Optimal NIV Medicare Access Promotion: Patients With Thoracic Restrictive Disorders: A Technical Expert Panel Report From the American College of Chest Physicians, the American Association for Respiratory Care, the American Academy of Sleep Medicine, and the American Thoracic Society.

The existing coverage criteria for noninvasive ventilation (NIV) do not recognize the benefits of early initiation of NIV for those with thoracic restrictive disorders and do not address the unique needs for daytime support as the patients progress to ventilator dependence. This document summarizes the work of the thoracic restrictive disorder Technical Expert Panel working group. The most pressing current coverage barriers identified were: (1) delays in implementing NIV treatment; (2) lack of coverage for many nonprogressive neuromuscular diseases; and (3) lack of clear policy indications for home mechanical ventilation (HMV) support in thoracic restrictive disorders. To best address these issues, we make the following key recommendations: (1) given the need to encourage early initiation of NIV with bilevel positive airway pressure devices, we recommend that symptoms be considered as a reason to initiate therapy even at mildly reduced FVCs; (2) broaden CO2 measurements to include surrogates such as transcutaneous, end-tidal, or venous blood gas; (3) expand the diagnostic category to include phrenic nerve injuries and disorders of central drive; (4) allow a bilevel positive airway pressure device to be advanced to an HMV when the vital capacity is < 30% or to address severe daytime respiratory symptoms; and (5) provide additional HMV when the patient is ventilator dependent with use > 18 h per day. Adoption of these proposed recommendations would result in the right device, at the right time, for the right type of patients with thoracic restrictive disorders.

Thoracic endometriosis syndrome in Nigeria: a single-centre experience.

OBJECTIVES: Thoracic endometriosis syndrome (TES) is the presence of functional endometrial tissue in or around the lung. There seem to be differences in the clinical presentation of this condition among Nigerian patients. We aim to study the clinical presentation and management outcome of TES in our centre. METHODS: This is an analysis of consecutive patients with TES treated over a 5-year period and followed up for 6 months to 5 years. Information collected included the gynaecological history, clinical presentation, causes of misdiagnosis, modalities of treatment and outcome. RESULTS: Twenty-three patients with TES aged between 24 and 45 years (median 32 years) were treated. Severe dysmenorrhoea was a prominent symptom in 91.3% of cases (median dysmenorrhoea score 8) and was uninfluenced by the marital status (P = 0.522). The patients usually presented with massive or recurrent haemothorax associated with massive ascites [16/23 (69.5%) of cases (P = 0.0006)]. The right side alone was involved in 21 cases and 1 patient had catamenial haemoptysis as a part of her symptoms, even though there was bronchial bleed at bronchoscopy in 6 patients. In 40%, tuberculosis was the misdiagnosis. Diagnosis was established histologically in 18/23 (78.3%) of the cases. Treatment was multimodal and multidisciplinary with notable macroscopic lesions in 77.8% of the patients that had surgery. CONCLUSIONS: TES is not an uncommon lesion. Presentation with massive haemothorax is usually associated with massive ascites. A large percentage of such have pleural and diaphragmatic lesions that require surgical treatment. The ascites may be refractory to treatment requiring repeated paracentesis.

Refractory thoracic endometriosis.

Thoracic endometriosis syndrome (TES) is a rare entity caused by thoracic implantation of endometrial tissue, manifesting as catamenial pneumothorax, pleural effusion and haemoptysis in young female individuals. Its management and long-term prevention of recurrences, can be challenging. We present the case of a young woman who presented with recurrent pneumothorax, haemopneumothorax and pleural effusion. The diagnosis of TES was confirmed based on cytological findings of pleural fluid. She underwent treatment with mechanical pleurodesis twice but continued to have recurrences. Hormonal treatment failed to produce a satisfactory resolution. She underwent chemical pleurodesis, which successfully induced remission of her TES. A review of the literature suggests that chemical pleurodesis produces better results compared with mechanical pleurodesis and that hormonal treatment with gonadotropin-releasing hormone agonists is effective at preventing recurrences.

A Randomized Trial of Initiation of Chronic Noninvasive Mechanical Ventilation at Home vs In-Hospital in Patients With Neuromuscular Disease and Thoracic Cage Disorder: The Dutch Homerun Trial.

BACKGROUND: There is an increasing demand for home mechanical ventilation (HMV) in patients with chronic respiratory insufficiency. At present, noninvasive ventilation is exclusively initiated in a clinical setting at all four centers for HMV in the Netherlands. In addition to its high societal costs and patient discomfort, commencing HMV is often delayed because of a lack of hospital bed capacity. RESEARCH QUESTION: Is HMV initiation at home, using a telemonitoring approach, noninferior to in-hospital initiation in a nationwide study? STUDY DESIGN AND METHODS: We conducted a nationwide, randomized controlled noninferiority trial, in which every HMV center recruited 24 patients (home [n = 12] vs hospital [n = 12]) with a neuromuscular disease or thoracic cage disorder, all with an indication to start HMV. Change in arterial CO2 (Paco2) over a 6-month period was considered the primary outcome, and quality of life and costs were assessed as secondary outcomes. RESULTS: A total of 96 patients were randomized, most of them diagnosed with neuromuscular disease. We found a significant improvement in Paco2 within both groups (home: from 6.1 to 5.6 kPa [P < .01]; hospital: from 6.3 to 5.6 kPa [P < .01]), with no significant differences between groups. Health-related quality of life showed significant improvement on various subscales; however, no significant differences were observed between the home and hospital groups. From a societal perspective, a cost reduction of more than €3,200 ($3,793) per patient was evident in the home group. INTERPRETATION: This nationwide, multicenter study shows that HMV initiation at home is noninferior to hospital initiation, as it shows the same improvement in gas exchange and health-related quality of life. In fact, from a patient's perspective, it might even be a more attractive approach. In addition, starting at home saves over €3,200 ($3,793) per patient over a 6-month period. TRIAL REGISTRY: ClinicalTrials.gov; No.: NCT03203577; URL: www.clinicaltrials.gov.

[Haemothorax revealing thoracic endometriosis]. / Hémothorax spontané révélant une endométriose pleurale.

INTRODUCTION: Thoracic endometriosis (TE) is a rare disorder affecting women during their reproductive years. Manifestations of TE include pneumothorax and haemothorax. Treatment is based on surgical and hormonal therapy that aims at eradicating existing endometrial thoracic plaques and to prevent reseeding from pelvic endometriosis. CASE REPORT: We report the case of a 36 year-old young woman presenting thoracic endometriosis revealed by a recurring spontaneous, large and isolated right haemothorax. Diagnosis, pathogeny and treatment are discussed. CONCLUSION: Thoracic endometriosis needs to be considered as a cause of haemothorax in women of childbearing age.

Unmasking catamenial hemoptysis in the era of CFTR modulator therapy.

BACKGROUND: Thoracic endometriosis syndrome (TES) is a rare condition that occurs in women when endometriosis implants into the thoracic cavity. Catamenial hemoptysis, the occurrence of hemoptysis with menstruation, is a recognized clinical manifestation of TES commonly treated with hormonal therapy. CASE SUMMARY: We present the first documented case describing the recrudescence of catamenial hemoptysis in the setting of Lumacaftor/Ivacaftor administration in a 25-year-old woman with cystic fibrosis (CF). DISCUSSION: We review the literature on TES, pharmacologic management, and reported cystic fibrosis transmembrane conductance regulator (CFTR) modulator drug interactions. We propose that our patient's recrudescence of catamenial hemoptysis was secondary to a drug-drug interaction between Lumacaftor/Ivacaftor and oral contraceptive therapy. CONCLUSION: Our case suggests that women with CF who have catamenial hemoptysis and a genetic mutation approved for Tezacaftor/Ivacaftor or Elexacaftor/Tezacaftor/Ivacaftor can be managed effectively with either CFTR modulator and hormonal contraceptive therapy.

Thoracic endometriosis-related non-catamenial pneumothorax with peculiar histological findings.

Thoracic endometriosis-related non-catamenial pneumothorax is a rare entity whose pathogenesis is still less unclear than catamenial pneumothorax one. Hormonal therapy and/or talc pleurodesis are not sufficient for successful management. Surgical videothoracoscopic resection has a central role in the treatment. We displace a case of thoracic endometriosis-related non-catamenial pneumothorax presenting with recurrent right pneumothorax, surgically treated three times and misdiagnosed at first two interventions. At third operation, unusual histological findings on diaphragmatic and pulmonary specimens were disclosed. These results could partially clarify the presentation of some complicated misdiagnosed cases. More has to be investigated about pathogenesis of the disease and influence of the hormonal balance on it.

Non-invasive ventilation during surgery under neuraxial anaesthesia: a pathophysiological perspective on application and benefits and a systematic literature review.

Unlike general anaesthesia, neuraxial anaesthesia (NA) reduces the burden and risk of respiratory adverse events in the post-operative period. However, both patients affected by chronic obstructive pulmonary disease (COPD) and chest wall disorders and/or neuromuscular diseases may experience the development or the worsening of respiratory failure, even during surgery performed under NA; this latter negatively affects the function of accessory respiratory muscles, resulting in a blunted central response to hypercapnia and possibly in an exacerbation of cardiac dysfunction (NA-induced relative hypovolemia). According to European Respiratory Society (ERS) and American Thoracic Society (ATS) guidelines, non-invasive ventilation (NIV) is effective in the post-operative period for the treatment of both impaired pulmonary gas exchange and ventilation, while the intra-operative use of NIV in association with NA is just anecdotally reported in the literature. Whilst NIV does not assure a protected patent airway and requires the patient's cooperation, it is a handy tool during surgery under NA: NIV is reported to be successful for treatment of acute respiratory failure; it may be delivered through the patient's home ventilator, may reverse hypoventilation induced by sedatives or inadvertent spread of anaesthetic up to cervical dermatomes, and allow the avoidance of intubation in patients affected by chronic respiratory failure, prolonging the time of non-invasiveness of respiratory support (i.e., neuromuscular patients needing surgery). All these advantages could make NIV preferable to oxygen in carefully selected patients.

Spine Deformity With Fused Ribs Treated With Proximal Rib- Versus Spine-Based Growing Constructs.

STUDY DESIGN: Retrospective review of prospectively collected data. OBJECTIVE: To compare the use of spine-based versus rib-based implants for the treatment of early-onset scoliosis (EOS) in the setting of rib fusions. SUMMARY OF BACKGROUND DATA: Treatment for severe early-onset spinal deformity with rib fusions includes growing spine devices with proximal rib or spine anchors. The results of treatment, however, have not been compared between spine-based versus rib-based proximal anchors. METHODS: 169 patients with rib fusions treated with rib-based or spine-based constructs and minimum two-year follow-up were included. Sixteen patients were treated with proximal spine-based anchors and 153 with proximal rib-based devices (VEPTRs). Overall, 104 of the patients with rib-based fixation underwent thoracoplasty at the index surgery. We evaluated change in T1-T12 and T1-S1 height, coronal Cobb angle, kyphosis, and number of lengthening/revision surgeries. RESULTS: Kyphosis increased a mean of 7° in the rib-based group and decreased a mean of 20 degrees in the spine-based group (p = .002). Major Cobb angle decreased in both groups (p < .0001); however, the spine-based group had greater Cobb angle improvement (24 vs. 11 degrees, p = .04). From implant and lengthening of distraction devices, there was a mean 3.3-cm (22%) increase in T1-T12 height and a mean of 8.0 lengthenings in the rib-based group compared with a 5.7-cm increase and 6.3 lengthening surgeries in the spine-based group. Patients with rib-based constructs had a mean of 11 total procedures, whereas spine-based patients had a mean of 8. CONCLUSIONS: Patients underwent a mean of eight lengthening surgeries before final fusion or cessation of lengthening with a modest 2.3-cm increase in T1-T12 height. Compared with proximal rib anchors, proximal spine anchors controlled kyphosis and improved Cobb angle correction for early-onset scoliosis with rib fusions.

Sudden Death Due to Recent Hemorrhage From an Intrathoracic Chronic Expanding Hematoma.

Chronic expanding hematoma (CEH) is a rare disease that can develop in any region of the body, but it most frequently develops in the thorax. When intrathoracic CEH is left untreated, gradually expanding hematoma can be life-threatening, leading to respiratory failure or hemoptysis. We encountered an 89-year-old man with cardiopulmonary arrest on arrival. He had been healthy, and it was unclear whether CEH had previously been detected. A very large mass was observed on chest computed tomography (CT), but the cause of death could not be determined. In the autopsy, this mass was identified as CEH and no malignant findings were noted. A fresh hemorrhage had occurred in the hematoma and perforated the bronchial lumen, which caused airway obstruction/asphyxia and resulted in sudden death. CEH should be suspected when a very large tumorous lesion occupying the entire hemithorax is observed on chest imaging, and it is important to recognize that sudden death can occur in the natural course of CEH.

[Catamenial pneumothorax: an intriguing cause of recurrent pneumothorax in women]. / Catameniale pneumothorax.

BACKGROUND: Thoracic endometriosis (TE) is one of the causes of secondary pneumothorax in women. According to the literature, 1 in 3 premenopausal women with pneumothorax can be diagnosed with 'catamenial pneumothorax'. The diagnosis is often not or only belatedly made in practice, even though treatment is significantly different than that of primary pneumothorax. CASE DESCRIPTION: A 40-year-old woman came to the emergency department because of dyspnoea and right-sided chest pain. The patient had recurrent pneumothorax and chest pain related to the menstrual cycle. Thoracoscopy revealed thoracic endometriosis. The endometriosis lesions were removed and the patient subsequently received hormonal menstrual suppression treatment. CONCLUSION: In premenopausal women with pneumothorax or a recurrence of pneumothorax, it is important to consider catamenial pneumothorax. Infertility, earlier proven abdominal endometriosis and chest pain linked to menstruation are indications of thoracic endometriosis or catamenial pneumothorax.