Magnetic resonance imaging of the feto-placentar unit after fetoscopic laser coagulation for twin-to-twin transfusion syndrome.

Twin-to-twin transfusion syndrome (TTTS) is a severe complication in monochorionic twin pregnancies that results from a hemodynamical imbalance of placentar vascular anstomoses that connect the circulation of both fetuses. In TTTS, a poly/oligohydramnios sequence with high fetal morbidity and mortality rates occurs. Fetoscopic laser coagulation of the placentar anastomoses can limit or prevent fetal injury. The purpose of this report is to present and discuss fetal magnetic resonance imaging as a postoperative imaging tool after fetoscopic laser coagulation.

Outcome of fetal renal pelvic dilatation diagnosed during the third trimester.

OBJECTIVES: The aim of this study was to evaluate renal function and the need for postnatal treatment--antibiotic therapy and/or surgery--in relation to the grade of fetal renal pelvic dilatation (RPD) found on third-trimester ultrasound examination. METHODS: The retrospective study included 78 children, born between 1995 and 2000, with 115 dilated fetal renal pelvic units. The children were allocated to three groups based on pelvic anteroposterior diameter (APD) detected on third-trimester ultrasound: APDs of 7-9.9 mm, 10-14.9 mm and > or = 15 mm were classified as mild dilatation, moderate hydronephrosis and severe hydronephrosis, respectively. Renal function was assessed by scintigraphy. RESULTS: None of the 20 children with mild dilatation experienced a urinary tract infection (UTI) or underwent surgery; two had associated renal or urinary tract abnormalities. In contrast, five out of 22 (23%) children with moderate hydronephrosis and 23 out of 36 (64%) with severe hydronephrosis had either a UTI or required surgery (P < 0.001); associated abnormalities were also more common (6 out of 22 and 15 out of 36, respectively). There was no significant correlation between the grade of antenatal RPD and postnatal ipsilateral renal function. CONCLUSIONS: The need for postnatal treatment increased significantly with the grade of antenatal RPD. Children with antenatal mild dilatation were discharged early from follow-up whereas those with moderate and severe fetal hydronephrosis needed close follow-up by a multidisciplinary team.

Medial clavicular epiphysiolysis in children: the so-called sterno-clavicular dislocation.

We retrospectively reviewed six pediatric cases of medial clavicular injury, i.e., epiphyseal separation (Salter/Harris type I or II injury), diagnosed between 1993 and 1997. The clavicular metaphysis was displaced posteriorly in three cases and anteriorly in three. On conventional radiographic views the diagnosis was initially missed in two of three retrosternal dislocations. A special X-ray projection (described by Heinig) or computed tomography (CT) permitted correct diagnosis. Anterior dislocations were immediately and correctly diagnosed. Closed reduction successfully treated retrosternal displacement in two of the three patients. The third patient needed open reduction and internal fixation. Open reduction and internal fixation had to be performed in all three patients with anterior displacement. Follow-up assessment showed perfect functional results in all cases. Direct visualization during open reduction, which was necessary in four of six cases, yielded clear evidence that the so-called sternoclavicular dislocation in children and young adults is, in fact, a fracture of the medial growth plate with posterior or anterior displacement of the metaphysis.

[Nephro-urologic ultrasound diagnosis in the child]. / Nephro-urologische Ultrasonographie beim Kind.

The most common and characteristic abnormalities of the child's kidneys and urinary tract and the pertinent modalities of diagnostic imaging are briefly discussed. In this context, ultrasonography (US) is the basic imaging tool. Its advantages and limits are exposed. A wide spectrum of anatomic parameters can be demonstrated by US, while functional aspects must be evaluated by complementary methods in order to complete the diagnostic work-up. Voiding cystourethrography and scintigraphy are excellent means in the evaluation of the child's urinary tract and should both be performed by experts in order to combine a maximum of diagnostic information with minimal invasiveness.--Some technical aspects regarding the child's specific clinical condition and the involved imaging methods are mentioned.

Diagnostic and therapeutic impact of whole body positron emission tomography using fluorine-18-fluoro-2-deoxy-D-glucose in children with chronic granulomatous disease.

AIMS: To compare whole body positron emission tomography (PET) using fluorine-18-fluoro-2-deoxy-D-glucose (FDG) with computed tomography (CT) in detecting active infective foci in children with chronic granulomatous disease. METHODS: We performed 22 whole body FDG PET studies in seven children with X linked (n = 6) or autosomal recessive (n = 1) CGD. All had clinical signs of infection and/or were evaluated prior to bone marrow transplantation (BMT). Nineteen PET studies were also correlated with chest and/or abdominal CT. All PET scans were interpreted blinded to the CT findings. Diagnoses were confirmed histologically and bacteriologically. RESULTS: We detected 116 lesions in 22 FGD PETs and 126 lesions on 19 CTs. Only two of the latter could be classified reliably as active lesions by virtue of contrast enhancement suggesting abscess formation. PET excluded 59 lesions suspicious for active infection on CT and revealed 49 infective lesions not seen on CT. All seven active infective lesions were identified by PET, allowing targeted biopsy and identification of the infective agent followed by specific antimicrobial treatment, surgery, or subsequent BMT. CONCLUSIONS: Identification of infective organisms is more precise if active lesions are biopsied. CT does not discriminate between active and inactive lesions. Whole body FDG PET can be used to screen for active infective lesions in CGD patients.

Cerebral venous thrombosis in childhood.

This was a retrospective study to determine different etiologies of cerebral venous thrombosis (CVT) in childhood and to correlate extent and location of thrombosis with the etiology and the age of the child as well as the final outcome. In addition, the radiologic approach is discussed. This was a retrospective analysis of 19 children with CVT. The children were examined by contrast-enhanced dynamic CT. Radiologic findings were correlated with the etiology of CVT. Cerebral venous thrombosis is not as infrequent in children as has been thought. Cerebral venous thrombosis in children can occur due to trauma (n=9), infections (n=7), or coagulation disorders (n=3). Extent and location of thrombosis, as well as complications, final outcome, and therapy, depend on the etiology. Computed tomography remains a valuable primary imaging modality in the diagnosis of CVT in the acutely injured or diseased child.

Nonocclusive ischemic colitis in a 12-year-old girl: value of unenhanced spiral computed tomography.

A 12-year-old girl was hit by a car and arrived in the emergency room in hemorrhagic shock. Contrast-enhanced computed tomography of the abdomen showed traumatic rupture of the liver and large amounts of intraperitoneal hemorrhage. Unenhanced computed tomography showed a hyperdense thickening of the wall of the descending colon. This finding was consistent with a nonocclusive ischemic colitis, which was confirmed some days later by endoscopy, at a time when the patient had already developed Gram-negative bacteremia. We discuss the pathogenesis of nonocclusive ischemic colitis, computed tomography findings, and the value of unenhanced computed tomography.

Randomised controlled trial of three day versus 10 day intravenous antibiotics in acute pyelonephritis: effect on renal scarring.

BACKGROUND: Acute pyelonephritis often leaves children with permanent renal scarring. AIMS: To compare the prevalence of scarring following initial treatment with antibiotics administered intravenously for 10 or three days. METHODS: In a prospective two centre trial, 220 patients aged 3 months to 16 years with positive urine culture and acute renal lesions on initial DMSA scintigraphy, were randomly assigned to receive intravenous ceftriaxone (50 mg/kg once daily) for 10 or three days, followed by oral cefixime (4 mg/kg twice daily) to complete a 15 day course. After three months, scintigraphy was repeated in order to diagnose renal scars. RESULTS: Renal scarring developed in 33% of the 110 children in the 10 day intravenous group and 36% of the 110 children in the three day group. Children older than 1 year had more renal scarring than infants (42% (54/129) and 24% (22/91), respectively). After adjustment for age, sex, duration of fever before treatment, degree of inflammation, presence of vesicoureteric reflux, and the patients' recruitment centres, there was no significant difference between the two treatments on renal scarring. During follow up, 15 children had recurrence of urinary infection with no significant difference between the two treatment groups. CONCLUSION: In children with acute pyelonephritis, initial intravenous treatment for 10 days, compared with three days, does not significantly reduce the development of renal scarring.

Meningeal hemangiopericytoma in childhood.

Meningeal hemangiopericytoma (MHP) is extremely rare in childhood. Mean age at diagnosis is between 38 and 43 years. We present an 8-year-old boy with MHP of the middle cranial fossa. Imaging findings were indistinguishable from an aggressive bone tumor such as Ewing's sarcoma. Imaging findings are presented and discussed. Our case indicates that MHP should be considered in the differential diagnosis of skull-base tumors despite the fact that MHP is extremely rare in childhood.

Pitfalls in therapy of upside-down stomach.

The upside-down stomach (UDS) is a special form of gastric organoaxial volvulus in a supradiaphragmatic hernial sac. The authors report five cases treated between 1979 and 1998, and seek to point out possible problems and pitfalls in the surgical management of this uncommon anomaly. Retrospective analysis of these cases shows that brachyesophagus as described is not a problem in UDS, as the esophagus is of normal length. The hiatus, on the other hand, is always very large and needs proper narrowing, which may be achieved through a transabdominal approach more easily than through a transthoracic access. A common esophago-aortal hiatus is often present. In conclusion, the authors recommend a hiatal repair and gastropexy, i.e. fundophrenopexy and corpoventropexy along the esophageal axis to prevent recurrent gastric herniation or torsion. Transabdominal access should be chosen since brachyesophagus is not expected. Beware of a common hiatus when preparing the hiatal crura. An antireflux procedure is not necessary as gastro-esophageal reflux usually resolves spontaneously after hiatal repair and gastropexy.

The painful hip: evaluation of criteria for clinical decision-making.

UNLABELLED: Children with a painful hip present a diagnostic challenge since clinical differentiation between septic arthritis, transient synovitis and Perthes disease may be difficult. Septic arthritis, a potentially life-threatening and debilitating medical emergency, requires early recognition for successful treatment, while transient synovitis and Perthes disease may be managed conservatively. An "ideal" single test for discrimination between these conditions is currently not available. We assessed the value of clinical examination and simple laboratory tests together with radiography and hip ultrasound in differentiating septic arthritis from transient synovitis and Perthes disease by analyzing the records of 89 children treated at our institution for hip pain. Ultrasound, radiographs, laboratory, clinical, and follow-up data were available for all the children. Diagnoses were made according to established criteria. Transient synovitis was present in 64 patients, septic arthritis in 8 (of whom 2 had additional osteomyelitis), and Perthes disease in 4. All children with septic arthritis had hip effusion shown by ultrasound and at least two of the following criteria: fever, elevation of erythrocyte sedimentation rate (ESR) and of C-reactive protein (CRP). None of the children without effusion on ultrasound or who lacked two or all criteria had septic arthritis. Radiographs had no significant impact on the decision-making in primary evaluation of acute hip pain. CONCLUSION: We conclude that investigation of painful hips in children, based on hip ultrasound, body temperature, ESR and CRP, may allow cases for hip joint aspiration to be selected efficiently and may reduce the number of radiographs and hospital admissions.

Hepatic steatosis: a frequent non-specific finding in HIV-infected children.

Thirty HIV-infected children were cross-sectionally examined for morphologic hepatic abnormalities, using ultrasonography or histology. Abdominal ultrasonography was performed in 27 children. The liver structure was normal in four patients, one of whom had moderate symptoms of the HIV infection and three of them severe symptoms. Abnormal liver structure, compatible with hepatic steatosis, was found in 23 (85%) patients. Five of them were in an early stage of the HIV infection (category N or A), three patients were ranked in category B and 15 patients in category C. Histological examination of the liver was performed in 11 children and steatosis was documented in ten (91%). In seven (70%) of these ten children steatosis had been suspected by ultrasonography. In conclusion, steatosis is common in HIV-infected children. It is non-specific and has no impact on disease, diagnostic evaluation or management. Conclusion Ultrasonography is a sensitive, accurate, non-invasive screening tool. It is more reliable than liver function tests.

"Growing fontanelle": a serious complication of difficult vacuum extraction.

Growing skull fractures in combination with leptomeningeal cysts are well known in childhood. A rare case of a growing fontanelle due to a leptomeningeal cyst is presented. The cyst occurred due to a traumatic delivery with vacuum extraction. Operative repair of the cyst revealed a dural tear at the border of the fontanelle. The imaging findings are discussed.

Langer-Giedion syndrome associated with scimitar syndrome.

We describe an infant with Langer-Giedion syndrome combined with scimitar syndrome.

Successful treatment of invasive aspergillosis in chronic granulomatous disease by bone marrow transplantation, granulocyte colony-stimulating factor-mobilized granulocytes, and liposomal amphotericin-B.

X-linked chronic granulomatous disease (X-CGD) is a primary immunodeficiency with complete absence or malfunction of the nicotinamide adenine dinucleotide phosphate (NADPH) oxidase in the phagocytic cells. Life-threatening infections especially with aspergillus are common despite optimal antimicrobial therapy. Bone marrow transplantation (BMT) is contraindicated during invasive aspergillosis in any disease setting. We report an 8-year-old patient with CGD who underwent HLA-genoidentical BMT during invasive multifocal aspergillus nidulans infection, nonresponsive to treatment with amphotericin-B and gamma-interferon. During the first 10 days post-BMT, the patient received granulocyte colony-stimulating factor (G-CSF)-mobilized, 25 Gy irradiated granulocytes from healthy volunteers plus G-CSF beginning on day 3 to prolong the viability of the transfused granulocytes. This was confirmed in vitro by apoptosis assays and in vivo by finding nitroblue tetrazolium (NBT)-positive granulocytes in peripheral blood 12 and 36 hours after the transfusions. Clinical and biological signs of infection began to disappear on day 7 post-BMT. Positron emission tomography with F18-fluorodeoxyglucose (FDG-PET) and computed tomography (CT) scans at 3 months post-BMT showed complete disappearance of infectious foci. At 2 years post-BMT, the patient is well with full immune reconstitution and no sign of aspergillus infection. Our results show that HLA-identical BMT may be successful during invasive, noncontrollable aspergillus infection, provided that supportive therapy is optimal.

Fibrous tumours in children: imaging features of a heterogeneous group of disorders.

BACKGROUND: Fibrous tumours are predominantly soft tissue lesions which are relatively frequent in childhood but are little known. Imaging is often used in the evaluation of these tumours but their characteristics, particularly on US or MRI, have not been studied systematically. OBJECTIVES: To provide an overview of the clinical and imaging features of the different disorders, and to correlate them with the currently used classification schemes. MATERIAL AND METHODS: Twenty-five patients with fibrous tumours were evaluated retrospectively. Clinical histories were studied for the histopathological diagnosis, age, signs and symptoms at presentation, mode of therapy and follow-up where available. Imaging findings were analysed for the following variables: number, location, size, margin and architecture of soft tissue and/or visceral lesions and the presence and pattern of osseous involvement. Comparison with the available literature was performed. RESULTS: The following tumour types were encountered: desmoid fibromatosis (n = 9), myofibromatosis (n = 7), fibromatosis colli (n = 2), congenital-infantile fibrosarcoma (n = 2), adult-type fibrosarcoma (n = 2), fibrous hamartoma of infancy (n = 1), angiofibroma (n = 1) and hyaline fibromatosis (n = 1). CONCLUSIONS: While some tumours were non-specific in their clinical and radiological manifestation, others such as myofibromatosis, fibromatosis colli, fibrous hamartoma of infancy and angiofibroma exhibited a characteristic pattern which allowed a diagnosis to be made even without histology.

Cystic nephroma: a rare benign renal tumor.

We report two boys aged 1 year and 2 years 2 months, respectively, with cystic nephromas. Both presented with a painless abdominal mass. Computed tomography showed a homogeneous, multicystic tumor of the lower pole of the kidney in both cases with thin septa without solid parts. Macroscopically, the surface of the tumor was smooth. Both patients underwent a renal-sparing procedure; histology confirmed the diagnosis of cystic nephroma.

Hypergalactosaemia in a newborn: self-limiting intrahepatic portosystemic venous shunt.

UNLABELLED: Hypergalactosaemia was found in 4 day-old boy during newborn screening. He had no enzyme deficiency but an intrahepatic vascular malformation permitting significant portosystemic venous shunting. The shunt caused hyperammonaemia, accentuated after meals, alimentary hyperglycaemia and hypergalactosaemia, and excess excretion of lactic, 3-hydroxy butyric and other organic acids in urine. Portal venous flow was unimpaired. The vascular anomaly regressed during the first 7 months of life. At this age, full tolerance to lactose-containing cows milk formula was evidenced by the normalization of pre- and postprandial blood glucose, ammonia and galactose, and closure of the shunt was confirmed by ultrasonography. This is one of the few observations of congenital intrahepatic venous shunt regressing spontaneously during infancy. CONCLUSION: A congenital intrahepatic portosystemic venous shunt can cause hypergalactosaemia in the newborn and hyperammonaemia in the small infant. The malformation may resolve spontaneously obviating the need for intervention.