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Ankylosing spondylitis.

Ankylosing spondylitis is an inflammatory disease involving entheses and joints, especially those in and around the spine. The most widespread involvement of the respiratory system by this disease occurs when this pathological process gives rise to chest wall pain, diminished chest wall movement and a dorsal stoop. As healing of the inflammatory process takes place, calcification occurs which leads to rigidity of these structures, with consequent loss of chest expansion which is exacerbated by the increasingly kyphotic spinal posture and intercostal muscle inefficiency. Fortunately diaphragmatic function is unimpaired and compensates well, so that there are only minor restrictive changes found in tests of respiratory function. Treatment is by mobilizing physiotherapy coupled with a home exercise programme encouraging mobility and improved cardiovascular fitness. As with many physical treatment methods, good quality controlled studies of efficacy are rare. The role of medication is to ease symptoms and hence enable exercise. Apical fibrobullous lung disease is found in a small proportion of AS patients. The initial changes are mainly fibrotic, with bullae becoming more important as the condition progresses. The disease may progress to major cavitation, which is prone to infection, especially with aspergillae. No methods exist which can either prevent the development of fibrobullous disease or halt its progression, although this may happen spontaneously. The main effects of therapy are aimed at the diagnosis and treatment of superinfection. Treatment of established aspergillosis, especially when aspergilloma formation has taken place, is unsatisfactory and carries substantial risks of morbidity and death. Non-apical pleural involvement, including pleural effusion, is very rare. The most common cause of breathlessness in AS patients is cardiac involvement by the disease.

Humans↗

Factors affecting long-term survival after en-bloc resection of lung cancer invading the chest wall.

OBJECTIVE: Several reports emphasize the importance of en-bloc resection as the optimal surgical treatment of lung cancer with chest wall invasion. We investigated possible factors which could affect long-term survival following radical resection of these tumors. METHODS: Between 1981 and 1998, 100 patients (90 male; ten female), with a median age of 60 years (36-84), underwent radical en-bloc resection of non-small cell lung cancer (NSCLC) with chest wall involvement. Patients with superior sulcus tumors invading the thoracic inlet were excluded from this series. There were 43 squamous and 57 non-squamous tumors. The median number of resected ribs was three (1-5). Lung resection included 73 lobectomies, two bilobectomies, 18 pneumonectomies and seven segmentectomies. Chest wall resection also extended to the sternum in one patient, the transverse process in one, the costotransverse foramen and hemivertebrae in two. All patients had a complete resection. Sixty-three patients received postoperative radiotherapy and 12 received chemotherapy. Histological data, including differentiation and depth of chest wall invasion, were carefully reviewed. The effect of various factors on survival were studied. RESULTS: There were four in-hospital deaths. Lymph node involvement was negative on surgical specimens in 65 patients, and 28 patients had positive N1 nodes; the final histology revealed seven N2 diseases. Chest wall invasion was limited to the parietal pleura in 29 patients and included intercostal muscles, bones and extrathoracic muscles in 67, 24 and seven cases, respectively. The overall 2-year survival rate was 41%. The 5-year survival for patients with N0, N1 and N2 disease was 22, 9 and 0%, respectively. A local recurrence occurred in 13 patients, with four having a new resection and 45 patients developing systemic metastases. The nodal status (N0-1 vs. N2; P=0. 026) and the number of resected ribs(<2 vs. >2; P=0.03) were survival predictors in univariate analysis. By multivariate analysis, the two independent factors affecting long-term survival were the histological differentiation (well vs. poorly differentiated; P=0. 01) and the depth of chest wall invasion (parietal pleura vs. others; P=0.024). CONCLUSIONS: Histological differentiation and depth of chest wall involvement were the main factors affecting long-term survival in this series. The role of induction chemotherapy for tumors with poor prognosis should be investigated.

Adenocarcinoma↗

Gastrointestinal stromal tumor of the posterior mediastinum.

Gastrointestinal stromal tumor (GIST) is a rare, but potentially aggressive tumor. We present an asymptomatic 64-year-old man with an incidental 9-cm GIST that arose in the posterior mediastinum. Wide surgical excision was performed with rotation of an intercostal muscle flap to buttress a surgically created esophageal wall defect. The patient is now free of disease 26 months postoperative. This tumor is defined by the carcinogenic over-expression of KIT-protein, a tyrosine kinase receptor. Accurate diagnosis of gastrointestinal stromal tumor is imperative, as specific medical therapy is now available for potential control of recurrent or metastatic disease.

Follow-Up Studies↗

Impact of different coverage techniques on incidence of postpneumonectomy stump fistula.

OBJECTIVE: Postpneumonectomy bronchial stump fistula (PBSF) is a serious complication with a reported incidence between 0 and 12%. The aim of this retrospective study was to investigate the effectiveness of different coverage techniques of the bronchial stump applied in a consecutive series of pneumonectomies in avoiding this particular problem. METHODS: Between 1/87 and 10/97, 129 patients (90 male, 39 female, mean age 57.8 years, range: 15-78 years) underwent pneumonectomy by one surgeon (W.K.). In 14 patients, additional resection procedures were performed (aorta n = 6, vena cava n = 5, thoracic wall n = 3). In all patients with malignancies (n = 123), mediastinal lymphadenectomy was routinely added to the procedure. Bronchial stump closure was performed by means of stapling devices in all patients. Coverage of the bronchial stump was performed with a generous pedicled pericardial flap and concomitant reconstruction of the pericardium with Vicryl mesh (n = 50), with a portion of the posterior pericardium (n = 16), with the azygos vein (n = 12), with surrounding mediastinal tissue (n = 25), with pleura (n = 16), or with intercostal muscle flap (n = 3); no coverage at all was performed in seven patients. In all patients with high risk for development of PBSF, i.e. patients who received any form of neoadjuvant therapy or had extended resections, the pericardial flap technique was used. RESULTS: Perioperative mortality was 5.4% (n = 7) and five patients (3.9%) experienced significant perioperative complications, with one of them directly related to the method of bronchial stump coverage (cardiac tamponade due to the use of a too small Vicryl mesh for reconstruction of the pericardium). Follow-up was 96.1% complete (five patients were lost to follow-up). Fourty-seven patients (36.4%) died late after operation (mean 19+/-13 months, median 17 months), mainly due to recurrence of their underlying malignant disease. PBSF occurred in one patient only (0.8%), 2 weeks after operation (coverage with pleura). No PBSF was seen in the long term follow-up period. CONCLUSION: Coverage of the bronchial stump contributes to a low incidence of PBSF. In view of the fact, that this serious complication was completely avoided in the pericardial flap group (used in patients with expected higher risk for PBSF), this particular technique seems to offer the best results.

Adolescent↗

Comparison of omentum, pleura and diaphragm for tracheal autograft survival in rats.

Pleura, diaphragm, pericardial fat pad, intercostal muscles and omentum can be used to protect and revascularize the bronchial suture line. To compare the efficiency of pleura, diaphragm and omentum, an experimental study was designed. Heterotopic tracheal autotransplantation was performed in 15 rats. Animals were divided into three groups; omentum, diaphragm and pleura. Tracheal Segment Necrosis Scoring System was used for pathologic examinations. Pleural and diaphragmatic groups showed the least necrosis and there was significant statistical difference among these groups and omental group (P < 0.05). Our study showed that pleura and diaphragm could be used as safely as omentum for protection and survival of bronchial suture lines.

Analysis of Variance↗

Patterns of palmar skin temperature alterations during transthoracic endoscopic T2 sympathectomy for palmar hyperhidrosis.

Transthoracic endoscopic T2 sympathectomy has been widely applied to the treatment of a variety of sympathetically mediated disorders. Palmar hyperhidrosis is probably the most common indication for thoracic sympathectomy, especially in certain subtropical areas. Which sympathetic ganglion is to be ablated and how extensive such ablation is enough to eliminate palm sweating are two important issues. Intraoperative monitoring of palmar skin temperature (PST) is the most frequently used method for assessing the accuracy as well as adequacy of ablation of the target sympathetic ganglia. With continuous monitoring of bilateral PST during the operative course of T2 sympathectomy, it was possible to depict the alterations of bilateral PST in response to specific surgical procedures in a real-time manner. For each case, a PST graph was obtained, which represented the graphical expression of intraoperatively recorded bilateral PST data plotted against time. The PST graphs of 93 consecutive cases were analysed. Three types of PST graphs existed, reflecting different responses of bilateral PST to different surgical procedures during the operation. In Type I PST graph pattern, found in 58 cases, skin incision and intercostal muscle dissection caused dramatic bilateral PST drop; and unilateral T2 sympathectomy induced synchronous bilateral PST elevation. Twenty-four cases demonstrated Type II PST graph pattern, in which unilateral T2 sympathectomy caused only ipsilateral PST elevation, although the PST-depressing effect of skin incision and muscle dissection was as significant as in Type I graph pattern. In the 11 cases who showed Type III PST graph pattern, neither skin incision nor T2 sympathectomy induced any apparent changes of PST on either side, giving rise to two rather flat PST curves on the PST graphs. These findings implicate that reciprocal interactions between bilateral sympathetic activities exist in the majority of cases, and that crossover sympathetic modulation may play a role in the neural control of the sudomotor and vasomotor activities of the palms. This study also provides information regarding how PST would possibly change following specific surgical procedures during transthoracic endoscopic T2 sympathectomy, which may be of importance to those who use intraoperative PST monitoring as a guide in determining whether or not the correct sympathetic ganglia are ablated for adequate sympathetic denervation of the palms.

Adolescent↗

Detection of spontaneous schwannomas by MRI in a transgenic murine model of neurofibromatosis type 2.

Spontaneous schwannomas were detected by magnetic resonance imaging (MRI) in a transgenic murine model of neurofibromatosis type 2 (NF2) expressing a dominant mutant form of merlin under the Schwann cell-specific P0 promoter. Approximately 85% of the investigated mice showed putative tumors by 24 months of age. Specifically, 21% of the mice showed tumors in the intercostal muscles, 14% in the limb muscles, 7% in the spinal cord and spinal ganglia, 7% in the external ear, 14% in the muscle of the abdominal region, and 7% in the intestine; 66% of the female mice had uterine tumors. Multiple tumors were detected by MRI in 21% of mice. The tumors were isointense with muscle by T1-weighted MRI, showed strong enhancement following administration of gadolinium-DTPA, and were markedly hyperintense by T2-weighted MRI, all hallmarks of the clinical manifestation. Hematoxylin and eosin staining and immunohistochemistry indicated that the tumors consisted of schwannomas and Schwann cell hyperplasias. The lesions stained positively for S-100 protein and a marker antigen for the mutated transgenic NF2 protein, confirming that the imaged tumors and areas of hyperplasia were of Schwann cell origin and expressed the mutated NF2 protein. Tumors were highly infectable with a recombinant herpes simplex virus type 1 vector, hrR3, which contains the reporter gene, lacZ. The ability to develop schwannoma growth with a noninvasive imaging technique will allow assessment of therapeutic interventions.

Animals↗

Angiosarcoma of the chest wall.

Angiosarcoma is a rare but highly malignant disease. This report presents a 22-year-old male who has survived a primary angiosarcoma arising from the chest wall. The patient had been complaining of anterior chest pain for 3 years. CT and MRI revealed a tumor protruding into the right thoracic cavity from the anterior chest wall but no further findings. First, the tumor was resected using video-assisted thoracoscopy. The pathological diagnosis established from a frozen section was of a benign mesothelioma, but this was changed to angiosarcoma based on findings from a paraffin-embedded specimen. As a consequence, the third rib and intercostal muscles were resected in a second operation. Three years following surgery there was no evidence of tumor recurrence or metastasis. This case supports a suggestion that not all patients with angiosarcomas experience the same level of malignancy.

Adult↗

[High-resolution magnetic resonance tomography (HR-MRT) of the pleura and thoracic wall: normal findings and pathological changes].

AIM: To determine the value of high-resolution MRI in pleural and chest wall diseases, the normal and pathologic costal pleura and adjacent chest wall between paravertebral and the axillary region were examined with contrast enhanced high-resolution T1-weighted MRI images using a surface coil. MATERIAL AND METHODS: Normal anatomy was evaluated in 5 healthy volunteers and a normal specimen of the thoracic wall, and correlation was made with corresponding HR-CT and histologic sections. CT-proved focal and diffuse changes of the pleura and the chest wall in 36 patients underwent HR-MRI, and visual comparison of MRI and CT was done retrospectively. RESULTS: Especially sagittal T1-weighted HR-MRI images allowed accurate delineation of the peripleural fat layer (PFL) and the innermost intercostal muscle (IIM), which served as landmarks of the intact inner chest wall. PFL and IIM were well delineated in 3/4 patients with tuberculous pleuritis, and in all 7 patients with non-specific pleuritis, as opposed to impairment of the PFL and/or the IIM, which was detected in 15/18 malignancies as a pattern of malignant chest wall involvement. In one case of tuberculous pleural empyema with edema of the inner chest wall HR-MRI produced false positive diagnosis of malignant disease. CONCLUSION: HR-MRI images improved non-invasive evaluation of pleural and chest wall diseases, and allowed for differentiation of benign and malignant changes.

Adult↗

Cough induced stress fracture and arthropathy of the ribs at extreme altitude.

Cough and chest wall pain at high altitude have only received passing mention in the medical literature. Increased minute ventilation of cold dry air at very high altitude is likely to cause airway irritation. This in turn may result in airway drying, mucus production, postnasal drip from vasomotor rhinitis, and bronchospasm acting individually or in combination to stimulate the vagal cough reflex. The cough is exacerbated further at extreme altitudes above 5500 m, and may result in intercostal muscle strain and single or multiple rib fractures. We present a case of multiple cough induced stress fractures and arthropathy documented by technetium-99 bone scan in a high altitude climber and suggest the addition of the term High Altitude Cough Syndrome (HACS) to the medical syntax to identify this discrete medical problem of exposure to very high altitude.

Adult↗

Kinetic parameters for acetylcholine interaction in intact neuromuscular junction.

The dependency of miniature endplate current (mepc) rise time upon mepc amplitude and acetylcholine receptor site density was measured in lizard intercostal muscles and used to fit the rate constants in a simple kinetic scheme. The kinetic scheme included diffusion, two-step sequential binding of acetylcholine to receptor, and opening of the ion channel. Numerical simulation of the observed mepc behavior yielded the following kinetic constants; (i) diffusion constant, 4 X 10(-6) cm2 sec-1; (ii) forward binding rates, 4.7 X 10(7) M-1 sec-1; (iii) channel relaxation rate, 25 msec-1. The value above for the forward binding rates assumed both rates to be equal. If they are different, the slower of the two is in the range of 2-5 X 10(7) M-1 sec-1. A radial profile of bound receptor indicated that activation of the receptor was very local, occurring essentially within a radius of about 0.3 micrometers from the point of acetylcholine release.

Acetylcholine↗

Acetylcholinesterase of mammalian neuromuscular junctions: presence of tailed asymmetric acetylcholinesterase in synaptic basal lamina and sarcolemma.

A sarcolemma-rich fraction can be isolated after subcellular fractionation of mouse intercostal muscles by sedimentation on a discontinuous sucrose gradient. The quantitative recovery of the acetylcholine receptor in this fraction is about 50%, which indicates the presence of a high proportion of postsynaptic membranes. Acetylcholinesterase (AcChoEase; EC 3.1.1.7) is found mainly in three different layers: the top layer, which contains soluble AcChoEase, the intermediate layer (fraction A), and the last, AcChoR-rich, layer (fraction C). The relative proportions of the molecular forms of AcChoEase are different in the three layers. The "16S" AcChoEase is in a higher proportion in both types of membrane fractions (A and C) compared to soluble AcChoEase. Both total AcChoEase and 16S AcChoEase are enriched in the A and C fractions. In the C fraction, the sequential use of homogenizations in the presence of detergent and high ionic strength allows the "solubilization" of two distinct AcChoEase pools. One is detergent-soluble and mainly composed of slow-sedimenting forms; the other one is detergent-insoluble, high-ionic strength-soluble, and composed mainly of collagen-like, tailed, asymmetric (16S) AcChoEase. Thus, most of the asymmetric AcChoEase is specifically localized in the synaptic extracellular matrix of the mammalian muscle fiber. However, in the A fraction, most of the 16S AcChoEase found is solubilized by detergent alone, suggesting an association with microsomal membranes. It may mean that at least some of the basal lamina-embedded 16S AcChoEase is preassembled intracellularly in the sarcoplasmic reticulum.

Acetylcholinesterase↗

Electron microscopic localization of acetylcholinesterase and nonspecific cholinesterase at the neuromuscular junction by the gold-thiocholine and gold-thiolacetic acid methods.

By means of the gold-thiocholine (AuThCh) and gold-thiolacetic acid (AuThAc) methods, it has been demonstrated electron microscopically that acetylcholinesterase (AChE) is located at the prejunctional axoplasmic membrane and the postjunctional sarcoplasmic membrane, including the full lengths of its invaginations, at the motor end plate of mouse intercostal muscle. Nonspecific cholinesterase (ChE) is present in relatively low concentrations at the same sites, and in greater concentrations in the teloglial Schwann sheath cells. Significant amounts of reaction product appeared in the junctional cleft only after prolonged incubation with both methods. The identification of AChE and ChE was confirmed by the use of appropriate concentrations of several selective inhibitors. In confirmation of previous studies by light microscopy, the AuThCh method is more specific for AChE and ChE, whereas the AuThAc method allows greater accuracy of localization.

Acetates↗

A diazo coupling method for the electron microscopic localization of cholinesterase.

The presence of cholinesterase at the myoneural junction of intercostal muscle has been demonstrated in both light and electron microscopic preparations. A new simultaneous diazo coupling technique using alpha-naphthyl acetate as substrate and "hexazonium pararosanilin" as coupler has been applied to cold formalin-fixed tissues. After postfixation in buffered osmium tetroxide the sites of esterase activity are faithfully demonstrated at a high level of resolution. The details of cholin-esterase distribution and some technical aspects of the procedure are discussed.

Biological Phenomena↗

Interpleural local anaesthesia: anatomical basis for mechanism of action.

India ink was injected through an extradural catheter placed in the right interpleural space in cadavers at autopsy. The lung was removed and the spread of the ink noted and photographed. In six cadavers, block sections were cut at the anterior axillary line through three ribs, intercostal muscles and parietal pleura. The blocks were fixed in formalin and then decalcified, sectioned, stained and mounted on slides. In all cases the ink covered the chest wall, lung and diaphragm, and was seen to pool in the paravertebral area. Examination of the histological slides revealed black pigment on the pleural surface and in the subpleural space. The mechanism of transport across the parietal pleura is discussed.

Adult↗

A new mitochondrial myopathy. Biochemical studies revealing a deficiency in the cytochrome b-c1 complex (complex III) of the respiratory chain.

A 20-year-old Chilean girl presented with lifelong ptosis and fatiguable weakness which was initially thought to be due to a congenital myasthenic syndrome. Studies of an intercostal muscle biopsy showed normal endplate morphology, abundant acetylcholinesterase activity and a normal number of junctional acetylcholine receptors as determined by radiochemical assay, but a high proportion of the muscle fibres contained large peripheral aggregations of abnormal mitochondria. Biochemical investigation of mitochondria isolated from the vastus lateralis muscle demonstrated a hitherto unreported respiratory chain deficiency localized to complex III.

Adult↗

Surgical management of primary lung cancer in an elderly patient with preoperative empyema.

A 74-year-old man with primary lung cancer developed preoperative empyema but was successfully managed surgically. The patient was given a diagnosis of c-T2N1M0, stage IIB, moderately differentiated squamous cell carcinoma, but before surgery pneumothorax and empyema developed, resulting from rupture of the carcinoma. Thoracic drainage, lavage and systemic administration of antibiotics improved his empyema. As there were no malignant cells in the drainage fluid, right middle-lower bilobectomy, empyemal cavity resection and lymph node dissection were performed. The bronchial stump was covered with an intercostal muscle flap. Thoracic drainage, lavage and systemic administration of antibiotics were performed for 6 days following the operation. The patient was discharged on the 27th postoperative day without any complications having developed. The pathological diagnosis of the tumor was p-T4N2(#7)M0, stage IIIB, br(-), ly(+), v(+), p3(pleura), pm1 and d0. He died of recurrence at home 18 months after the operation. We believe the following to be the minimum requirements for surgical management of such patients: (1) immediate thoracic cavity drainage and lavage with systemic antibiotic therapy, aiming at infection control before surgery; (2) prophylactic lavage of the thoracic cavity during and after surgery and (3) coverage of the bronchial stump with an adequate flap. Six reported cases of primary lung cancer with preoperative empyema are also discussed.

Aged↗