Atypical forms of isolated partial atrioventricular septal defect increase the risk of initial valve replacement and reoperation.

OBJECTIVE: We consider the short- and long-term outcomes of the repair of the isolated partial atrioventricular (AV) septal defect to determine the role played by the atypical forms on the initial AV valve replacement and on the risk of reoperation. METHODS: Two hundred and eight patients underwent an operation for this malformation between 1974 and 2001. Clinical and echocardiographic examinations were performed on all patients, the AV valve regurgitation was graded from 1 to 4 and a residual interatrial shunt was sought. Median age at the intervention was 5.8 years (3 months to 67 years). RESULTS: Median follow-up time was 7.5 years (range 0-22.6 years). The cumulative 30-day, 5- and 20-year survival rates were 96.5, 95.4 and 94.6%, respectively. AV valve replacement was associated with a high mortality (P<0.001). A reoperation was performed on 12 patients (5.7%) including six patients within less than a 30-day period, especially to repair residual AV valve regurgitation. We performed four AV valve repairs by annuloplasty and six AV valve replacements. Two patients who had initially undergone an AV valve replacement underwent a reoperation for valve thrombosis. The cumulative 30-day, 5- and 20-year rates of freedom from reoperation were 96.5, 93.6 and 83%, respectively. An atypical form was present in 24 patients (11.5%) and was a risk factor for initial AV valve replacement (P<0.001) and for reoperation (P<0.001). A complete AV block occurred in 13 patients (6.2%), all of them within a 30-day period. The AV valve replacement was a high risk factor for a complete AV block (P<0.001). At the end of our study 180 patients (96%) were in NYHA I and 8 in NYHA II. CONCLUSIONS: The morbi-mortality of the isolated partial AV septal defect is primarily perioperative and is linked with the presence of an atypical form of the lesion. This atypical form was the main reason for reoperation for AV valve regurgitation. The AV valve replacement was associated with a high mortality and with the occurrence of complete AV block. Using a standardized technique, the AV septal defect can be repaired with excellent long-term clinical and echographic results.

Changing management of cardiac myxoma based on a series of 40 cases with long-term follow-up.

BACKGROUND: Cardiac myxoma is generally considered to be a surgical emergency. However, as a result of progress in echocardiography and the increasing age of the patients presenting with this disease, the clinical presentation has changed and the management of cardiac myxoma now needs to be reviewed. METHODS: Between 1978 and 2001, 40 patients (16 men and 24 women) between the ages of 6 months and 82 years (mean age, 55.6 years) were operated on for cardiac myxoma. Signs of heart failure with pulmonary congestion (22%) or pulmonary embolism (20%) indicated a high-risk emergency situation in some cases, whereas, in other cases (58%), the patient's condition was stable and the clinical presentation was less worrying. However, the tumor was always removed within 24 hours of admission. Most cases of cardiac myxoma observed over the last decade correspond to stable forms, as echocardiography has revealed smaller tumors in generally elderly patients. RESULTS: The postoperative mortality was 7.5% (3 patients). No patients were lost to follow-up, and the mean follow-up was 13.6 years. One patient was reoperated for recurrence 3 years postoperatively. Five patients required further cardiac surgery: three mitral valve replacements, one coronary artery bypass graft, and one angioplasty. The 15-year survival rate was 69%. CONCLUSIONS: Myxoma tends to be observed in a more elderly and higher risk population, often at an early stage. The classic approach of emergency surgery is not always appropriate in these stable forms, allowing more thorough preoperative assessment of these patients.

[Testicular Leydig cell hyperplasia in children]. / Hyperplasie à cellules de Leydig du testicule chez l'enfant.

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.

Extracapsular hematoma of the parathyroid glands.

The objective of this study was to standardize surgical treatment of cervicothoracic hematoma due to parathyroid gland rupture. Only 19 such hematomas have been reported in the literature, and there is no consensus about the best time to operate or the surgical approach. We have now treated four new cases of extensive hematoma from the time of the initial bleeding except in the case of severe hemodynamic or respiratory troubles or nerve compression. Two of the patients were operated on after a minimal 3 months delay, with perfect results. The other two were operated on during the first month with great technical difficulty and incomplete results. In one case the gland was not excised, but parathyroid apoplexy afforded a spontaneous remission, although the hyperparathyroidism recurred 7 years later. We concluded that, first, if there are no severe compressive or hemodynamic symptoms, the surgical treatment must be performed more than 3 months after the bleeding, as the dissection then is as simple as any well ordered surgery. In case of an extensive hematoma accompanied by shock or compression, we propose simple emergency drainage, with reoperation 3 months later. Second, parathyroid apoplexy sometimes offers spontaneous remission of primary hyperparathyroidism, although late recurrence is always possible and surgical treatment then cannot be avoided.