Search

Polyorchidism is an urogenital curiosity defined by the presence of more than two testes confirmed by histology. This anomaly is extremely rare and only a hundred world cases were described in the literature. Although it can remain asymptomatic, polyorchidism is often associated to processus vaginalis anomalies in childhood (hernia, hydrocele) and undescended testis. The review of the literature finds cases of polyorchidism revealed by testis torsion and an increased risk of malignancy and infertility. We report the case of polyorchidism in a 14 year old child, at whom a painful testis mass indicate surgical exploration and documented the histological diagnosis of polyorchidism. Through this observation and review of the literature, authors describe pathological and managment findings of polyorchidism.

Objective: Genetic diagnosis available since 1993 established germinal mutations of the RET proto-oncogene at codon 634 as the main cause of inherited medullary thyroid carcinoma (MTC). International guidelines established in 1999 recommend that children with such mutations undergo a total thyroidectomy before age 5, with unspecified cervical neck dissection. Since 1993, only 41 of 275 thyroidectomies reported in RET 634 children were performed before age 5 (15%). The aim of this study was to evaluate the implementation of these guidelines in a single centre., Design and Patients: Genetic diagnosis was proposed to the parents of all eight children born after 1992 from two RET 634 families. Total thyroidectomy was proposed before age 5 if the child carried a mutation., Results: Genetic diagnosis was performed in all the children (aged 1-3) and thyroidectomy in the five who carried a mutation (aged 2-5). Cervical lymph node dissection varied from lymphadenectomy of central and lateral compartments in the eldest child to pickings in the youngest. There was no permanent hypoparathyroidism or recurrent nerve paralysis. C-cell hyperplasia, medullary thyroid carcinoma and lymph node metastasis were present in 5/5, 3/5 and 0/5, respectively. Undetectable pentagastrin-stimulated CT levels were achieved and maintained postoperatively in all five children (average follow-up 3.6 years)., Conclusions: MEN 2 guidelines on thyroidectomy can be efficiently and safely implemented by a multidisciplinary team operating in a single centre. The lack of guidelines on cervical neck dissection remains a problem; this could be solved by determining an age under which this procedure would be deemed unnecessary.

Pediatric thoracic emergencies are frequent and may be due to a large spectrum of lesions including traumatic and non traumatic pathologies, such as foreign bodies, mediastinal tumors, pulmonary infections, asthma, pneumothorax and delayed manifestations of congenital chest malformations. Emergencies require rapid diagnosis to make a treatment plan and in most cases, radiology plays an essential role. Plain chest radiographs remain the initial study with inspiratory films. In certain circumstances, the use of expiratory films is absolutely necessary. Ultrasonogragraphy is the primary modality for evaluation of pleural effusions. Computed tomography (CT), with volume acquisition and more rapid scanning, is a technique capable of imaging the lungs and mediastinum with excellent spatial resolution in the pediatric population. CT provides more information than chest radiographs. This explains the increasing indications of CT in the evaluation of pediatric thoracic emergencies, more particularly traumatic emergencies.

The transverse colon is an exceptional location of intestinal duplication. Perforated duplications are rarely described in neonates. Meconium peritonitis (MP) can originate from prenatal perforated intestinal duplication. The authors report a case of a baby girl with prenatal diagnosis of MP. Rapid worsening of clinical aspects at birth and the presence of a pneumoperitoneum on systematic abdominal plain radiographs led to urgent surgery on the 1st day of life. Laparotomy showed a perforated necrotizing tubular duplication of the transverse colon. Removal of the duplication followed by limited segmental colonic resection and double colostomy were carried out. Follow-up was uneventful.

In a previous anatomical study of the oblique sinus of pericardium, we reported on the morphological variations of this recess. We noted a previously undescribed variation of its top in 32.7% of our cases. Here we detail this variation using microscopic and macroscopic mediastinal dissections of 107 adult fresh cadavers. It seems a top fold of the oblique sinus contains a permeable esophago-atrial vein or a fibrous cord. These unique variations of the oblique sinus modify the classical anatomical and embryological descriptions. Abnormal esophago-atrial veins have not been described previously but they are predictable according to embryological knowledge. From a clinical point of view, such a vein, permeable or not, may facilitate extension of esophageal carcinoma to the pericardium and may represent a porto-systemic bypass in cases of portal hypertension.

Introduction: Leydig cell hyperplasia (LCH) of the testis is rarely described in children. The authors report a case of incidental discovery of LCH on ultrasound examination., Case Report: The authors report the case of a 9-year-old boy presenting with isolated and painless increased volume of the left testis, with no clinically palpable mass. Scrotal ultrasound revealed an echogenic mass, 12 mm in diameter, with a solid appearance and several hypoechoic areas, without calcification, situated in the lower pole of the testis. Tumour markers and gonadotropin axis hormonal assessment were normal. On surgical exploration, the testis had a macroscopically normal appearance; opening of the tunica albuginea revealed the lesion and enucleation was performed. Histological examination confirmed the presence of LCH, 5 mm in diameter. The postoperative course was uneventful. Physical examination and scrotal ultrasound have remained normal with a follow-up of two years., Discussion: The authors recall the characteristics of LCH, which usually presents, in children, in the form of signs of precocious puberty or more rarely by gynaecomastia. The lesion is rarely palpable. Scrotal ultrasound reveals a homogeneous mass with several hypoechoic nodules. An endocrine assessment must always be performed (frequent elevation of LH). Surgery should be as conservative as possible (enucleation-resection). Histological diagnosis may be difficult.

Objective: To evaluate the anatomy and histology of the ureterovesical junction resected during secondary surgical reimplantation for persistent reflux after failure of initial endoscopic treatment by polytetrafluoroethylene (Teflon) in 27 cases and polydimethylsiloxane (Macroplastique) in 13 cases., Material and Method: 61 ureterovesical junctions from 40 children were studied histologically. The mean age of the patients at the time of the operation was 4.1 years (range: 1 to 15 years). The mean interval between endoscopic injection and surgical reimplantation was 15.3 months (range: 2 to 54 months)., Results: Persistent reflux was not correlated with the anatomical situation of the implant, which was found to be in a satisfactory position in 52.4% of cases. Both of the substances used induced a giant-cell macrophage reaction which colonized the implant and triggered new vessel formation. Macroplastique appeared to be associated with a more intense inflammatory reaction than Teflon. Despite the difference in particle size, the two substances induced a macrophage phenomenon characterized by microfragmentation into 6 micron particles. No conclusions can be drawn concerning distant migration, but this study showed rarefaction of particles which were replaced by fibrosis, the density of which was correlated with the age of the implant., Conclusion: Extinction of the local reaction induced by the products used in this study appears to be long and the end of this process is unknown, which justifies prolonged surveillance of children treated for reflux by endoscopic submeatal injection.

Introduction: Thyroglossal duct cyst (TGDcs) is the most common malformation of the neck. The risk of infection and malignant transformation impose its treatment., Objectives: The purpose of our study were: 1) to specify some points about the symptomatology and preoperative evaluation necessary for TGDcs diagnosis; 2) to analyse the factors who can explain an unsuccessful surgical treatment., Patients and Methods: Our study is based on a retrospective review of cases and a review of the literature. From 1981 to 2000, 99 children with a mean age of five years were treated for a TGDcs with a surgical procedure in the Grenoble University Hospital. 3 excision and 96 Sistrunck's procedure were performed. In all cases a histological study was made., Results: Ultrasonography was the most frequent preoperative evaluation. We have had 7 complications: 3 haematoma, 2 abscess and 2 desunited scar. 6 children have had recurrent disease. Among these 6 children, 3 have had an excision and 3 a Sistrunck's procedure. No case of malignant transformation was reported., Conclusion: Ultrasonography is the first preoperative evaluation to obtain before surgical treatment of a TGDcs. Sistrunck's procedure is the best surgical treatment with a value of recurrence from 1% to 10%. Areas of surgical failure included breaking of cyst during the dissection, inadequate hyoïd bone resection, an anatomical variation with many ductuli in the base of tongue, inadequate muscles of tongue resection.

A placebo-controlled, single blind, crossover study was done to evaluate the inotropic effects of single oral doses of mexiletine and disopyramide assessed by the measurement of Systolic Time Intervals (STI). Each of 8 healthy volunteers received five treatments in random order: 200 and 400 mg mexiletine, 100 and 200 mg disopyramide, and placebo. There was a significant increase in cumulated PEP after 400 mg mexiletine and 200 mg disopyramide. There was no significant change in LVET and QS2. Peak plasma levels were in the lower range of the reputed antiarrythmic levels. Plasma concentration-effect relationships are discussed. Although the study revealed large inter- and intrasubject variability in the measured STIs, it is concluded that a negative inotropic effect was detected despite the low plasma levels of the minor negative inotropic drugs.