Osmotic gradient ektacytometry: A valuable screening test for hereditary spherocytosis and other red blood cell membrane disorders.

INTRODUCTION: New generation osmotic gradient ektacytometry has become a powerful procedure for measuring red blood cell deformability and therefore for the diagnosis of red blood cell membrane disorders. In this study, we aim to provide further support to the usefulness of osmotic gradient ektacytometry for the differential diagnosis of hereditary spherocytosis by measuring the optimal cutoff values of the parameters provided by this technique. METHODS: A total of 65 cases of hereditary spherocytosis, 7 hereditary elliptocytosis, 3 hereditary xerocytosis, and 171 normal controls were analyzed with osmotic gradient ektacytometry in addition to the routine red blood cell laboratory techniques. The most robust osmoscan parameters for hereditary spherocytosis diagnosis were determined using receiver operating characteristic curve analysis. RESULTS: The best diagnostic criteria for hereditary spherocytosis were the combination of decreased minimal elongation index up to 3% and increased minimal osmolality point up to 5.2% when compared to the mean of controls. Using this established criterion, osmotic gradient ektacytometry reported a sensitivity of 93.85% and a specificity of 98.38% for the diagnosis of hereditary spherocytosis. CONCLUSION: Osmotic gradient ektacytometry is an effective diagnostic test for hereditary spherocytosis and enables its differential diagnosis with other red blood cell membrane diseases based on specific pathology profiles.

[Lung surgery in haematological patients: useful? hazardous?]. / Chirurgie pulmonaire chez le malade d'hématologie : pourquoi ? Pour qui ?

Respiratory complications are frequent in haematological patients. Lung surgery, either for diagnosis or treatment, is considered useful but hazardous in these patients. We performed a reappraisal study of this purpose; retrospective study in a university centre, located in the Paris area, France. We analysed the entire records of all the haematological patients admitted in the Thoracic Surgery department from October 2001 to January 2009, among 400 haematological patients with pulmonary complications admitted to the Respiratory Diseases department. Seventeen patients (male: n=13, mean age 47 ± 15 years) underwent lung surgery. Underlying haematological disease was acute (n=7) or chronic (n=5) leukaemia, lymphoma (n=4), and eight have had stem cell transplantation. Thirteen patients had been exposed to a cytotoxic chemotherapy with known pulmonary toxicity. Respiratory diseases have been evolving for 112 days (10-663 days), and 14 patients received previously antibiotic and/or antifungal therapy. One patient was neutropenic and three had thrombopenia. Five patients underwent curative surgery for a residual pulmonary nodule after medical treatment of invasive aspergillosis, and 12 had a diagnostic procedure (open lung biopsy by video-assisted thoracoscopy [n=2]; thoracotomy [n=8]). Surgery permitted a final diagnosis in all 12 cases: non-specific infiltrative pneumonia (n=4), pulmonary graft versus host disease (n=3), granulomatous pneumocystosis (n=1), invasive aspergillosis (n=1), bronchial carcinoma (n=1), EBV-related lymphoproliferation (n=1), and tuberculosis (n=1). Therapeutic regimens were modified according to the surgical results in ten cases (84%). All patients were extubated at the end of surgery. Post-operative complications were: prolonged air leaks (n=3), pneumonia (n=1), parenchymal hematoma (n=1), acute coronary syndrome (n=1). Seven patients were admitted in the Intensive Care Unit, and five had non-invasive ventilation. Three patients died from respiratory failure: NSIP (n=2), pneumocystosis (n=1). Lung surgery for selected haematological patients has two indications: (1) curative surgery, for a residual pulmonary nodule after medical treatment of invasive aspergillosis; (2) diagnostic procedure, leading frequently to modifications of therapeutic regimens, with low rate of complications, in highly selected patients.

[Pulmonary cystic adenomatoid malformation in an adult patient: an underdiagnosed disease]. / Malformation kystique adénomatoïde pulmonaire de l'adulte : une pathologie méconnue.

Currently, most congenital lower respiratory tract malformations are detected during pregnancy or at birth, thanks to antenatal imaging. However, a pulmonary congenital cystic adenomatoid disease may be found in adulthood. The diagnosis is difficult, due to its rarity. We present the case of a patient whose diagnosis of pulmonary cystic adenomatoid malformation was confirmed when she had tuberculosis. A lobectomy was performed, which enabled identification of tuberculosis and also multiple cysts of adenomatoid malformation. The risk posed by this malformation, i.e. the risk of developing bronchioloalveolar carcinoma and of infection or pneumothorax, is the incentive for proposing formal surgical removal.

Hipertensión intracraneal asociada a trombosis de senos venosos cerebrales y mastoiditis. A propósito de dos casos pediátricos / Intracranial hypertensión associated with cerebral venous sinus thrombosis and mastoiditis. Two paediatric case reports

Introducción. La trombosis de senos venosos cerebrales es una entidad poco frecuente, en general asociada a procesos agudos, entre los que destaca la mastoiditis. En aproximadamente la mitad de los casos, cualquiera que sea su etiología, pueden detectarse trastornos protrombóticos asociados. Habitualmente los pacientes presentan clínica de cefalea, convulsiones y disminución de la conciencia, siendo este último factor de mal pronóstico. Casos clínicos. Presentamos dos pacientes pediátricos con clínica de hipertensión intracraneal en el contexto de mastoiditis como complicación de una otitis media aguda, en los que se identificó una trombosis de senos venosos cerebrales. Ambos se trataron con punciones lumbares evacuadoras y tratamiento diurético. En el segundo paciente se añadió tratamiento anticoagulante. No obstante, ante la persistencia del cuadro de hipertensión intracraneal, este paciente precisó una derivación ventriculoperitoneal. Conclusiones. El método diagnóstico de elección es la resonancia magnética con venografía, si bien, en caso de urgencia, la tomografía computarizada puede orientar el diagnóstico en un tercio de los casos. Además del tratamiento sintomático, se acepta la anticoagulación (grado de evidencia 1B) para evitar la progresión (AU)

Introduction. Cerebral venous sinus thrombosis is a rare condition that is generally associated with acute processes, one of the most important being mastoiditis. Associated prothrombotic disorders can be detected in approximately half of all cases, regardless of the aetiology. Patients usually present clinical symptoms like headache, convulsions and diminished level of awareness, this latter factor being linked to a poor prognosis. Case reports. We report the cases of two paediatric patients with a clinical picture of intracranial hypertension within a context of mastoiditis as a complication of an acute ear infection, in whom cerebral venous sinus thrombosis was identified. Both patients were treated with lumbar punctures to evacuate erebrospinal fluid and diuretic therapy. Anticoagulant therapy was added in the case of the second patient. Nevertheless, because of the persistence of the symptoms of intracranial hypertension, a ventriculoperitoneal shunt had to be performed in this patient. Conclusions. The preferred diagnostic method is magnetic resonance imaging with venography, although in an emergency computerised tomography can help reach a diagnosis in a third of cases. In addition to treating the symptoms, anticoagulation is also accepted (degree of evidence 1B) to prevent further progression (AU)

[Intracranial hypertension associated with cerebral venous sinus thrombosis and mastoiditis. Two paediatric case reports]. / Hipertensión intracraneal asociada a trombosis de senos venosos cerebrales y mastoiditis. A propósito de dos casos pediátricos.

INTRODUCTION: Cerebral venous sinus thrombosis is a rare condition that is generally associated with acute processes, one of the most important being mastoiditis. Associated prothrombotic disorders can be detected in approximately half of all cases, regardless of the aetiology. Patients usually present clinical symptoms like headache, convulsions and diminished level of awareness, this latter factor being linked to a poor prognosis. CASE REPORTS: We report the cases of two paediatric patients with a clinical picture of intracranial hypertension within a context of mastoiditis as a complication of an acute ear infection, in whom cerebral venous sinus thrombosis was identified. Both patients were treated with lumbar punctures to evacuate cerebrospinal fluid and diuretic therapy. Anticoagulant therapy was added in the case of the second patient. Nevertheless, because of the persistence of the symptoms of intracranial hypertension, a ventriculoperitoneal shunt had to be performed in this patient. CONCLUSIONS: The preferred diagnostic method is magnetic resonance imaging with venography, although in an emergency computerised tomography can help reach a diagnosis in a third of cases. In addition to treating the symptoms, anticoagulation is also accepted (degree of evidence 1B) to prevent further progression.

Surgical management of non-small cell lung cancer with synchronous brain metastases.

OBJECTIVES: Published series on the synchronous combined resection of brain metastases and primary non-small cell lung cancer are small and scarce. We therefore undertook a multicenter retrospective study to determine long-term survival and identify potential prognostic factors. DESIGN: Our series includes 103 patients who were operated on between 1985 and 1998 for the following tumors: adenocarcinomas (74); squamous cell carcinomas (20); and large cell carcinomas (9). Three patients had two brain metastases, and one patient had three metastases; the remaining patients had a single metastasis. Ninety-three patients presented with neurologic signs that regressed completely after resection in 60 patients and partially, in 26 patients. Neurosurgical resection was incomplete in six patients. Seventy-five patients received postoperative brain radiotherapy. The time interval between the brain operation and the lung resection was < 4 months. Pulmonary resection was incomplete in eight patients. RESULTS: The survival calculated from the date of the first operation was 56% at 1 year, 28% at 2 years, and 11% at 5 years. Univariate analysis showed a better prognosis for adenocarcinomas (p = 0.019) and a trend toward a better prognosis for patients with small pulmonary tumors (T1 vs T3, p = 0.068), N0 stage disease (N0 vs N+, p = 0.069), and complete pulmonary resection (p = 0.057). In a multivariate analysis, adenocarcinoma histology also affected the survival rate (p = 0.03). CONCLUSIONS: It seems legitimate to proceed with lung resection after complete resection of a single brain metastasis, at least in patients with an adenocarcinoma and a small lung tumor and without abnormal mediastinal lymph nodes seen on the CT scan or during mediastinoscopy.

Surgical treatment of lung cancer invading the chest wall: results and prognostic factors.

BACKGROUND: The study was performed to assess prognostic factors in patients with lung cancer invading the chest wall treated by surgery. METHODS: We reviewed retrospectively clinical records of all patients operated on for lung cancer invading chest wall structures between 1984 and 1998. RESULTS: Two hundred one patients were operated on in this 14-year period. One hundred thirty-seven lobectomies, 55 pneumonectomies, and 9 wedge resections were performed. Extrapleural resection (when invasion was limited to the parietal pleura) and chest wall resection (in the case of invasion of deeper structures) were combined with pulmonary resection in 79 (39%) and 122 (61%) cases, respectively. Pathologic TNM stages were T3N0 in 116 (57.5%) cases, T3N1 in 52 (26%), T3N2 in 27 (13.5%), and T4N0-N1 in 6 (3%). A complete resection was achieved in 167 (83%) cases. Fourteen postoperative deaths (7%) occurred. One hundred thirty-nine patients (74%) underwent postoperative radiotherapy. Actuarial 5-year survival was 24% and 13% after complete and incomplete resection, respectively (p < 0.05). Actuarial 5-year survival after complete resection was 25% in T3N0 patients, 20% in T3N1, and 21% in T3N2. In completely resected patients, univariate and multivariate analyses identified three independent prognostic factors: nodal involvement, depth of parietal invasion, and age. Radiation therapy did not improve survival if a complete resection was possible. CONCLUSIONS: Completeness of resection, nodal involvement, depth of invasion, and age affect survival of patients with lung cancer invading the chest wall. N2 disease should not be considered a contraindication to surgery.

Management of empyema complicating lobectomy with superior vena cava replacement.

We present the case of a 49-year-old man with right upper lobe adenocarcinoma invading the right brachiocephalic vein and the origin of the superior vena cava. En bloc resection of right upper lobe with the involved venous segments was carried out through a median sternotomy. Venous pathway was reestablished with a Gore-Tex (W.L. Gore & Assoc, Flagstaff, AZ) prosthesis. Postoperative course was marked by right pneumonia complicated by empyema. The patient underwent thoracotomy with completion pneumonectomy and latissimus dorsi transposition to cover both the prosthesis and the bronchial stump, as well as to fill the cavity. A favorable outcome was observed and long-term survival achieved.

Open window thoracostomy followed by intrathoracic flap transposition in the treatment of empyema complicating pulmonary resection.

OBJECTIVE: Successful treatment of postoperative empyema remains a challenge for thoracic surgeons. We report herein our 12-year experience in the management of this condition by means of open window thoracostomy. METHODS: Open window thoracostomy was used in the treatment of 46 patients with empyema complicating pulmonary resection. A bronchopleural fistula was associated in 39 of 46 cases. Previous operations included pneumonectomy (n = 30), bilobectomy (n = 5), lobectomy (n = 9), and wedge resection (n = 2) performed for benign (n = 10) or malignant (n = 36) disease. In 10 patients open window thoracostomy was definitive because of patient death (n = 2), concomitant major illness (n = 2), tumor recurrence (n = 4), spontaneous closure (n = 1), or patient choice (n = 1). In 36 cases intrathoracic flap transposition was eventually performed. Muscular (n = 29), omental (n = 5), or combined muscular and omental (n = 2) flaps were used to obliterate the thoracostomy cavity and to close a possibly associated bronchopleural fistula. In 9 patients with postpneumonectomy cavities too wide to be filled by the available flaps, a limited thoracoplasty represented an intermediate step. RESULTS: Among patients treated with definitive open window thoracostomy, local control of the infection was achieved in all the survivors (8/8). After open window thoracostomy and subsequent flap transposition, success (definitive closure of the thoracostomy and, if present, of the bronchopleural fistula) was achieved in 27 (75. 0%) of 36 patients. Four initial failures could be salvaged by means of reoperation (initial reopening of thoracostomy and subsequent muscular or omental transposition). CONCLUSION: Open window thoracostomy followed by intrathoracic muscle or omental transposition represents a valid therapeutic option in patients with empyema complicating pulmonary resections.

Extended resections for bronchogenic carcinoma invading the superior vena cava system.

BACKGROUND: Extended resection of non-small-cell lung cancer (NSCLC) involving the superior vena cava (SVC) system is infrequently performed and oncologic benefits are still uncertain. METHODS: From 1983 to 1996, 25 patients underwent resection of the SVC system for T4, NSCLC. RESULTS: A total of 12 pneumonectomies (48%), ten lobectomies (40%), and three wedge resections (12%) were performed. Seven patients had complete resection of the SVC with graft interposition, 12 patients underwent tangential resection of the SVC, and 1 patient had a pericardial patch; 5 patients underwent resection of right innominate and subclavian veins without vessel reconstruction. The lymph node status was N0 in 8 patients (32%), N1 in 3 (12%) and N2 in 14 patients (56%). Five patients (20%) underwent incomplete resection. Nine patients (36%) developed postoperative complications (36%) that were fatal in 3 patients (12%). At the completion of the study, 10 patients were still alive. The median survival was 11.5 months and the 5-year actuarial survival rate was 29%, with 4 patients alive at 5 years. CONCLUSIONS: The resection of the SVC system for direct involvement by T4, NSCLC can be performed in selected patients with an acceptable postoperative mortality. Even though no significant prognostic factors were observed, the patients who required a lobectomy with limited lymph node involvement seemed to benefit the most from surgery.

Lymphangiohemangioma of the mediastinum.

Lymphangiohemangiomas of the mediastinum are exceedingly rare. Clinically they tend to behave like lymphangiomas. We report 3 new cases. All 3 cases showed both lymphatic and vascular components. One case was associated with a left superior vena cava. In both other cases computed tomography with dynamic scans demonstrated the vascular components. Combined lymphatic venous malformation was thus evidenced in the 3 cases and supports that lymphangiohemangiomas are malformative in origin.

[Mucoepidermoid tracheobronchial tumors. Apropos of a series of 11 cases]. / Tumeurs muco-épidermoïdes trachéobronchiques. A propos d'une série de 11 cas.

Mucoepidermoid tumours (TME) are rare tumours which develop at the level of the submucous bronchial glands of the tracheobronchial tree. The majority of these tumours develop in a benign fashion but some of them are malign. Amongst these many are probably confused with adenosquamous bronchial cancers. We have reviewed eleven patients suffering from TME who were observed over a period of twelve years. Two of these tumours were at the level of the trachea: nine others were at the level of the bronchial cartilaginous trachea. Seven of these tumours had the macroscopic and histological criteria of low grade malignancy and four corresponded to those tumours said to show high grade malignancy. The only death concerned a patient with a tracheal tumor of high grade malignancy but the death occurred immediately after laser therapy to relieve obstruction in a patient with acute asphyxia. None of the other patients died of tumour progression and the longest follow up (eleven years of survival) involved a patient with a bronchial form and a high grade malignancy with glandular invasion. Even mucoepidermoid tumours of high grade malignancy have a good prognosis and it is a cardinal point to clearly distinguish these forms from adenosquamous cancers. Nevertheless it has been suggested that adenosquamous and mucoepidermoid carcinomas could have a common origin and be the extremes of the same overall disorder just as the image we have of neuroendocrine tumours whose spectrum extends from carcinoid tumours to small cell cancers.

[Techniques of correction of partial right abnormal pulmonary venous return associated with atrial septal defect]. / Techniques de correction des retours veineux pulmonaires anormaux partiels droits associés à une communication interauriculaire.

Many techniques have been described for correcting partial right anomalous pulmonary venous drainage to avoid the possible complications of stenosis of the systemic or pulmonary venous return, residual shunt or arrhythmias. Between 1985 and 1994, 33 patients aged 1 to 69 years underwent repair of this malformation. The anomalous drainage was situated at the cavo-atrial junction or above in 25 cases and to the right atrium in 8 cases. Depending on the level of the drainage of the anomalous pulmonary veins, the size of the superior vena cava, the site of atrial septal defect and the age of the patient, 3 techniques were used: simple tunneling, tunneling with widening of the superior vena cava by a patch, tunneling with section of the superior vena cava and its transposition to the right atrium. There was no hospital mortality. Postoperative echocardiography showed a minimal residual shunt which regressed at the two months control examination. No cases of restriction of the systemic or pulmonary venous return were observed. Six patients developed arrhythmias during the hospital period. At the end of follow-up, all patients were asymptomatic without residual shunts or restriction of venous drainage. Persistent arrhythmias were observed in one case (3%). There were no differences in the results of the three techniques used. By using the most appropriate technique of repair for the anatomical form allows correction of this malformation with the minimal number of postoperative complications.

[Chylomediastinum after mediastinoscopy. Apropos of a case]. / Chylomédiastin après médiastinoscopie. A propos d'une observation.

A mediastinal chylous effusion occurred in a young woman after a mediastinoscopy. This effusion healed rapidly with medical treatment. An understanding of the anatomy of these intra-thoracic/lymphatic channels explains that intra-thoracic chylous effusions may occur in case of incontinence of the lymphatic vessels which connect the tracheo-bronchial nodes to the thoracic duct in the mediastinum. This is very important following the surgical excision of a node because incontinent lymphatic vessels are directly sectioned. When the lymph nodes are biopsied in an enclosed cavity such as the mediastinum, the quantity of lymphoid tissue acts as an obstacle to reflux of the lymph which is thus less significant and more easily controlled.