Pericallosal lipoma associated with Pai syndrome: prenatal imaging findings.

Pai syndrome is a rare disorder characterized by the association of a midline pericallosal lipoma, median cleft (palate or lip) and cutaneous polyps of the face. Only seven cases have been reported in the medical literature and we are not aware of any previously reported prenatally detected cases. In this article, we present the prenatal ultrasound and magnetic resonance imaging findings of a syndromic pericallosal lipoma with associated anomalies that led to the prenatal diagnosis of Pai syndrome. We underline the impact on parental counseling following prenatal detection of pericallosal lipoma.

[Superior mesenteric artery syndrome]. / La pince aortomésentérique supérieure primitive.

BACKGROUND: Early diagnosis of superior mesenteric artery syndrome, less frequent in children than in young adults, is improved by the use of ultrasonography and CT scan. CASE REPORT: An eleven year-old boy was admitted because he suffered from bilious vomiting for 24 hours. A diagnosis of superior mesenteric artery syndrome was made from the results of plain films of the abdomen, ultrasonography and roentgenograms after instillation of dilute barium solution. Diameter of the left renal vein appeared increased. The patient was given fractionated meals and placed in left lateral position. The CT scan performed 5 days later confirmed the diagnosis but did not show dilatation of the left renal vein. The patient is well one year later without treatment. Ultrasonography performed 3 hours after a meal showed similar findings to those seen in the acute phase. CONCLUSION: Abdominal scanning and ultrasound are valuable tools for diagnosis of such a syndrome. The diagnostic value of dilatation of the left renal vein remains to be determined.

[Aseptic femur head osteonecrosis during treatment of acute lymphoblastic leukemia in the child]. / Ostéonécrose aseptique de la tête fémorale au cours du traitement des leucémies aiguës lymphoblastiques de l'enfant.

UNLABELLED: Avascular femoral head necrosis (AFN) is an uncommon complication of acute lymphoblastic leukemia (ALL) occurring in association with serious functional late effects. One of the many risk factors is high-dose corticosteroid therapy. CASE REPORT: Three children belonging to a series of 266 patients developed AFN. The diagnosis was not made immediately when X-rays were normal. In spite of the fact that treatment was begun as soon as possible, the three children had a difference in the length of their legs, with reduction of their walking perimeter and, in one case, an arthroplasty was necessary. CONCLUSION: If some patients treated for ALL limp or suffer when walking or when practising sports, the diagnosis of AFN is to be evoked. The diagnosis is not only based on simple X-rays but also on magnetic resonance imaging, which is more sensitive and reveals lesions earlier. The treatment consists of immobilization of the hip, whether or not associated with surgical procedures.

[A rare cause of hyponatremia during introductory treatment of acute lymphoblastic leukemia in an infant: inappropriate secretion of atrial natriuretic factor?]. / Une cause rare d'hyponatrémie lors du traitement d'induction d'une leucémie aiguë lymphoblastique chez un nourrisson: la sécrétion inappropriée de facteur atrial natriurétique?

BACKGROUND: --Hyponatremia is frequently seen during the treatment of acute lymphoblastic leukemia: its causes are numerous. This work aims to present a case in whom hyponatremia was possibly due to an increased secretion of atrial natriuretic factor. CASE REPORT: --A 3 week-old baby was admitted because of malignant hemopathy. A diagnosis of acute lymphoblastic leukemia was rapidly made and the patient was firstly given alkaline diuresis, urate-oxidase and corticosteroids. Vincristine and daunorubicin were associated one week later. Insertion of a central intravenous line in the right subclavicular artery failed so that this catheter was finally inserted into the left jugular vein. Natremia was 126 mmol/l at that time and dramatically decreased within 24 hours to 109 mmol/l without net changes in water and electrolytic input. At that time, sodium urinary excretion was 6 mmol/kg/day (diuresis: 420 mlF/day). There was no hemodynamic changes, nor digestive or cardiac manifestations. Ultrasonography showed that the left superior cava vein drained into the right cardiac atrium. The catheter was withdrawn and the patient was given sodium supplementation permitting complete and definitive cure of hyponatremia within 2 days. CONCLUSIONS: --All usual causes of hyponatremia having been ruled out in this patient, we postulate that hyponatremia was due to direct stimulation of atrial natriuretic peptide through an increase in atrial pressure secondary to the catheter insertion near the cardiac atrium.

[Mountain frostbite (apropos of 1260 recorded cases)]. / Les gelures de montagne (à propos d'une statistique de 1260 cas).

Based on an important statistic of mountain frostbite (1260 cases), the authors try to explain the underlying physiopathological mechanism which remains largely unknown. An early prognosis may nowadays be improved by several diagnostic tools whose efficiency is reviewed. Difficult problems arise with severe frostbite only. In those cases, the treatment as such seems still limited to saving what has been spared. In any case, a quick warming up is the keystone of the treatment.

Management of primary obstructive megaureter without reflux in neonates.

Systematic antenatal ultrasonography has significantly altered the conditions of diagnosis of megaureters. Pediatric urologists are now confronted with a large group of neonates with asymptomatic megaureter. Furthermore, reports of spontaneous resolution of primary megaureter without reflux have become common. We were confronted with 59 renal units in 48 neonates. We postulated that primary megaureter represented a significant obstructive uropathy when the kidney exhibited stasis and large pelvic and caliceal dilatations. So, 35 ureters were operated on initially. The other 24 cases were managed conservatively but among these patients, 11 ureters were operated on secondarily 7-29 months after the diagnosis because they were unchanged [6] or worsened [5]. 13 ureters are currently without treatment: 7 total regressions and 6 incomplete regressions with persistent mild pelvic dilatation. The results of reimplantation, early or delayed, have been excellent (1 postoperative necrosis reoperated, 1 secondary reimplantation, 2 persistent mild dilatations). Relief of obstruction without reflux was obtained in 36/39 long-term follow-up cases (92%). There is a disagreement about the relative merits of various modalities in the assessment of ureteral obstruction and impairment of renal function. Therefore, we chose to use essentially intravenous pyelography (IVP) and to operate initially when there was a delayed appearance of the contrast agent, a massive dilatation and delayed drainage from the ureter into the bladder. This attitude is open to question but no more illogical than waiting for impairment of renal function to decide on surgery.

Coexisting calcified inferior vena cava thrombus and adrenal hemorrhage in the neonate: report of three cases.

Coexisting calcified inferior vena cava (IVC) thrombus and adrenal hemorrhage are uncommon in children. We present three such cases diagnosed by ultrasound in three boys who were born after 41-, 38-, and 38-week gestations, respectively. In all cases the pregnancy and delivery were unremarkable. The birthweight was 3,630 g, 3,210 g, and 3,390 g, respectively. Left adrenal hemorrhage occurred in all cases. Associated left renal vein thrombus occurred in two cases. The mean followup was 8.5 years. In two cases ultrasound showed persistence of a calcified thrombus and residual stenosis of the IVC. In the third case the IVC thrombus had almost disappeared.

Hepatic tumours during androgen therapy in Fanconi anaemia.

The occurrence of liver tumours in the course of Fanconi anaemia (FA) has been well documented. We present a case, review the literature and conclude that androgen therapy would increase the risk of developing tumours, most of which appear to be benign (adenomas or peliosis) and androgen-dependent, generally decreasing in size after cessation of treatment. Survival of patients is poor, mostly because of the rapid evolution of the tumour. In the absence of an allogenic bone marrow transplantation, administration of haematopoietic growth factors might be effective. As a preventive measure, other types of unsubstituted androgens may be used.

Sclerosing hemangioma of the lung: radiological findings and pathological diagnosis.

We present a case of sclerosing hemangioma of the lung, revealed as a solitary round mass on routine chest radiography in a 14-year-old patient. CT and MRI findings were consistent with a solid neoplasm but no specific characteristics were identified. Analysis of the surgical biopsy material led to a cytological diagnosis of adenocarcinoma. Definitive diagnosis of sclerosing hemangioma of the lung was established on pathological examination of the whole surgical specimen.

Giant hypothalamic hamartoma: an unusual neonatal tumor.

A case of neonatal manifestation of giant hypothalamic hamartoma is reported. It is suggested that hypothalamic hamartoma should be included in the list of neonatal intracerebral tumors. Magnetic resonance imaging appearance similar to that of normal gray matter on T1-weighted images and slightly hyperintense on T2-weighted images, without enhancement after gadolinium injection, is suggestive of the diagnosis. Hypothalamic hamartomas are congenital malformations, consisting of disorganized mature neuronal elements in proportions similar to that of normal tissue [1]. They are clinically evidenced in infants ranging from 1 to 7 years of age [1-5]. This report describes a histologically proved giant hypothalamic hamartoma diagnosed in the neonatal period. Magnetic resonance imaging (MRI) is helpful to distinguish this congenital non-evolutive malformation from more aggressive neonatal tumors.

Sonography of the hip and soft tissues of the thigh in children.

High-resolution real-time sonography (US) has been used for many years in departments of pediatric radiology to image soft tissues and joints in children. The purpose of this study was to review the main indications and results of US in assessing the hip (except for congenital dislocation and subluxation) and the soft tissues of the thigh and groin in children. The technical and anatomical factors relative to the optimal imaging of the hip joint and thigh muscles are also discussed and a variety of conditions considered, ranging from painful hips to soft tissue masses. In conclusion, high-resolution US emerges as a particularly useful tool in the study of children with acute hip pain (hip joint effusion), suspected osteomyelitis (fluid collection around the femoral saft) and various soft tissue of the thigh.

Ultrasound diagnosis of midgut volvulus: the "whirlpool" sign.

The authors present their US findings in 24 patients with proved complicated midgut malrotation: volvulus in 18 and occlusive Ladd's bands in 6. All the 24 patients have had US examination prior to surgery. Contrast examinations were performed in only 9 patients, always after US and before surgery. The sonographic "whirlpool" pattern of the superior mesenteric vein and mesentery around the superior mesenteric artery was detected in 15 of the 18 patients with midgut volvulus, and was best seen using Doppler color. Embryological signification of midgut malrotation is discussed.