[Diastematomyelia diagnosed prenatally]. / Diastématomyélie diagnostiquée en période prénatale.

We report a case of a 32-week-old foetus affected by diastematomyelia. A third trimester echography suggested a spine abnormality. Further investigations by CT-scanner and MRI were performed to precise the anomaly and to better counsel the couple on the prognosis. We performed a review of the literature about embryological hypotheses, clinical presentations, antenatal investigations and prognosis of such a rare spinal malformation.

An unusual case of congenital cardiac cyst of the interventricular septum.

We present a very rare case of a neonatal multiple haemangiomatosis with a cystic interventricular cardiac haemangioma. An anechoic cyst of 10 mm of diameter, located in the superior third of the interventricular septum was found by foetal echocardiography at 30 weeks of pregnancy. The baby was born at 39 weeks of pregnancy and the skin was covered with multiple purple cutaneous haemangiomas. As neonatal haemangiomatosis may be associated with visceral haemangiomas, the diagnosis of cardiac haemangioma was considered and transthoracic echocardiography revealed a translucent cyst consistent with this diagnosis. At 6 months of age, the cardiac mass was barely visible and most cutaneous lesions had regressed without treatment. Cardiac haemangiomas must be considered in the diagnosis of cystic masses, and the present case emphasizes the tendency towards spontaneous regression of these lesions.

Antenatal embolization of a large chorioangioma by percutaneous Glubran 2 injection.

We describe a case of a large chorioangioma diagnosed at 18 weeks' gestation. Because of advanced fetal heart failure at 23 weeks' gestation, embolization of the chorioangioma's vessels was performed by percutaneous injection of Glubran 2 surgical glue. There was no immediate secondary effect of treatment. Devascularization was complete and durable. Signs of fetal cardiac failure normalized after 1 month and a healthy infant was delivered at 38 weeks. To our knowledge this is the first reported case of perinatal survival after successful embolization of a chorioangioma using tissue glue.

Magnetic resonance imaging in the prenatal diagnosis of congenital diarrhea.

OBJECTIVE: Congenital diarrhea is very rare, and postnatal diagnosis is often made once the condition has caused potentially lethal fluid loss and electrolyte disorders. Prenatal detection is important to improve the immediate neonatal prognosis. We aimed to describe the prenatal ultrasound and magnetic resonance (MRI) imaging findings in fetuses with congenital diarrhea. METHODS: The study reports the pre- and postnatal findings in four fetuses that presented with generalized bowel dilatation and polyhydramnios. We analyzed the fetal ultrasound and MRI examinations jointly, then compared our provisional diagnosis with the amniotic fluid biochemistry and subsequently with the neonatal stool characteristics. RESULTS: In each of the four cases an ultrasound examination between 22 and 30 weeks' gestation showed moderate generalized bowel dilatation and polyhydramnios suggesting intestinal obstruction. MRI examinations performed between 24 and 32 weeks' gestation confirmed that the dilatation was of gastrointestinal (GI) origin, with a signal indicating intraluminal water visible throughout the small bowel and colon. The expected hypersignal on T1-weighted sequences characteristic of physiological meconium was absent in the colon and rectum. This suggested that the meconium had been completely diluted and flushed out by the water content of the bowel. The constellation of MRI findings enabled a prenatal diagnosis of congenital diarrhea. The perinatal lab test findings revealed two cases of chloride diarrhea and two of sodium diarrhea. CONCLUSION: Congenital diarrhea may be misdiagnosed as intestinal obstruction on prenatal ultrasound but has characteristic findings on prenatal MRI enabling accurate diagnosis; this is important for optimal neonatal management.

[Imaging of urinary tract infection in children]. / Imagerie de l'infection urinaire de l'enfant.

Urinary tract infection is very common in children. Its diagnosis, management and treatment have led to abundant literature and many controversies. The main challenges are to identify patients at risk of infection and those at risk of complicated infections. Long-term complications include reflux nephropathy, renal hypertension and stage renal disease. Imaging has a triple role: to confirm the diagnosis in patients with equivocal clinical symptoms; to determine patients at risk of recurrence; prevention of infection by detecting already in utero patients with congenital malformation. During the acute phase, Ultrasound has an important role in detecting favouring conditions such as urinary tract dilatation. The use of color Doppler increases the sensibility and specificity of ultrasound. To date, DMSA scan is the key examination for demonstrating the parenchymal lesions. CT-scan and MRI are rarely used in this acute phase. Retrograde voiding cystography (RVC) will be used whenever vesico-ureteric reflux is suspected. A decision algorithm can be proposed bases on US and DMSA; RVC will be performed whenever one of them is positive. CT or MRI will be used to look for abscesses complicating the UTI. DMSA scan is the gold standard for detecting renal scanning, sequellae of the infection. In the future, the role of MRI will surely increase for the demonstration of uropathy acute and sequelae of the renal involvement.

Perineal lipoma in a newborn boy--a case report.

We report the case of a newborn presenting with a pediculated mass arising from the anal margin. Antenatal sonogram and magnetic resonance imaging were unable to diagnose the precise nature of the lesion. Sacrococcygeal teratoma, an enterogenous cyst, a polyp, a prolapse or other perineal tumors were all proposed as possible entities. At birth, no other anatomic anomaly than this homogenous 2 cm para-anal lesion was seen. Excision of the mass was performed under general anesthesia. The postoperative histological exam showed mature fat cells. Reviewing the literature, there have been few previously reported cases of congenital perineal lipoma. It is a rare, benign and easy-to-treat condition that can be evocated by morphological sonography or magnetic resonance imaging (MRI).

In utero urinary bladder rupture: a case report.

We report a case of foetal urinary bladder rupture due to posterior urethral valves. A megacystis was diagnosed in a male foetus during routine second trimester ultrasound examination. The diagnosis of bladder rupture was made as, one week later, the bladder became undetectable with the appearance of ascites. During the follow-up, no oligohydramnios developed and intercurrent ascites resolved spontaneously. There are three described mechanisms releasing bladder hyperpressure: bladder diverticles, unilateral vesicoureteral reflux and bladder rupture. In this case, another mechanism might be involved: a patent urachus. The urethral valves were resected and no other surgical treatment was needed. The renal function remained normal. No long-term vesical follow-up of this pathology is available in the literature.

Three-dimensional reconstruction of human diaphragm with the use of spiral computed tomography.

We developed a technique of diaphragm imaging by using spiral computed tomography, and we studied four normal subjects who had been previously investigated with magnetic resonance imaging (A. P. Gauthier, S. Verbanck, M. Estenne, C. Segebarth, P. T. Macklem, and M. Paiva. J. Appl. Physiol. 76: 495-506, 1994). One acquisition of 15- to 25-s duration was performed at residual volume, functional residual capacity, functional residual capacity plus one-half inspiratory capacity, and total lung capacity with the subject holding his breath and relaxing. From these acquisitions, 20 coronal and 30 sagittal images were reconstructed at each lung volume; on each image, diaphragm contour in the zone of apposition and in the dome was digitized with the software Osiris, and the digitized silhouettes were used for three-dimensional reconstruction with Matlab. Values of length and surface area for the diaphragm, the dome, and the zone of apposition were very similar to those obtained with magnetic resonance imaging. We conclude that satisfactory three-dimensional reconstruction of the in vivo diaphragm may be obtained with spiral computed tomography, allowing accurate measurements of muscle length, surface area, and shape.

[The respiratory muscles in emphysema. The effects of thoracic distension]. / Les muscles respiratoires dans l'emphysème. Effets de la distension thoracique.

Besides increasing the work of ventilation, emphysema increases lung volume which in itself has a deleterious effect on the inspiratory muscles. We review here the effects of an acute change in lung volume on the configuration of the rib cage and muscle function. We also discuss the effects of the chronic distension associated with emphysema. The effects produced by changes in muscle length and configuration on the mechanical force and action of inspiratory muscles is detailed with particular focus on the diaphragm and its structural adaptations to experimental emphysema. We also analyze the activation pattern of inspiratory and expiratory muscles during the breathing process in patients with emphysema. Finally, we discuss the effects of single-lung transplantation and reduction surgery on chest distension and improved inspiratory muscle function.

[The medical imaging and radiology department]. / Le Service d'Imagerie Médicale et de Radiologie Interventionnelle.

Technological developments arising from research have affected the whole wide spectrum of medical endeavor and have made a very significant impact on clinical practice and especially on imaging sciences. Ultrasonography brought spectacular advances, but CT and MRI became important landmark techniques. A further important development, which greatly increased the involvement of radiologists in direct patient management, was the growth of interventional and therapeutic techniques, called interventional radiology. Some statistics: approximately 155,000 patients per year including 19,000 CT Scans, 10,000 MRI exams, 21,000 ultrasound examinations and 1,000 therapeutic procedures. Some research activities: CT quantification of pulmonary emphysema, respiratory mechanics, MR and CT angiography, antenatal diagnosis of congenital and genetic diseases of the fetus, quantification of portal haemodynamics, MR imaging of bile and pancreatic ducts, morphologic and functional imaging of the brain, radiology of bone trauma, MR characterization in hepatic lesions.

[Encysted gossypiboma of the lesser omentum: complementary aspects of echography and computerized tomography]. / Gossypibome enkysté du petit épiploon: complémentarité de l'échographie et de la tomodensitométrie computérisée.

The authors report the fortuitous demonstration of an asymptomatic gossypiboma during the preoperative staging of a rectal carcinoma 13 years after abdominal surgery for perforated gastric ulcer. Ultrasonography disclosed a well-defined hypoechoic mass containing highly echogenic foci with a strong posterior shadow. The CT findings are reported, as well as the complementary aspects of sonography. Differential diagnosis from tumors of the lesser omentum is also presented.

The case of HNF-1beta mutation with medullary cysts.

We describe one case of long-term post-natal follow-up of hyperechoic fetal kidneys related to HNF-1beta mutation with cystic changes over a 9-year period in a female patient. This diagnosis was suspected on the basis of the renal US findings and was confirmed by complementary genetic examination. After birth, cortical cysts were detected and at the age of 4, medullary cysts were found, that disappeared with time. Currently our patient displays hyperechoic kidneys with only cortical cysts. This case report highlights the variability of US appearances in relation with HNF-1beta genetic mutation.

Atypical presentation of a Langerhan's cell histiocytosis of the forearm in a child.

We report a case of a 2-year-old child presenting with right forearm pain. Based on imaging analysis, the initial diagnosis was osteomyelitis but the final diagnosis demonstrated by histology was Eosinophilic Granuloma (EG) of the forearm. We detail the rare radiological presentation of such a lesion, the various clinical presentations and the work-up advised in this context.

[Postnatal evaluation and management of fetal pyelectasis on prenatal ultrasound]. / Mise au point postnatale des dilatations des voies urinaires de diagnostic anténatal.

Fetal pyelectasis is the most frequently encountered anomaly on prenatal ultrasound. The ultrasound diagnostic criteria are well established: nature of dilated structures, degree of dilatation, ultrasound appearance of the kidneys, volume of amniotic fluid. Ultrasound can also determine if the anomaly is isolated or not, which is important for the management and prognosis of the pregnancy. Ultrasound is the initial postnatal study to evaluate the urinary tract. Further management will be based on suspected diagnosis. In cases of suspected vesico-ureteral reflux, VCUG is performed. In cases of obstructive uropathy or complex malformation (duplications), MRI is performed at 6 months of age to further define the anatomy of the urinary tract. Radionuclide renogram, performed in most cases, allows evaluation of split renal function. Follow-up ultrasound is important to monitor renal development, urinary tract dilatation and appearance of the renal parenchyma. Functional follow-up is assured by radionucline renography. This comprehensive follow-up is recommended to prevent complications and progressive loss of renal function.

Antenatal diagnosis of a sacrococcygeal teratoma.

Although rare, sacrococcygeal teratoma is the most common congenital tumor. We report a case of a precociously diagnosed and rapidly growing cystic lesion. These tumors may be associated with hemodynamic and hemorrhagic complications. Therefore, affected fetuses should be carefully followed during the entire pregnancy by ultrasound and MR imaging in order to evaluate the evolution of the mass, its consequences on the fetal organs and to appreciate the wellbeing of the fetus. Delivery and post natal work up can therefore be optimized.