Ovarian fibromatosis and sotos syndrome with a new genetic mutation.

BACKGROUND: Sotos syndrome is one the most common overgrowth conditions, after Beckwith-Wiedemann syndrome. As with other overgrowth syndromes, Sotos syndrome can be associated with an increased risk of tumors. CASE: We describe a young girl with Sotos syndrome and ovarian fibromatosis with a new mutation not reported before in the literature. SUMMARY AND CONCLUSION: Development of ovarian tumor in Sotos syndrome has been poorly documented. Ovarian fibromatosis is a very rare non neoplastic disease. Management is guided by the benignity of the lesion and consists of surgical excision of the fibroma.

[Erythropoietin before severe hypospadias repair: fewer complications?]. / Préparation à l'érythropoïétine avant chirurgie de l'hypospade : de meilleurs résultats ?

OBJECTIVE: To study the effect of a preparation by erythropoietin before Onlay's intervention on postoperative hematocrits and medium-term results of the surgery. PATIENTS AND METHODS: Twenty-one patients were operated on by Onlay's technique between 2001 January and 2008 September, after being treated by erythropoietin. Seven had a midshaft hypospadias, two a posterior hypospadias and 12 a penoscrotal hypospadias. All children were examinated four months after surgery to evaluate the surgical results. RESULTS: After the surgery, 18 children had an apical meatus (85.7%). Three had a balanopreputial meatus, two a stenosis of the meatus. Two children were reoperated on, one with the Duplay's technique, the other for a meatostomy. No fistula, no necrosis of the preputial flap was observed. The preoperative hematocrit was measured at 41%, with an increase of 3.8% because of the preparation. Peroperative blood loss was evaluated at 6.6%. After surgery, 12 children had an hematocrit inferior than 35%, four an hematocrit inferior than 30%. No transfusion was needed. CONCLUSION: Preparation by erythropoietin before severe hypospadias surgery seemed to have several advantages: a more elevated hematocrit after surgery than with an iron preparation alone, and a lower rate of postoperative complications, including fistulas and necrosis of the preputial flap.

[Imaging evaluation of renal function: principles and limitations]. / Mesure de la performance rénale en imagerie: principes et limites.

The kidney performs multiple functions. Glomerular filtration is the most studied of these functions. In clinical practice, the surgical indication for patients with unilateral uropathy is frequently based on the split renal function as demonstrated by scintigraphy. MRI is not yet validated as a technique but nonetheless offers an interesting non-radiating alternative to achieve both morphological and functional renal evaluation. Recent pulse sequences such as diffusion, arterial spin labeling, and blood oxygenation dependent imaging may also provide additional information. CT and US remain of limited value for the evaluation of renal function.

Conservative treatment of big yolk sac tumour of the ovary in young girl.

We present the case of a large yolk sac tumour of the ovary in a 14-year-old girl with high level of serum alpha-feto-protein (AFP). Multidisciplinary care is important to do appropriate surgical treatment with the aim of fertility preservation.

[Sternal cleft: repair during the neonatal period]. / Fente sternale : intérêt d'une prise en charge chirurgicale néonatale.

Sternal cleft is a rare congenital abnormality. We report on a neonatal case of sternal cleft with median raphe with a follow-up of 3 years. We review the advantages of surgical repair in the neonatal period because the flexibility of the chest wall is maximal and compression of underlying structures is minimal.