[Anaesthesic management of vaginal delivery in a parturient with C1 esterase deficiency]. / Prise en charge anesthésique d'un accouchement chez une patiente atteinte d'un déficit en inhibiteur de C1 estérase.

Hereditary and acquired angioedema (HAE/AAE) are the clinical translation of a qualitative or a quantitative deficit of C1 esterase inhibitor (C1 INH). The frequency and severity of clinical manifestations vary greatly, ranging from a moderate swelling of the extremities to obstruction of upper airway. Anaesthesiologists and intensivists must be prepared to manage acute manifestations of this disease in case of life-threatening laryngeal edema. Surgery, physical trauma and labour are classical triggers of the disease. The anaesthesiologists should be aware of the drugs used as prophylaxis and treatment of acute attacks when considering labour and caesarean section. Androgens are contraindicated during pregnancy. If prophylaxis is required, tranexamic acid may be used with caution. The safest obstetric approach appears to be to administer a predelivery infusion of C1 INH concentrate. It is important to avoid manipulation of the airway as much as possible by relying on regional techniques. We report the case of a patient suffering from an HAE discovered during pregnancy. The management included administration of C1 INH during labor and early epidural analgesia for pain relief. A short review of the pathophysiology and therapeutic options follows.

Early localization and reoperation for persistent primary hyperparathyroidism.

BACKGROUND: Reoperation for persistent primary hyperparathyroidism is often performed after a delay of 4-6 months. Success can be expected in over 90 per cent of cases but exploration is technically difficult and there is the possibility of creating permanent hypoparathyroidism and vocal cord paralysis. This is a study of early localization and reoperation. METHODS: In a consecutive series of 273 patients who had surgery for primary hyperparathyroidism, three remained hypercalcaemic and in three the abnormal parathyroid was not found at initial exploration. They underwent early (6-48 h) single-tracer 99mTc Sestamibi scintigraphy with factor analysis of dynamic structures (FADS) and single photon emission computed tomography (SPECT) followed by reoperation within 24-72 h. RESULTS: Scintigraphy with FADS and SPECT was helpful in all six patients, who were cured by reoperation with no morbidity or symptomatic hypocalcaemia. CONCLUSION: Reoperation for persistent primary hyperparathyroidism is possible and may be easier within days of an initially unsuccessful procedure. With the intact 1-84 parathyroid hormone measurement, the diagnosis is accurate. Single-tracer 99mTc Sestamibi scintigraphy with FADS and SPECT is non-invasive, easily and rapidly performed, and was accurate in these six patients.

[Surgical treatment of primary hyperparathyroidism]. / Traitement chirurgical de l'hyperparathyroïdie primaire.

The authors report their experience with treatment of primary hyperparathyroidism in a series of 1496 patients (September 1991). The clinical profile of the disease has changed in the past few years, incomplete or even asymptomatic forms of the disease are now more common. The results of treatment have considerably improved (99% cure rate), progress is essentially due to two factors: surgeons have gained in experience and diagnostic errors have disappeared thanks to the reliability of laboratory tests and hormone level determination.

Short- and long-term efficacy of total parathyroidectomy with immediate autografting compared with subtotal parathyroidectomy in hemodialysis patients.

A retrospective study was performed in chronic hemodialysis patients comparing total parathyroidectomy (PTX) followed by immediate autografting (IA) (total PTX+IA) with subtotal parathyroidectomy (subtotal PTX). One hundred six patients with severe, uncontrolled hyperparathyroidism were referred to this center and underwent surgery during the period from 1980 to 1990. Long-term follow-up after PTX was available in 49 of them: 28 patients had total PTX+IA and 21 had subtotal PTX. The two surgical methods were evaluated with respect to preoperative severity of hyperparathyroidism, immediate postoperative results, and long-term parathyroid status, as evaluated by an RIA measuring intact immunoreactive parathyroid hormone (intact iPTH; normal values, 15 to 65 pg/mL). The initial degree of hyperparathyroidism was comparable in the two groups. An excellent short-term control of hyperparathyroidism was achieved in the great majority (95%) of patients with either surgical procedure. However, long-term normalization of parathyroid gland activity was achieved in only one third of patients whereas 33% had elevated intact iPTH levels (> 130 pg/mL; i.e., higher than twice the upper range of normal) and 32% had low intact iPTH levels (< 15 pg/mL), consistent with permanent hypoparathyroidism. No difference was found in the immediate failure rates: 0 of 28 cases after total PTX+IA compared with 2 of 21 cases after subtotal PTX. Similarly, long-term intact iPTH levels were comparable: 400 +/- 105 versus 212 +/- 82 pg/mL (mean +/- SE; P = not significant). Interestingly, long-term serum intact iPTH levels were higher in patients with nodular (N = 18) than with diffusely (N = 26) hyperplastic glands: 556 +/- 146 versus 126 +/- 52 pg/mL (P < 0.001) and recurrence of hyperparathyroidism was more frequent with nodular hyperplasia (11 of 18) than with diffuse hyperplasia (4 of 26) (P < 0.02). In conclusion, although excellent short-term results were obtained with both procedures, satisfactory long-term control of parathyroid gland function was achieved in only one third of the patients, the other two third remaining either hypoparathyroid or developing recurrent hyperparathyroidism. Last, the histological subtype of parathyroid glands was partially predictive of the recurrence of hyperparathyroidism.

[Anatomic localization of parathyroid adenomas. Experience of 1200 cases of primary hyperparathyroidism]. / Localizzazione anatomica degli adenomi paratiroidei. Esperienza di 1200 casi di iperparatiroidismo primario.

From 1969 to December 1988 1200 patients were successfully operated on for primary hyperparathyroidism. In 1086 cases the parathyroid lesion was an adenoma and in 252 cases (23.2%) we considered this adenoma in an unusual location. Analysis of the patient's records enables us to describe the operative difficulties for each of those locations, and to assess the value of complementary investigations in the patients whose adenomas are difficult to find.

Secondary hyperparathyroidism: diagnosis of site of recurrence.

Persistent or recurrent hyperparathyroidism after total parathyroidectomy with forearm implant may be caused by hyperplastic grafted tissue, residual parathyroid tissue left in the neck or the presence of a supernumerary gland not found during surgery. A correct assessment of graft function is needed to localize the source of hormone excess and to avoid an unnecessary neck reoperation in cases of graft dependent hyperparathyroidism. In 12 patients with relapsing hyperparathyroidism after total parathyroidectomy with forearm implant, total ischemic blockade of the arm bearing the parathyroid graft produced a "transitory implantectomy" with a significant reduction of serum levels of intact PTH in those with graft hyperfunction. In 6 patients with proved supernumerary glands, total ischemia of the graft was not followed by significant changes in intact PTH. Hyperparathyroidism was reversed after surgical resection of the parathyroid implants in the 6 patients with positive responses to the ischemic maneuver. A repeat neck reoperation removing cervical or mediastinal supernumerary glands was followed by control of recurrent hyperparathyroidism in the 6 patients with a negative response to the ischemic blockade. Total ischemic blockade of the arm bearing the parathyroid graft is a valuable method for a correct assessment of graft function after total parathyroidectomy with forearm autotransplantation.

[Mediastinal exploration for hyperparathyroidism]. / Exploration médiastinale pour hyperparathyroïdie.

Five per cent of parathyroid adenomas are situated in the mediastinum. Many of them are accessible via a cervical incision: virtually all posterior mediastinal adenomas, by dissecting well behind the oesophagus, and most intrathymic adenomas, by cervical exteriorisation of the thymus. Finally, the indications for sternotomy are are: less than 2% of operations for hyperparathyroidism. After describing the technical details, the authors review the indications, the imaging modalities used and the results of their last 20 sternotomies.

Ventricular septal defect associated with aortic valve incompetence: results of two surgical managements.

Aortic valve insufficiency with ventricular septal defect is usually treated by plication of the commissures. However, long-term deterioration is common. We propose a new technique that corrects the aortic annulus dilatation and the leaflet prolapse and reinforces the sinus of Valsalva. Two groups were compared: group I (29 patients) had plication of the prolapsed leaflet(s) and folding of the free edge; group II (26 patients) had triangular resection of the prolapse cusp, annuloplasty, and reinforcement of the aortic wall. The two groups were similar with regarding to preoperative clinical data. There was no perioperative mortality. Primary failure (aortic valve replacement) occurred in 8 patients in group I (28%) and in 2 patients in group II (8%). The rate of secondary failure was 31% in group I and 4% in group II. The actuarial rate of freedom from reoperation at 5 years is 55% in group I and 88% in group II (p less than 0.05). The late mortality was 6.5% in group I and 10.9% in group II (no difference). We conclude that aortic valve insufficiency with ventricular septal defect is a malformation of the aortic leaflets, the annulus, and the sinus of Valsalva, and that the proposed technique offers a better result than the usual methods in terms of residual aortic valve insufficiency.

[Surgery of endomyocardial fibrosis. Apropos of 32 cases]. / Chirurgie des fibroses endomyocardiques. A propos de 32 cas.

From 1971 to 1984, 32 patients with endomyocardial fibrosis (EMF) were treated by endocardial resection (decortication) and valve replacement. The population consisted in 20 men and 12 women (age ranged from 8 to 64); 19 patients were european and 13 african. All were symptomatic, 78 p. 100 in stages III or IV of the NYHA. Hypereosinophilia was detected in 21 patients and its cause was determined in 11 cases. Cardiac involvement was biventricular in 22 patients and monoventricular in 10 patients. Six patients died in the immediate postoperative period and 6 late deaths were observed, owing to extracardiac causes in 4. There were no recurrence of EMF. Despite a high mortality rate, the authors suggest that all symptomatic EMF should benefit from endocardial resection.

Beta 2-microglobulin amyloidosis: a sternoclavicular joint biopsy study in hemodialysis patients.

The incidence of beta 2-microglobulin deposits appears to increase with time on dialysis. However, the precise prevalence of the disease is not known at present because adequate, noninvasive diagnostic procedures are still lacking. We performed systematic synovial biopsies of the sternoclavicular joint during surgical parathyroidectomy in 22 chronic hemodialysis patients with severe hyperparathyroidism. Nine of the patients proved to have beta 2-microglobulin amyloid deposits as demonstrated by Congo red staining and by immunofluorescence. They had undergone dialysis for longer time periods (12.6 vs 8.5 years, p less than 0.02) and tended to be older than the 13 amyloid-negative patients. They also had a significantly higher body aluminum overload, as demonstrated by a higher increase of plasma aluminum after desferrioxamine infusion. Finally, the presence of Congo-red-positive deposits correlated well with clinical and x-ray findings suggestive of dialysis amyloidosis.

[Hyperparathyroidism with severe hypercalcemia. Treatment and results]. / Hyperparathyroïdisme avec hypercalcémie majeure. Traitement et résultats.

From 1960 to 1988, we successfully treated 1,200 primary hyperparathyroid patients. The serum calcium level was higher than 3.75 mmol/l (150 mg/l) in 75 patients. Sixty-five patients had acute primary hyperparathyroidism and 10 had asymptomatic severe hypercalcemia. Before admission to our department, most of the patients were given medication to lower their serum calcium levels. Analysis of our diagnostic and therapeutic experience leads us to insist upon the importance of prompt medical treatment and rapid surgical intervention. Only surgery can adequately treat severe hyperparathyroidism.

[Vicissitudes of the surgery of primary hyperparathyroidism]. / Vicissitudes de la chirurgie de l'hyperparathyroïdie primaire.

The difficult and disappointing cases encountered in patients who were operated upon for hyperparathyroidism between 1960 and 1987 have been studied. Ninety two patients underwent negative cervicotomy. Among the 12 patients who underwent a second operation, 4 were found to have cervical adenomas and in 6 others mediastinal adenomas were discovered by sternotomy. Most of the other cases were diagnostic errors. The present frequency of negative surgical operations is very low: 2 to 3 per cent of the cases; errors of diagnosis are exceptional, and the unusual sites of adenomas are better known. Seventeen patients were reoperated upon for persistent or recurrent hypercalcaemia despite excision of a 1st parathyroid lesion; 3 cancers which had been mistaken for adenomas at the 1st operation; 6 had a 2nd adenoma which had passed unnoticed during an exploration that was probably too limited and as hypercalcaemia persisted all 6 were reoperated upon with success at different intervals; finally, 8 patients had undiagnosed primary hyperplasia (as part of polyadenomatosis in 5 cases). Recurrences were observed, often after a long period, up to 3 to 5 years. Hypercalcaemia was corrected by the 2nd operation in only 5 out of 8 cases. In retrospect, it was found that the pathological examination could not always determine if the lesion was malignant or distinguish between adenoma and glandular hyperplasia.

Acute primary hyperparathyroidism: experience of 59 cases.

Acute primary hyperparathyroidism is a rare disease with a high mortality rate in some series. Between 1960 and 1986, 1000 patients were treated for primary hyperparathyroidism, among them 59 patients with acute hyperparathyroidism. All these patients were successfully operated on; none of them died before surgery. This study reveals the importance of resuscitation and urgent surgery. It is necessary to rehydrate, to correct electrolyte disorders and to lower serum calcium levels, but it is not necessary to obtain normal serum calcium levels before surgery.

Short-term effects of parathyroidectomy on plasma biochemistry in chronic uremia.

Parathyroidectomy (PTx) is indicated in hemodialysis (HD) patients who have severe osteitis fibrosa unresponsive to vitamin D therapy or in whom the latter treatment is contraindicated. Immediately after PTx, plasma immunoreactive parathyroid hormone, calcium and phosphorus concentrations decline abruptly. However, little is known in such patients about the short-term effects of PTx on plasma alkaline phosphatase (AP) activity and plasma aluminum (Al) levels. The present, preliminary study was performed to determine such parameters in 37 HD patients, and to correlate them with data of bone histology. Mean plasma AP activity started to increase after PTx from day 4 onwards. Thus, AP values significantly higher than pre-PTx values were observed at day 7 and 14 (415 +/- 54 vs. 619 +/- 77 and 749 +/- 83 IU/liter, means +/- SEM; N = 37; P less than 0.05 and 0.001, respectively). This increase, in the absence of changes in liver function, was mainly due to the bone-specific iso-AP. Moreover, the degree of increase in plasma AP activity was higher in the subgroup with negative (group I, 21 patients) than in that with positive bone Al staining (group II, 16 patients). However, plasma osteocalcin (BGP) did not change after PTx (N = 8). Basal plasma Al levels were significantly higher in group II both before and two weeks after surgery.(ABSTRACT TRUNCATED AT 250 WORDS)

[Severe hyperparathyroidism in 2 uremic patients. Diagnostic and therapeutic difficulties]. / Iperparatiroidismo grave in due pazienti uremici. Difficoltà diagnostiche e terapeutiche.

We report here the unusually difficult cases of two patients with end-stage renal failure who suffered from severe hyperparathyroidism requiring surgical correction. The first patient had previously undiagnosed primary hyperparathyroidism. A first surgical neck exploration led to the discovery of four glands, paradoxically normal in size and aspect, which were removed. Subsequently, a supernumerary gland was localized in the mediastinum by computerized tomography and removed via sternotomy. After confirming the hypoparathyroid state, parathyroid autotransplantation was performed using cryopreserved tissue. The second patient had five hyperplastic parathyroid glands removed during the first neck exploration, followed by immediate parathyroid autografting. Because of the persistence of severe hyperparathyroidism, forearm autografts were subsequently removed and a sternotomy performed. Both failed to improve parathyroid hyperfunction. Numerous localization procedures remained negative. A repeat surgical neck exploration was performed because of positive double isotope scanning but was of no success in preventing fatal outcome, as were all medical treatments. These observations of two patients illustrate the difficulties in localizing and removing ectopic parathyroid lesions. Even when relying on the presently available powerful diagnostic means, correction of severe hyperparathyroidism may be extremely difficult.