Infantile non-African Burkitt's lymphoma presenting as bilateral fulminant exophthalmos.

A case of non-african Burkitt's lymphoma presenting an acute bilateral fulminant exophthalmos in an infantile male is reviewed. The rapid progression of proptosis, and the dramatic response to chemotherapy is documented photographically. A brief description on non-African Burkitt's lymphoma and a differential diagnosis of bilateral infantile fulminant exophthalmos is included.

Significance of roentgenographic abnormalities in children hospitalized for asthma.

The relatively high incidence of roentgenographic abnormalities in patients hospitalized for acute asthma has led some to recommend that all such patients receive chest x-ray examination upon admission to the hospital. We studied the incidence and clinical predictability of the roentgenographic findings by asking the admitting physician to indicate, prior to obtaining a roentgenogram, whether he thought pneumonia, pneumothorax, pneumomediastinum or other significant abnormality was present. A treatment plan was also outlined at that time. Seven of 128 patients (5.5 percent) had roentgenograms suggestive of pneumonia or pneumothorax; three of these were suspected clinically. In 14 cases the clinical suspicion of pneumonia or pneumothorax was not borne out roentgenographically. The initial treatment plan was altered in only three cases. The low incidence of roentgenographic abnormalities and the failure of these minor abnormalities to substantially influence treatment suggest that routine chest roentgenograms may not have to be performed on all children hospitalized for acute asthma.