Persistent localized pulmonary interstitial emphysema and lymphangiectasia: a causal relationship?

The mechanism responsible for persistent regional pulmonary interstitial emphysema under tension, which occasionally complicates assisted ventilation, has not been explained. It is difficult to comprehend how interstitial air may cause lobar hyperinflation and not escape to the mediastinum or pleura. Histologic examination of resected emphysematous lung in two neonates with this type of interstitial emphysema revealed air dissecting from the interstitial connective tissue into markedly dilated lymphatics. It is proposed that intralymphatic air dissection causing emphysematous lymphangiectasia may be responsible for fixation of the lung in the emphysematous state.

Urinary basic fibroblast growth factor: a noninvasive marker of progressive cystic renal disease in a child.

Autosomal recessive polycystic kidney disease (ARPKD) is a hereditary condition with an estimated incidence of 1 in 20,000 live births. Various growth factors have been implicated in the causation of this disease. We describe a child with ARPKD whose levels of urinary basic fibroblast growth factor (bFGF) were markedly elevated. The concentrations of bFGF increased further following right nephrectomy, in response to the compensatory growth of the remaining kidney. We hypothesize that measurement of urinary bFGF may be useful as a noninvasive marker to assess progression of cystic renal development.

Incidence, management, and outcome of childhood empyema: a prospective study of children in Cambodian refugee camps.

To determine the incidence, outcome, and optimal management of empyema, all children less than 15 years of age admitted to Khao-I-Dang Hospital with a diagnosis of empyema during a 23-month period were prospectively studied. Khao-I-Dang Hospital provides care to 137,000 Cambodian children residing in eight refugee camps along the Thai-Cambodian border. Ninety-eight children with empyema were identified, for an annual incidence of 0.37 cases per 1,000 children. All patients had chest tubes inserted on admission, and all were treated with parenteral antibiotics, which included chloramphenicol in 92% of the patients and cloxacillin in 72%. Patients were hospitalized a mean of 30 days, and chest tubes were in place for a mean of 12 days. Surgery was performed on four patients who had bronchopleural fistulas that persisted for more than 14 days. Only one (1%) of the 70 patients treated with cloxacillin required thoracotomy, compared with three (11%) of the 28 patients who did not receive cloxacillin (P = 0.07). In a multiple regression analysis, the presence of pneumatoceles or mediastinal shift on admission chest radiograph, a history of tuberculosis in the family, and an age of more than five years were predictive of a longer duration of chest tube drainage. No patient died in the hospital, and only one patient died in the six months following discharge from the hospital. Chest radiographs that were obtained six months after discharge in 25 patients were all essentially normal, despite marked abnormalities on chest radiographs obtained at discharge. In summary, conservative medical management with the use of chest tubes for these 98 children with empyema resulted in a mortality rate of 1.0%, and should be considered as an effective alternative to the surgical management of patients presenting with this complication.

Bronchogenic cyst.

Two recent patients with bronchogenic cysts, presenting in an atypical manner, stimulated our review of this subject. Twenty patients with bronchogenic cysts have been treated at the Boston Floating Hospital over the past 20 yr. Of these patients, 19 out of 20 were symptomatic, the most common symptom being fever (6 out of 20). Half of our patients had no respiratory symptoms but only one was found to have an asymptomatic mass on chest x-ray. The majority of theses cysts were found within the pulmonary parenchyma, the right lung being affected three times more commonly than the left. These facts are at odds with the reports in the literature. Three were found in the neck. Bronchogenic cysts are generally thought to be small, solitary, and limited in area. However, a significant number in our series (8 out of 20) were large, multiple or multicystic, and involved segments, lobes, multiple lobes, and, on one occasion, an entire lung. The correct pre-operative diagnosis was made in only 30% of the cases. The fact that the diagnosis was often missed led to long delays in treatment resulting in recurrent infectious complications and repeated hospitalizations. The most common cause of error in diagnosis was failure to follow a pneumonia to complete resolution. Despite the frequent delays in diagnosis, surgical treatment was curative in all patients.

Characterization of gold electrodes in phosphate buffered saline solution by impedance and noise measurements for biological applications.

Impedance spectroscopy and noise measurements have been used to study gold electrodes of three different surface areas in phosphate buffered saline (PBS) solution. The gold-PBS solution interface can be modeled by a charge transfer resistance in parallel with an interfacial constant phase element (CPE) which are in series with the solution resistance. The voltage noise fluctuations were analyzed using the fast Fourier transform (FFT) method. It is found that the voltage noise power is characterized by a 1/f(alpha) spectrum in the low frequency range. The value of alpha is observed to be double that of the CPE coefficient beta. The authors suggest a link between the interface impedance and the measured noise.

Osteochondritis of the tarsal navicula: radioisotopic appearances.

A case of Köhler's disease of the tarsal navicula is described as presenting with swelling, tenderness, and hyperthermia of the right foot. Scintigraphy revealed decreased uptake in the right midtarsal area. The ability of the pinhole collimator to image these tiny bones is demonstrated.

Juvenile gastrointestinal polyposis.

Two cases of juvenile gastrointestinal polyposis are described and the literature is reviewed. Despite the benign nature of the individual polyps, the disease has a poor prognosis. Of 13 reported patients, 4 had macrocephaly, and one of the authors' 2 patients had large peripheral intracranial cysts. This appears to be the first reported documentation of the etiology of macrocephaly associated with juvenile polyposis. Major clinical problems are related to severe loss of blood and protein from the gastrointestinal tract.

Diaphragmatic paralysis evaluated by phrenic nerve stimulation during fluoroscopy or real-time ultrasound.

Stimulation of the phrenic nerve by applying an electrical impulse to the neck during fluoroscopy or real-time ultrasound (sonoscopy) of the diaphragm allows more precise functional evaluation than fluoroscopy and/or sonoscopy alone. This is especially true of patients who are unable to cooperate because they are on a ventilator, unconscious, or very young. The authors cite cases in which diaphragmatic paralysis was diagnosed by conventional methods but stimulation of the phrenic nerve demonstrated good diaphragmatic motion, leading to a change in prognosis in some cases and a change in therapy in others.

Hypertrophic bursopathy of the subacromial-subdeltoid bursa in juvenile rheumatoid arthritis: sonographic appearance.

Hypertrophic bursopathy is a term used to describe the massive synovial proliferation occasionally seen in the bursae of patients with arthritis. Involvement of the subacromial-subdeltoid (SA-SD) bursa in adults in uncommon, and it is still rarer in children. It may simulate synovial proliferation or fluid within the adjacent shoulder joint. Sonography clearly shows the location and nature of the soft-tissue swelling. Two cases of this entity in children with juvenile rheumatoid arthritis are described, one with a uniquely severe sonographic picture.

"Step-off" vertebral body: Gaucher's disease versus sickle cell hemoglobinopathy.

Two patients with Gaucher's disease and radiographic findings in the spine similar to sickle cell hemoglobinopathy are reported, making a total of four known patients with Gaucher's disease who have these steplike vertebral endplate deformities. Serial films of a patient having Gaucher's disease compared with patients having sickle cell hemoglobinopathy suggest that different events lead to formation of the vertebral deformities. In Gaucher's disease, there is an initial collapse of the entire vertebral body with subsequent growth recovery peripherally. In sickle cell hemoglobinopathy, the endplate deformity is nontraumatic and is caused by an underlying growth arrest.

Blue rubber bleb nevus syndrome.

Two cases of pediatric blue rubber bleb nevus syndrome are reported. The main features of the disease are rubbery blue cutaneous nevi and hemangiomatous, frequently hemorrhagic malformations of the bowel wall. Polypoid filling defects were seen throughout the bowel on barium studies. This entity should be considered in the differential diagnosis of polyposis, and the skin should be carefully examined for nevi when multiple bowel polyps are demonstrated, especially in patients with gastrointestinal bleeding.

Air embolism as a complication of hip arthrography.

As an aid to correctly placing the needle tip within the hip joint during arthrography, injection of small amounts of air has been used to outline the joint space. Over a period of seven years, air embolus has been noted in three pediatric patients, twice with minor symptoms, and once with cardiac arrest. Even small amounts of air (less than 5 cc) injected accidentally intravenously may cause dangerous complications in small infants. If this technique is to be used, it is recommended that small amounts of carbon dioxide or oxygen be used instead of air.

Referred pain in the lower leg -- a cause of delayed diagnosis.

Three patients presenting with ankle pain are described. In each case the pain was referred from a lesion in the proximal tibia (two osteoid osteomas; one stress fracture). There was considerable delay in diagnosis in two of these cases. It would appear that pain referred from the proximal lower leg to the ankle is not well recognized as a clinical entity. In such cases, radiography of the more proximal limb is suggested. If this proves negative, a bone scan may then be performed.

Pediatric radiologists in the United States and Canada: involvement with newer imaging modalities.

A survey of the members of the Society for Pediatric Radiology indicates that currently the majority of pediatric radiologists do not perform or interpret examinations using radionuclides, ultrasound, or computed tomography (CT). A decisive factor determining involvement with these techniques is the type of institution in which the radiologists practices. Most of those involved with newer imaging modalities practice in autonomous children's hospitals, which also have the highest concentration of subspecialists. However, equipment is not always available in children's hospitals. Those practicing in major academic centers do not usually perform or interpret such imaging studies despite the widespread availability of equipment. The few pediatric radiologists in community hospitals almost always have equipment available and are involved with newer imaging techniques.

Milk of calcium fluid collection in dermatomyositis: ultrasound findings.

Extensive subcutaneous and intermuscular calcium laden fluid collections (milk of calcium) were found in two young girls with severe dermatomyositis. Sonographic examination clearly showed the nature and extent of the collections. Knowledge of this new feature of dermatomyositis should help avoid confusing these fluid collections with soft tissue infection and deep abscess in these often steroid dependent children.

Ultrasonography in the study of prevalence and clinical evolution of popliteal cysts in children with knee effusions.

The prevalence and clinical evolution of popliteal cysts in children with knee arthritis is not well known. Using ultrasonography, we studied 44 children with clinically detectable knee effusions secondary to juvenile rheumatoid arthritis (n = 35), spondyloarthritis (n = 3) and psoriatic (n = 2), septic (n = 2) and lupus (n = 2) associated arthritis. Popliteal cysts, defined as anechoic or hypoechoic masses measuring at least 1 cm in 2 of 3 dimensions, were identified in 27 children (61%). Of the 30 children with bilateral arthritis, 11 (37%) had bilateral cysts. The size of the cysts ranged from 1 to 40 cm3 (median 3.0 cm3). There was a significant correlation between the presence of a cyst and popliteal pain and the size of the suprapatellar effusion (p less than 0.001) but not the child's age or underlying diagnosis (p greater than 0.05). A cohort of 25/27 children with cysts were followed prospectively with serial sonograms for 18-24 months. The resolution of the cyst followed that of the suprapatellar effusion in those children whose arthritis improved or resolved. Two children (8%) had rupture of the popliteal cysts. Popliteal cysts are readily documented in children with knee effusions using ultrasonography, and their presence and evolution correlates with the size of the suprapatellar effusion.

Multicystic dysplasia and crossed renal ectopia.

Multicystic renal dysplasia in a crossed fused or nonfused ectopic kidney is a rare occurrence that produces unusual imaging findings. In four such cases in neonates, three presented with a palpable abdominal mass and one was discovered in utero with maternal sonography. In three cases, sonography showed a multicystic mass, contiguous with the lower pole of the orthotopic kidney. In the fourth infant, a crossed, tiny dysplastic kidney was not visualized, but its markedly dilated ectopic ureter was seen traversing the spine on CT. Ureteropelvic junction obstruction was present in the three fused orthotopic kidneys. The characteristic imaging findings of multicystic dysplasia and crossed renal ectopia include a multicystic mass of variable size that is contiguous with the lower pole of a hydronephrotic, malrotated kidney; ureteral displacement and/or dilatation; and contralateral absence of the kidney and its renal artery.

The one-film urogram in urinary tract infection in children.

The sensitivity and specificity of the 5 min postinjection radiograph during urography were studied in 131 children. The 5 min film was overall accurate in 83.2%, with a 56% sensitivity and 91.3% specificity in patients with urinary tract infection (n = 62). In cases in which the 5 min film was not diagnostic, it nevertheless raised enough suspicions to dictate additional films. It is suggested that in urinary tract infection, errors will not be made if the single 5 min postinjection film is reviewed and additional films are obtained only if parts of the urinary tract are incompletely visualized or appear questionable. Further study is necessary to explore the merits of a similar approach for pediatric urography unrelated to urinary tract infection.

Extrarenal Wilms' tumor.

Extrarenal Wilms' tumor is a rare entity usually seen as a mass in the retroperitoneal area. It may surround and distort otherwise normal kidneys and ureters. Like many other abdominal masses, its true nature remains uncertain until microscopic examination has been performed after surgery. The tumors appear to behave clinically like intrarenal Wilms' tumors, though in the cases reviewed there was a relatively higher incidence in older patients. The prognosis of extrarenal Wilms' tumor is difficult to evaluate due to the small numbers of cases reported and the differing tumor therapy used. However, those cases treated most recently appear to have a good response to therapy.