Splenic lymphangiomatosis in children.

PURPOSE: To study the clinical and imaging features of splenic lymphangiomatosis. MATERIALS AND METHODS: The clinical and abdominal imaging data of 10 children with splenic lymphangiomatosis were retrospectively reviewed. The modalities used in the study included computed tomography (CT) (10 patients), sonography (five patients), and magnetic resonance (MR) imaging (two patients). Pathologic confirmation of lymphangiomatosis was obtained in nine patients. RESULTS: Splenic lymphangiomatosis was discovered incidentally in all cases and was a key finding in enabling the correct diagnosis in six children with extrasplenic disease as well. Only two children had clinical splenomegaly. Sonograms and MR images showed multiple, wEll-defined cysts. Multiple, low-attenuation lesions that did not enhance with intravenous administration of contrast material (n = 8) or a mottled spleen (n = 2) were seen at CT. One of the mottled spleens had target lesions on an early (arterial) image obtained after administration of a bolus of contrast material. One child underwent a splenectomy; one child underwent therapeutic embolization. Eight patients remained asymptomatic with respect to the spleen 1-20 years later. CONCLUSION: Splenic lymphangiomatosis is often an incidental imaging finding that frequently has a characteristic imaging appearance. The recognition of this appearance helps in diagnosis of this disease and may prevent the need for further invasive procedures. Splenic changes can be isolated or can coexist with bone or soft-tissue lymphangiomas.

Congenital intraluminal tracheal cyst causing an obstructed left lung in a newborn.

Intraluminal tracheal or bronchial cysts are rare. We present a congenital cyst of the distal trachea which caused obstruction of the left main-stem bronchus. The chest radiograph obtained at birth showed an expanded, irregularly opaque left lung. Within several hours, the opacity cleared and the lung became markedly hyperexpanded. The cyst ruptured spontaneously with resolution of the symptoms.

Thyroglossal duct cysts: variability of sonographic findings.

OBJECTIVE: The purpose of this study was to describe the sonographic features of thyroglossal duct cysts, emphasizing the variable sonographic appearances, and determine if the presence of infection or inflammation influences the sonographic appearance. MATERIALS AND METHODS: We reviewed the sonograms in 12 children (2 months-16 years old) with pathologically proven thyroglossal duct cysts (TDCs). The cysts were evaluated for location, size, cystic versus solid features, echogenicity, and wall thickness. The sonographic features then were compared with the pathologic findings. RESULTS: Sonograms of the 12 lesions showed that nine were midline at or near hyoid bone; two were to the right of midline in the submandibular region; and one was located to the left of midline in the neck. Cysts were from 0.5 cm to 4 cm in diameter. Five lesions were anechoic with no perceptible wall thickness. Seven lesions were hypoechoic. Two of the seven were homogeneously hypoechoic with thin borders. The remaining five lesions were hypoechoic but heterogeneous (complex). One lesion was hypoechoic with small anechoic spaces. Four lesions were largely cystic with dense internal echoes and thick walls. The presence of thick walls and internal echoes did not correlate with the presence or absence of inflammation on pathologic examination. No lesions in our series were hyperechoic. CONCLUSION: Our results indicate that TDCs have a variable sonographic appearance. Anechoic, homogeneously hypoechoic, and heterogeneous (complex) lesions occur. We found no correlation between the pathologic findings of infection and inflammation and the sonographic appearance of TDCs. Awareness of the variable sonographic appearance of the TDC is helpful when a midline lesion is encountered in the neck.

Wegener's granulomatosis in children: chest radiographic manifestations.

OBJECTIVE: The purpose of this study was to review the findings on chest radiographs in children with Wegener's granulomatosis. MATERIALS AND METHODS: The chest radiographs in 11 children with histologically proved Wegener's granulomatosis were retrospectively reviewed. The radiographs were evaluated for the presence of focal opacities, diffuse interstitial or alveolar opacities, nodular densities, cavitary lesions, hilar or mediastinal enlargement suggesting adenopathy, pleural fluid, and calcifications. RESULTS: Two children had normal findings on chest radiographs. One child had a focal opacity that developed into a large, thick-walled cavitary lesion, and one child had small bilateral nodules, several of which cavitated. Seven children had diffuse interstitial or alveolar opacities, five of whom had pathologically proved pulmonary hemorrhages. In one of these seven children, a thin-walled cavity subsequently developed. Pleural effusions were present in two children, one with pulmonary hemorrhage and one with pulmonary edema. No lymph node enlargement or calcifications were observed in any of our cases. CONCLUSIONS: Diffuse interstitial and alveolar opacities are the most common pulmonary findings in children with Wegener's granulomatosis, often secondary to pulmonary hemorrhage. Pulmonary nodules, with or without cavities, are less common findings in children.

Patient with double heterozygosity for achondroplasia and pseudoachondroplasia, with comments on these conditions and the relationship between pseudoachondroplasia and multiple epiphyseal dysplasia, Fairbank type.

We present a 7 1/2-year-old girl with achondroplasia and pseudoachondroplasia. Her mother has achondroplasia and her father has pseudoachondroplasia. Radiographic manifestations of these two conditions from infancy to age 6 years are outlined. The findings in this patient are compared with those of achondroplastic patients and pseudoachondroplastic patients of similar ages. Our review of radiographs of many pseudoachondroplastic patients and subsequently of those of patients with multiple epiphyseal dysplasia (MED), Fairbank type, reinforced our opinion that pseudoachondroplasia and multiple epiphyseal dysplasia, Fairbank type, are closely related conditions. MED, Fairbank type, may be the mildest form of pseudoachondroplasia. Recently published electron microscopic findings also suggest this.