Hepatic involvement in congenital cytomegalovirus infection - infrequent yet significant.

Congenital cytomegalovirus (cCMV) infection can reside in many organ systems; however, the virus has a particular predilection towards inhabiting the reticuloendothelial system, especially the liver. Specific studies focusing only on hepatic involvement in infants with cCMV are lacking. We report our experience with a large cohort of infants treated in our hospital clinic due to cCMV and hepatic involvement. Hepatic involvement was defined either as hepatitis (elevated alanine transaminases (ALT) >80 units/L without cholestatic disease) or cholestatic disease (elevated ALT >80 units/L combined with direct bilirubin >2 mg/dL). During the study period, 198 infants were diagnosed with symptomatic cCMV in our clinic. Hepatic involvement was observed in 13 infants (6.6%); 7 (3.5%) with hepatitis and 6 (3%) with cholestatic disease. Maternal primary infection with cytomegalovirus during pregnancy was diagnosed in 7 (53.8%) of the 13 infants, nonprimary in 3 (23.1%) and unknown in 3 (23.1%). Among these 13 infants, central nervous system (CNS) involvement was observed in 11 (84.6%) and hearing impairment in 7 (53.8%). Treatment with an antiviral agent was initiated in all cases. Gradual improvement of hepatic enzymes and cholestasis was observed over a prolonged period. We found that the incidence of hepatic involvement in infants with cCMV is much less frequent than previously reported. The hepatic involvement in these infants may manifest in two different ways, and thus, a high index of suspicion and a stepwise approach will help in correctly diagnosing these infants. Antiviral treatment due to CNS involvement is warranted and prognosis is excellent.

Functional hyposplenism is an important and underdiagnosed immunodeficiency condition in children.

AIM: Few studies have focused on paediatric hyposplenism/asplenism, in which splenic phagocytic activity is diminished or absent in an anatomically present spleen. This study aimed to evaluate clinical findings, laboratory tests and prognosis of children with functional hyposplenism/asplenism. METHODS: The study group comprised 74 children who had liver/spleen technetium-99m sulphur colloid scintigraphy from 2002 to 2008. Information collected included demographic features, background diseases, blood smear findings, indications for scintigraphy and outcome. Children with functional hyposplenism were followed until 2012. RESULTS: We found that 34 patients had functional hyposplenism/asplenism. The main indications for scintigraphy in the hyposplenic patients were persistent thrombocytosis and recurrent infections. Associated conditions included immunodeficiencies, autoimmune diseases, malignancies and genetic disorders. Main infections were sinopulmonary infections, bacteraemia and sepsis. The major pathogens were Streptococcus pneumoniae and Haemophilus influenza group A. There was no correlation between the presence of Howell-Jolly bodies in blood smear with clinical disease severity or scintigraphic findings. Repeated scintigraphy showed spontaneous normalisation in 40% of patients. CONCLUSION: Functional hyposplenism is an important and underdiagnosed immunodeficiency condition in children, associated with various clinical conditions including prolonged unexplained thrombocytosis, immune deficiency and autoimmunity. Technetium-99m sulphur colloid scintigraphy is the method of choice for evaluating splenic function.

Technetium-99m-HMPAO SPECT in Sturge-Weber syndrome.

UNLABELLED: Technetium-99m-HMPAO interictal SPECT was performed on three pediatric patients with Sturge-Weber syndrome (SWS). RESULTS: Brain SPECT of all three patients demonstrated markedly diminished tracer localization in the affected hemisphere. In one patient, the SPECT abnormality was more extensive than the associated abnormalities on CT and MRI. CONCLUSION: Technetium-99m-HMPAO brain SPECT can detect cerebral perfusion abnormalities associated with SWS and deserves consideration in the imaging evaluation of SWS patients.

The prognostic value of perfusion lung scintigraphy in patients who underwent single-lung transplantation for emphysema and pulmonary fibrosis.

UNLABELLED: The objective of this study was to evaluate the role of quantitative perfusion lung scintigraphy (QPLS) in predicting the development of chronic rejection in patients who underwent single-lung transplantation. METHODS: Eighteen patients (15 men, 3 women; age range, 41-60 y; mean age, 54.6+/-6.0 y) who underwent single-lung transplantation for emphysema (n = 14) or pulmonary fibrosis (n = 4) were studied. Patients were evaluated using QPLS and pulmonary function tests before surgery and at 1-3 mo and 1-3 y after transplantation. Relative perfusion of the transplanted lung was calculated from standard perfusion lung scintigrams. Values for forced expiratory volume in the first second (FEV1) were obtained from lung function tests at the same time points. The maximal and minimal relative perfusion and FEV1 values in the early (1-3 mo) and late (1-3 y) follow-up periods were identified for each patient. Receiver operating curve (ROC) analysis was performed on all parameters. RESULTS: In total, 82 lung scans were performed (mean, 4.8+/-1.55 per patient) and 484 FEV1 test determinations were obtained (mean, 30.0+/-15.6 per patient) during a follow-up period ranging from 8 to 84 mo (mean, 39.6+/-19.3 mo). In 7 of the 18 patients, chronic rejection developed, whereas 11 patients had a favorable outcome. No significant difference was found in the number of complications (acute rejection and infection episodes) between patients with a favorable outcome and patients with chronic rejection, up to 1 y after transplantation. At 1-3 mo, minimal relative perfusion values were 67.1%+/-12.2% in the favorable outcome group and 50.8%+/-9.6% in the chronic rejection group (P = 0.02). Before surgery and at 1-3 y, minimal relative perfusion was not significantly different between the 2 groups. The difference in maximal relative perfusion at 1-3 y was marginally significant, with 76.5%+/-8.9% in the favorable group and 64.3%+/-15.0% in the chronic rejection group (P = 0.051). FEV1 values were not significantly different preoperatively and 1-3 mo after surgery between the chronic rejection and the favorable outcome groups. Late in the follow-up period (1-3 y), FEV1 values in the chronic rejection and favorable outcome groups were 35.6%+/-7.9% and 56.9%+/-13.6%, respectively (P = 0.002). ROC analysis of minimal relative perfusion at 1-3 mo identified a threshold of 57% under which the sensitivity and specificity for chronic rejection were 83% and 88%, respectively. Minimal FEV1 for the same period identified a threshold of 48% under which the sensitivity and the specificity were 80% and 67%, respectively. CONCLUSION: QPLS early after transplantation in our patients predicted the development of chronic rejection in patients with single-lung transplantation for emphysema and pulmonary fibrosis, whereas surgical complications, acute rejection, infection episodes, and lung function tests did not predict the outcome. This early prediction could not be obtained from lung function tests performed at the same time.

Total parenteral nutrition-associated cholestasis after selective damage to acinar zone 3 hepatocytes by bromobenzene in the rat.

Total parenteral nutrition is known to cause cholestasis, but the hepatic site of this effect has not been determined. The purpose of our study was to observe the effect of TPN on bile flow and bile salt secretion rate in rats after selective damage to acinar zone 3. Bromobenzene, 3.8 mmol/kg, was injected i.p., and the animals were studied 48 hours later. Experimental groups received either parenteral nutrition or saline for 2 hours. Bromobenzene caused selective damage to acinar zone 3 hepatocytes, and reduced baseline bile flow (23.99 +/- 1.09 vs 37.2 +/- 1.66, mean +/- SEM, microliter/min/kg, p < 0.001). Bromobenzene had no effect on bile salt secretion rate. Total parenteral nutrition decreased bile flow in the bromobenzene treated groups, despite the selective hepatic damage to acinar zone 3 (20.54 +/- 1.07 vs 23.28 +/- 1.63, mean +/- SEM, p < 0.001). Total parenteral nutrition reduced bile salt secretion rate in healthy animals, but this reduction was not seen in bromobenzene treated rats. Our results suggest that bile flow reduction in response to total parenteral nutrition is mediated through an effect on acinar zones 1 and 2, as this reduction is still observed after zone 3 destruction by bromobenzene. Zone 3 hepatocytes may be involved in the effect of parenteral nutrition on bile salt secretion, as the reduction in secretion rate seen in healthy animals was not observed in bromobenzene treated rats.

Streptococcus acidominimus infection in a child causing Gradenigo syndrome.

UNLABELLED: Gradenigo syndrome is a rare presentation of acute petrositis. The clinical triad of Gradenigo syndrome consists of acute suppurative otitis media, severe unilateral headache and abducens nerve palsy. We report the first case of Gradenigo syndrome caused by Streptococcus acidominimus, a Gram-positive coccus of the Streptococcus viridans group, which rarely causes deep-seated infection in humans. CONCLUSION: Gradenigo syndrome may complicate acute otitis media and should be suspected in case of unilateral headache and abducens nerve palsy. Conservative medical treatment without surgery may be considered in some patients.

Scintigraphic evaluation of calcinosis in juvenile dermatomyositis with Tc-99m MDP.

Two children with juvenile dermatomyositis and extensive, debilitating soft tissue calcifications are described. Whole-body bone scans with Tc-99m MDP were performed in both cases before and during specific treatment for the calcinosis. Baseline studies showed marked tracer localization in the soft tissues and provided an objective baseline assessment of the extent of the soft tissue calcifications. Follow-up studies showed gradual clearance of the extraskeletal uptake and were useful in monitoring the therapeutic response. These cases show that skeletal scintigraphy can function as a useful auxiliary tool to evaluate calcinosis in children with juvenile dermatomyositis.

Scintigraphy of lower extremity cadaveric bone allografts in osteosarcoma patients.

PURPOSE: To describe scintigraphic characteristics of bone allografts used in limb salvage reconstruction after resection of lower extremity osteosarcoma. MATERIALS AND METHODS: The authors reviewed 85 skeletal scintigrams of 20 pediatric patients followed up for 0.5-5.7 years after resection of lower extremity osteosarcoma and allograft reconstruction. Uptake in the allograft and adjacent host tissues was assessed visually. RESULTS: Lack of tracer uptake in the allografts was seen in 99% of the studies and a faint rim of tracer localization outlining the allograft's periphery was seen in 95% of the studies. Increased uptake was noted at the allograft-host bone junction in 78% of the studies. Uptake was increased in the joint surfaces of native bones articulating with allografts (97% of studies), including the patella (93% of studies) when the knee was involved. These findings were stabilized as time passed. CONCLUSION: Cadaveric bone allografts have a characteristic scintigraphic appearance in this selected patient group that reflects the physiology of their incorporation process.

Tc-99m MIBI to evaluate children with Ewing's sarcoma.

PURPOSE: Tc-99m MIBI has been used increasingly to evaluate benign and malignant tumors because of its tumor-seeking properties and ability to provide an imaging assessment of multiple-drug resistance. This study investigated the clinical utility of Tc-99m MIBI in the management of Ewing's sarcoma in children. METHODS: Thirteen Tc-99m MIBI studies in nine (six male, three female) patients ages 6.5 to 20 years (mean, 13.4 years) with Ewing's sarcoma were reviewed. All patients had imaging studies at diagnosis, and four had follow-up studies during or after therapy. Scintigraphy was evaluated for Tc-99m MIBI uptake within the tumor and in metastases, which other imaging modalities had shown to be present in four patients. Scintigraphic results were correlated with the clinical course in all patients and with tumor P-glycoprotein status in six patients. RESULTS: Tc-99m MIBI accumulated in 6 of 9 primary tumors and did not accumulate in one recurrent tumor. No metastases showed Tc-99m MIBI uptake. The presence or absence of Tc-99m MIBI uptake at diagnosis or after therapy carried no prognostic significance. Tc-99m MIBI was present in the two tumors that were P-glycoprotein positive and in only one of four tumors that were P-glycoprotein negative. CONCLUSION: Tc-99m MIBI imaging does not appear to be useful in Ewing's sarcoma.

The radionuclide salivagram in children with pulmonary disease and a high risk of aspiration.

Purpose. We wanted to demonstrate the utility of the radionuclide salivagram in assessing children whose underlying medical problems suggest that aspiration of oral contents is a factor contributing to otherwise unexplained lung disease or recurrent pneumonias. Materials and methods. Following sublingual administration of 300 μCi (11.1 MBq) of technetium-99m sulfur colloid in a drop of saline, serial images were obtained for 60 min and evaluated for entrance of tracer into the major airways and lung parenchyma. Thirty-four studies were performed on 31 patients (age: 3 weeks - 16.7 years, mean 5.5 years) with recurrent pneumonias or unexplained lung disease. All had medical conditions, most frequently neurological, predisposing to aspiration. Results. Pulmonary aspiration was detected in eight patients. All positive studies demonstrated aspirated tracer distal to the tracheal bifurcation, either in bronchi or in bronchi and lung parenchyma. Aspiration was bilateral in six cases. Clearance of aspirated tracer was noted in one patient. Conclusion. The salivagram is an effective tool for demonstrating aspiration of oral contents and the level to which aspirated fluid penetrates the tracheobronchial tree. Additionally, by allowing for the evaluation of clearance of aspirated tracer, the salivagram may provide information on the status of airway-protective mechanisms.

Radial osteomyelitis as a complication of venous cannulation.

Venepuncture of the superficial veins in the forearm is considered a relatively safe procedure. We report two patients who presented with osteomyelitis of the proximal radius following venous cannulation of the median cubital vein, and one patient who developed osteomyelitis of the distal radius after cannulation of the cephalic vein. Osteomyelitis developing in proximity to a venepuncture site should raise the suspicion that a pathogen causing superficial thrombophlebitis has spread through the deep veins of the arm into the adjacent bone, thus causing osteomyelitis.

Thallium-201 uptake in Langerhans cell histiocytosis of bone.

A case in which there was thallium-201 (201Tl) uptake in Langerhans cell histiocytosis (LCH) of the left femoral diaphysis is presented. The authors propose that 201Tl scintigraphy is potentially useful in the diagnosis and follow-up of patients with LCH.

A rare coexistence of a multicentric hepatic hemangioendothelioma with a large brain hemangioma in a preterm infant.

A preterm infant with a rare association of a multicentric hepatic hemangioendothelioma (MHH) and a large brain hemangioma is described. The imaging investigations and their findings are discussed. It is recommended that whole-body radionuclide imaging with 99mTc-labeled red blood cells is employed immediately following ultrasonography to confirm the suspected diagnosis of MHH and to detect other unsuspected hemangiomas.

Pediatric skeletal scintigraphy: applications of pinhole magnification.

Pinhole magnification scintigraphy is an effective means of evaluating the pediatric skeleton because it provides optimal high-resolution images. This technique is indicated when diagnostic uncertainty persists after high-resolution imaging with parallel hole collimation. Pinhole magnification scintigraphy requires approximately 20 minutes of acquisition time per image and meticulous attention to details such as choice of pinhole insert, collimator positioning, and patient immobilization. However, the technique is superior to planar imaging in demonstrating acute osteomyelitis in bone adjacent to growth centers and epiphyseal involvement that is either primary or the result of local spread of infection. In addition, pinhole imaging has proved highly reliable in the early diagnosis of Legg-Calvé-Perthes disease and is useful in depicting osteonecrosis related to specific causes such as corticosteroid treatment or trauma. Scintigraphic manifestations of femoral head ischemia or infarction and findings indicative of osteomyelitis associated with a hip effusion are well demonstrated with pinhole imaging. This technique also helps characterize osteoid osteomas and may be used intraoperatively to confirm the complete excision of this benign tumor. Finally, pinhole magnification scintigraphy clearly depicts fractures of the femoral neck and allows a high degree of confidence in diagnosing injuries to the small bones of the hands and feet.