Anne Hernigou

Peritoneal Implantation of Pheochromocytoma Following Tumor Capsule Rupture During Surgery

CONTEXT Patients with pheochromocytoma (PH) or paraganglioma (PGL) may suffer from tumor persistence or recurrence after resection of the primary tumor. Malignancy and genetic determinants account for the vast majority of these cases, but tumor recurrence or persistence may also arise from tumor spillage during primary resection, followed by peritoneal implantation. We report here five such cases. MATERIALS AND METHODS Thirty-nine of the 181 patients referred to our unit for a PH experienced tumor persistence or recurrence as a result of malignant disease (n = 12), new PGL in a context of genetic determinants (n = 18) or incomplete primary surgical resection (n = 4). Another five patients presenting with adrenal PH could not be categorized into these three groups. RESULTS AND DISCUSSION All five patients (age range 45-63 years) presented evidence of tumor capsule rupture documented upon macroscopic examination or in the surgical report. Initial diagnostic examinations provided no evidence of malignancy. All had a period of apparent remission, lasting from 24 to 106 months. The principal site of recurrence was invariably the peritoneum or the retroperitoneum. Two patients suffered solid organ metastasis, involving the liver (n = 2), bones (n = 1), and lung (n = 1). Therapeutic management involved a combination of (131)I-metaiodobenzylguanidine therapy and surgery. Two patients died due to tumor progression. One patient experienced tumor progression despite surgery. Two patients are currently in a satisfactory condition. CONCLUSION Tumor rupture during surgical resection, with subsequent peritoneal and retroperitoneal dissemination, is a potentially lethal complication of primary pheochromocytoma resection. Even in cases of apparently benign disease, it may lead to peritoneal carcinomatosis and metastatic disease. Complete primary surgery is, therefore, crucial for a good prognosis in PH patients. Furthermore, in cases of tumor rupture, careful follow-up is mandatory, because recurrences may occur after long periods of apparent remission.

Bronchial rupture related to endobronchial stenting in relapsing polychondritis

To the Editor: Relapsing polychondritis is a rare multi-systemic disease characterised by recurrent episodes of inflammation and destruction of cartilaginous structures [1]. Airway involvement by relapsing polychondritis, which results in tracheobronchomalacia and airway stenosis, is associated with a poor prognosis. Several reports have suggested that endobronchial intervention can be beneficial in these subjects [2, 3]. Herein, we describe a case of bronchial rupture related to endobronchial intervention in a patient with relapsing polychondritis. A 47-yr-old male nonsmoker was referred for management of progressive dyspnoea, stridor and cough evolving over 12 months. He had been considered to have severe asthma and was treated with a fixed combination of inhaled corticosteroids and long-acting β-agonists, and short courses of oral corticosteroids. Spirometry showed fixed airflow limitation with a post-bronchodilator forced expiratory volume in 1 s (FEV1)/forced vital capacity of 22% and an FEV1 of 22% predicted. A thoracic computed tomography (CT) scan revealed marked thickening and narrowing of trachea and mainstem bronchi (fig. 1). A diagnosis of relapsing polychondritis was suggested; the patient had no auricular or nasal chondritis and no ocular symptoms. Fluorodeoxyglucose positron emission tomography showed no cartilaginous tracer uptake. Bronchoscopy showed marked thickening and inflammation of airway mucosa. Severe tracheobronchomalacia, …

Complex extralobar sequestration in a 24-year-old woman

Extralobar sequestration is a congenital acquired disease more frequently observed after birth or during infancy. In half of the cases, it is associated with another malformation. In the following case we report the observation of a 24-year-old female with right extralobar sequestration associated with a diaphragmatic hernia and containing gastric mucosa and a congenital cystic adenomatoid lung malformation. Such malformative lung tissue is known to potentially degenerate and justifies surgery even when patients are asymptomatic.

Dysfonctions et paralysies diaphragmatiques : de la physiopathologie au traitement chirurgical

The clinical presentations of diaphragm dysfunctions vary according to etiologies and unilateral or bilateral diseases. Elevation of the hemidiaphragm from peripheral origins, the most frequent situation, requires a surgical treatment only in case of major functional impact. Complete morphological and functional analyses of the neuromuscular chain and respiratory tests allow the best selection of patients to be operated. The surgical procedure may be proposed only when the diaphragm dysfunction is permanent and irreversible. Diaphragm plication for eventration through a short lateral thoracotomy, or sometimes by videothoracoscopy, is the only procedure for retensioning the hemidiaphragm. This leads to a decompression of intrathoracic organs and a repositioning of abdominal organs without effect on the hemidiaphragm active contraction. Morbidity and mortality rates after diaphragm plication are very low, more due to the patients general condition than to surgery itself. Functional improvements after retensioning for most patients with excellent long-term results validate this procedure for symptomatic patients. In case of bilateral diseases, very few bilateral diaphragm plications have been reported. Some patients with diaphragm paralyses from central origins become permanently dependent on mechanical ventilation whereas their lungs, muscles and nerves are intact. In patients selected by rigorous neuromuscular tests, a phrenic pacing may be proposed to wean them from respirator. Two main indications have been validated: high-level tetraplegia above C3 and congenital alveolar hypoventilation from central origin. After progressive reconditioning of the diaphragm muscles following phrenic pacing at thoracic level, more than 90% of patients can be weaned from respirator within a few weeks. This weaning improves the quality of life with more physiological breathing, restored olfaction, better sleep and better speech. The positive impact of diaphragm stimulation has also been evaluated in other degenerative neurological diseases, particularly the amyotrophic lateral sclerosis. For either central or peripheral diaphragm dysfunctions, a successful surgical treatment lies on a strict preoperative selection of patients.

Management of lobar torsion following lung transplantation.

We report 4 cases of acute lobar torsion in patients who had undergone bilateral lung transplantation. Bronchoscopy showed complete obstruction in only 2 of them. Torsion was confirmed by computed tomography in coronal minimal and maximal intensity projections with reconstruction. At operation, 1 detorsion and 3 lobectomies were carried out. Early diagnosis and rapid surgical intervention can save the affected lobe.

Hémoptysie grave, retour veineux pulmonaire anormal partiel et hypertension artérielle pulmonaire

A 73-year-old man with chronic respiratory insufficiency was referred to the intensive care unit because of life threatening haemoptysis and hypoxemia. The patient was in respiratory failure with pulmonary arterial hypertension. The bleeding was issuing from the left inferior lobe. A small arterio-venous fistula was suspected on tomodensitometry. Arterial embolization was contraindicated because of diffuse vascular disease. A left inferior lobectomy was performed. The left superior pulmonary vein was draining into the brachiocephalic vein forming a partial anomalous pulmonary venous return. An anatomic restoration of the venous return to the left atrium was performed. The postoperative course was uneventful. No recurrence of haemoptysis was observed, after 9-month follow-up the vascular anastomosis was patent. Despite the pulmonary vascular bed reduction following lobectomy, echocardiography showed a globally mild improvement of cardiac status that might be attributed to right heart preload amelioration.

Sarcoidosis presenting as severe renin-dependent hypertension due to kidney vascular injury

Renal sarcoidosis embraces a wide variety of clinical patterns. Renal vascular involvement has seldom been reported and usually in the setting of systemic vasculitis. We report the case of a 22-year-old patient in whom inaugural manifestation of renal sarcoidosis consisted of severe hypertension associated with bilateral perfusion defects and tumour-like nodules. In the setting of renal sarcoidosis, our case suggests that renin-dependant hypertension may arise from renal ischaemia as a result of extrinsic compression of kidney blood vessels due to severe granulomatous inflammation.