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Biomedical subjects

F Gudinchet

Publications and source records attributed to F Gudinchet.

At least 19 recordsLinked to original sources

Three-dimensional cholangio-spiral CT demonstration of a post-traumatic bile leak in a child.

Bilioma is a rare complication of traumatic liver injury, and the precise site of bile leak is often difficult to demonstrate with a non-invasive technique. We report a case of post-traumatic bile leak in a 15-year-old girl in whom spiral CT after intravenous cholangiography allowed excellent preoperative demonstration of the extent of the liver rupture and an exact location of the bile leak. We think that spiral-CT cholangiography could be an accurate, non-invasive technique to investigate the biliary system in cases of paediatric liver trauma.

Adolescent

Pulmonary oedema associated with mitral regurgitation: prevalence of predominant right upper lobe involvement in children.

OBJECTIVE: To evaluate the hypothesis that pulmonary venous congestion and oedema manifested predominantly in the right upper lobe in children with mitral regurgitation occur more frequently than previously thought. MATERIALS AND METHODS: Three radiologists retrospectively and independently reviewed the plain chest radiographs of 54 children (26 girls, 28 boys, age range 2 days-18 years, median 9.5 years) with mitral regurgitation admitted to our institution during a 5-year period. Radiographs showing pulmonary venous congestion and oedema manifested predominantly in the right upper lobe were identified. Clinical records of these patients were studied to exclude other causes of pulmonary disease. RESULTS: Radiographic signs of pulmonary venous congestion and oedema were present in all patients, with redistribution of flow and interstitial oedema in 39 patients (72 %) and alveolar oedema in 15 children (28 %). In 12 (22 %) of 54 children, these findings were localised or predominant in the upper lobe of the right lung; none of the children had predominantly left-sided involvement. CONCLUSIONS: Pulmonary venous congestion and oedema involving predominantly the upper lobe of the right lung in children with mitral regurgitation occur more frequently than previously thought. This finding is useful in the differential diagnosis of right upper lobe abnormalities, such as pneumonia.

Adolescent

Color Doppler ultrasound for evaluation of collagen implants after endoscopic injection treatment of refluxing ureters in children.

To determine whether color Doppler ultrasonography (CDUS) would add to the understanding of the bladder wall after endoscopic subureteral collagen injection (SCIN), 25 patients were studied with CDUS. The kidney length and echogenicity, the volume of the collagen, the mean length of the urinary jet, the longitudinal and transverse angles, and the distance between the origin of the jet and the midline of the bladder were measured 1 day, 1 month, and 3 months after SCIN and compared to the results of micturating cystourethrograms (MCUs) performed 3 months after SCIN. The collagen was hyperechogenic compared to the bladder wall at the time of injection, and was isoechogenic 1 and 3 months after SCIN. CDUS showed the relationship between the injected collagen and the position of the ureteral orifice. The measurements of jet length, angle, and distance of the ureteral orifice from the midline did not correlate with vesicoureteral reflux assessed by MCU. Although CDUS may demonstrate the location, the size of the injected collagen implant, and its relationship to the ureteral orifice, it is as yet unable to predict vesicoureteral reflux after SCIN.

Adolescent

Congenital ureteroceles: an indication for screening?

In the pediatric population, ureteroceles may present with different clinical pictures, including obstruction and lithiasis. We report two pairs of twins; one of each set had ureteroceles, and the other a related urinary tract malformation (ureterocele, polycystic kidney disease). These cases raise the question of whether the siblings of children with ureteroceles should be screened for urogenital abnormalities.

Child

Magnetic resonance detection of myelodysplasia in children with Currarino triad.

PURPOSE: To evaluate the role of MRI in the detection of myelodysplasia in children with Currarino triad. MATERIALS AND METHODS: Six patients (two girls, four boys, aged 7 months-14 years, mean age 6 years) were studied with MRI, voiding cystourethrogram and barium enema or fistulography. CT and ultrasonography were also performed in two patients. RESULTS: All patients presented with partial agenesis of the sacrum. Three patients suffered from an intermediate form of anorectal malformation (ARM) and three had a high form of ARM. The presacral masses consistent with Currarino triad included anterior meningocoele in three patients, lipoma in two patients and anterior lipomeningocoele in one patient. MRI diagnosed tethering of the spinal cord in four of six patients. The tethering of the spinal cord was due to a lipomeningocoele in one patient, an intradural lipoma in one patient and a lipoma of the filum in two patients. CONCLUSION: The association of Currarino triad with tethered spinal cord seems more common than generally reported in the literature. Preoperative MRI of the lumbosacral spine is essential to detect significant myelodysplasia in all patients with Currarino triad.

Adolescent

Lemierre's syndrome in children: high-resolution CT and color Doppler sonography patterns.

Lemierre's syndrome is an anaerobic sepsis occurring after oropharyngeal infection in healthy teenagers and young adults. We report two cases of adolescent girls suffering from Lemierre's syndrome studied with cervical color Doppler ultrasonography (CDUS), cervicothoracic helical CT, and high-resolution CT (HRCT) scanning. In both patients, HRCT allowed a good depiction of multiple cavitated pulmonary nodules of various sizes suggestive of this entity and was able to detect small or peripheral nodules. CDUS helped to pinpoint the extent of thromboses of the internal jugular vein demonstrated by CT. CDUS and HRCT should be performed as early as possible to confirm and treat this life-threatening condition.

Adolescent

GAX 65: new injectable cross-linked collagen for the endoscopic treatment of vesicoureteral reflux--a double-blind study evaluating its efficiency in children.

PURPOSE: In the experimental model glutaraldehyde cross-linked collagen GAX 65 with a collagen concentration of 65 mg./ml. has proved to have more persistent implant volume and, therefore, a better antireflux effect than GAX 35, which is injected more commonly. The aim of this study was to evaluate the potential clinical application of GAX 65. MATERIALS AND METHODS: Ten boys and 8 girls an average of 4.6 years old presented with unilateral or bilateral primary reflux and were randomly divided into 2 groups. All refluxing ureters received 1 endoscopic subureteral injection of an average of approximately 2.9 ml. collagen. In group 1, 16 refluxing ureters were treated with GAX 65 and in group 2, 12 were treated with GAX 35. In all patients voiding cystourethrography was performed immediately after injection and 3 months postoperatively, and ultrasound was done on day 1, and at 1 and 3 months. Implant volume was calculated at the day of injection and at 3 months. The distribution of reflux grades was comparable in the 2 groups. RESULTS: Both materials had excellent injection properties. Immediately after injection all ureters were reflux-free. Of the 16 ureters treated with GAX 65 14 (87.5%) were reflux-free at the 3-month followup. Three months postoperatively ultrasound revealed an average implant volume increase of one-third. However, of the 12 ureters treated with GAX 35 reflux had resolved in 59.3% and the average implant volume had decreased by half 3 months after injection. CONCLUSIONS: Three months after endoscopic subureteral injection of GAX 65 vesicoureteral reflux was absent in 87.5% of patients. Therefore, GAX 65 has greater potential for treating reflux in the short term than GAX 35. To define the final efficacy of GAX 65 for treating vesicoureteral reflux, long-term evaluation of this series is necessary. In addition, further studies are mandatory to prove the long-term advantage of this new injectable substance.

Biocompatible Materials

Evaluating bone marrow metastasis of neuroblastoma with iodine-123-MIBG scintigraphy and MRI.

Of 10 patients with neuroblastoma who had both 123I-MIBG scintigraphy and MRI at diagnosis, four presented with bone marrow metastasis that was diagnosed by both imaging modalities and confirmed by bone marrow biopsy and smears. This report focuses on the follow up of the four patients with bone marrow metastasis. MIBG scintigraphy and MRI were concordant in two patients, a case of normalization and a case of relapse in the seventh dorsal vertebra confirmed by surgical biopsy. The last two patients presented a normalized MIBG scan for marrow infiltration after chemotherapy but persistent abnormal MRI signal of several vertebrae, suggesting marrow infiltration, up to 27 mo after the end of chemotherapy in one case. In the second patient, MRI bone marrow aspect returned to normal 4 mo after the end of chemotherapy. Bone marrow biopsy remained negative in these two MIBG-negative patients. These cases suggest that in presence of complete normalization of the MIBG scan after chemotherapy, the persistence of a hypointense signal on bone marrow on T1WI does not necessarily indicate persistence of disease but may be due to delayed normalization. Therefore, attention must be paid to the delay of signal normalization on MRI (which can be as long as more than 2 yr after the end of chemotherapy) in order to avoid false-positive interpretation.

3-Iodobenzylguanidine

[Pulmonary arteriovenous fistula: a rare cause of progressive asymptomatic cyanosis in neonates].

A six month old girl with no significant medical history was admitted to hospital for progressive cyanosis of recent onset refractory to oxygen therapy. There were no detectable cardiac murmurs. Chest X-ray showed an irregular left posterior parahilar infiltration. Echocardiography showed dilatation of the left atrium and ventricle but no cardiac malformation. The diagnosis of pulmonary arteriovenous fistula was suspected. Chest CT scan, magnetic resonance imaging and pulmonary angiography demonstrated the arteriovenous fistula in the lower lobe of the left lung. It was much bigger than the appearances of the chest X-ray suggested. After the left lower lobectomy, the cyanosis completely disappeared. Progressive cyanosis refractory to oxygen therapy without any apparent cardiac or pulmonary disease is strongly suggestive of pulmonary arteriovenous fistula. Surgical treatment is curative whereas the spontaneous outcome of this condition may be lethal.

Angiocardiography

A new case of Pfeiffer syndrome with mutation in FGFR2.

We report on a sporadic case of Pfeiffer syndrome in a male newborn with complex craniosynostosis, broad thumbs and great toes and early demise. SSCP and direct sequencing revealed a missense mutation at position 1037 of the exon B (or IIIc) of the FGFR2 gene (codon 342) resulting in a cysteine to serine modification (TGC-TCC). Genotype-phenotype correlations between the FGFRs mutations and the different craniosynostotic syndromes are discussed.

Acrocephalosyndactylia

[Magnetic resonance angiography in vertebro-basilar ischemic accidents].

This study evaluates the use of MR angiography in the diagnosis of vertebrobasilar stroke. The MRI/MR angiography and clinical presentation of 50 unselected adult patients who presented with ischemic symptoms in the vertebrobasilar territory (10 TIAs, 40 strokes) were prospectively evaluated. Conventional T1 and T2-spin echo sequences were obtained for evaluation of the brain parenchyma, with gadolinium injection in 41 cases and 3D time-of-flight MR angiography in all patients. Data were reconstructed with a maximal intensity projection algorithm and displayed in 3D. Arterial and parenchymal lesions and clinical data were correlated. MR angiography showed intracranial vertebrobasilar lesions in 30 patients (5 stenosis or occlusion of a vertebral artery, 17 stenosis or occlusion of the basilar artery, 6 stenosis of arterial branches of the basilar artery, 10 dolichoectasia of the basilar artery). 7 patients had multiple lesions. Recognized etiologies of the strokes were: 23 vertebrobasilar lesions, 16 nonvertebrobasilar causes, 6 mixed (vertebrobasilar and associated alternative causes) cases, while 5 cases had no recognized cause of stroke. Our study demonstrates a high incidence of intracranial arterial lesions in patients suffering from vertebrobasilar stroke and suggests that MRA can help in a noninvasive way to determine the origin of vertebrobasilar strokes.

Adult

Leptomeningeal cyst in newborns due to vacuum extraction: report of two cases.

Two new cases of leptomeningeal cysts subsequent to vacuum extraction are reported. Both children presented with a huge, nonpulsating, transilluminating subgaleal collection over the anterior fontanel that appeared soon after instrument delivery. Plain X-rays, computed tomography, and magnetic resonance imaging confirmed that the subgaleal collection was cerebrospinal fluid and showed the presence of a diastatic coronal suture in both cases. Treatment consisted of duraplasty with periosteal flaps and application of fibrin glue. In one case, an associated porencephalic cyst was treated with a cystoperitoneal shunt. Surgical treatment of leptomeningeal cyst due to vacuum extraction is simple and should not be postponed, despite the tendency for the extracranial cyst to regress, because of the potential risk of continuous growth of an underlying porencephalic cyst and risk of neurological damage.

Arachnoid Cysts

Cushing's disease due to a giant pituitary adenoma in early infancy: CT and MRI features.

We report the case of a 12-month-old girl presenting with diabetes insipidus and Cushing s disease. Brain magnetic resonance imaging (MRI) demonstrated a large tumour arising from the sella turcica, extending up to the foramen of Monro and invading the cavernous sinuses. Surgery was performed to remove the suprasellar part of the tumour, and histology revealed an adrenocorticotrophin (ACTH) secreting pituitary adenoma. This entity is very rare in this age group and the MRI features have not previously been described.

Adenoma

Hepatic trauma in children: long-term follow-up.

The purpose of this report is to assess the role of long-term clinical and hepatic ultrasound (US) follow-up in children who sustained blunt liver trauma. Forty-three children with blunt hepatic injury were prospectively studied between 1976 and 1994 in our institution. Four patients died (9%). Fourteen children (52%) were operated upon and 13 (48%) were treated conservatively. Twenty-seven patients were recalled for a long-term echography (US) with a mean time span of 6 years after trauma. The physical examination and the liver tests were normal in all patients. The US aspect of the liver was abnormal in 22% of our cases, showing hypoechogenic areas and anomalies of the biliary tract in four patients (11%). Cholelithiasis secondary to hemobilia was demonstrated in 2 patients. Cholecystectomy was performed in one patient because of posttraumatic strictures of the biliary tract. All patients with US anomalies of the liver and biliary tract were clinically asymptomatic and had normal liver tests. We recommend a follow-up US in children having sustained blunt hepatic injury about 6 months after the trauma. If no cholelithiasis or alteration of the biliary tract is present, no other follow-up procedure seems necessary.

Abdominal Injuries

Bone mineral content in cystic fibrosis patients: correlation with fat-free mass.

OBJECTIVE: To assess the bone mineral content in well nourished patients with cystic fibrosis and to seek a correlation with fat-free mass. METHODS: Fourteen cystic fibrosis patients aged 6 to 20 years were studied and compared to 14 healthy controls matched for gender, age, and nutritional status. Bone mineral content was determined by dual energy x ray absorptiometry (DEXA). RESULTS: Nutritional inquiry showed higher ingestion of macronutrients and micronutrients by cystic fibrosis patients than by controls. Mean whole skeleton bone mineral content was 1.184 (SD 0.536) kg in cystic fibrosis patients and 1.229 (0.576) kg in controls (p = 0.84). Mean lumbar spine bone mineral content was 0.031 (0.013) kg and 0.031 (0.016) kg, respectively (p = 0.99). Anthropometry, bioelectrical impedance analysis, and DEXA showed that fat-free mass was similar in the two groups. Bone mineral content was strongly correlated to fat-free mass. Mean blood calcium, phosphorus, serum 25-hydroxyvitamin D (25-OHD), parathyroid hormone (PTH), and osteocalcin were similar in both groups. CONCLUSIONS: Bone mineral content and body composition are normal in a well nourished young cystic fibrosis population. Osteopenia previously reported in cystic fibrosis patients probably has nutritional origins and is therefore not related to a primary defect in bone mineral metabolism.

Adolescent

[Spontaneous intracranial hypotension syndrome].

Spontaneous intracranial hypotension is a rare but well known entity first described by the German neurosurgeon Schaltenbrand. We report the clinical and radiological findings of four patients (2 males, 2 females, mean age 55 years) presenting with this clinical entity and peculiar constant MRI findings. Intense postural headache was present in all patients together with a very low CSF pressure at lumbar tap although none of the patients had any history of recent lumbar puncture, spinal or cerebral surgery or cranio-cervical trauma. MRI revealed in all patients an intense meningeal enhancement and thickening which was most prominent on the dural side of the subdural space. The ventricular system was thin, presenting almost like slit ventricules. A downward shift of the cerebellar tonsils and hemorrhagic subdural collections were also observed in two patients. Biopsy of meninges performed in two patients showed fibrosis of the leptomeninges together with signs of old hemorrhage in one case. We postulate that histologic and radiologic changes are due to chronic subdural bleeding in relation with abnormal displacement of the nervous structures due to intracranial hypotension. The underlying cause of spontaneous intracranial hypotension is rarely established and the course of the disease is benign. Some authors have advocated to perform isotopic cysternography in search for a CSF leak, particularly in the spine, that could be surgically corrected. No such investigation has been conducted yet in our patients because the spontaneous evolution has been mostly favorable.

Cerebrovascular Disorders

[Multiple familial pheochromocytomas: sonographic demonstration of multiple adrenal, celiac and bladder localizations in a child].

Familial pheochromocytoma is an uncommon form of this neoplasia. It is characterized by an autosomal dominant inheritance and multicentric locations. It is more frequently encountered in children, but with a smaller risk of malignant transformation than in adults. The familial form may be associated with other disorders, particularly with multiple endocrine neoplasia (MEN), Von Hippel-Lindau's disease and Von Recklinghausen's disease. We report the case of a nine-year-old boy with multiple familial pheochromocytoma in whom sonography allowed to demonstrate five localizations.

Adrenal Gland Neoplasms