Unilateral diaphragmatic paralysis: inspiratory muscles, breathlessness and exercise capacity.

BACKGROUND: Patients with unilateral diaphragmatic paralysis (UDP) may present with dyspnoea without specific cause and limited ability to exercise. We aimed to investigate the diaphragm contraction mechanisms and nondiaphragmatic inspiratory muscle activation during exercise in patients with UDP, compared with healthy individuals. METHODS: Pulmonary function, as well as volitional and nonvolitional inspiratory muscle strength were evaluated in 35 patients and in 20 healthy subjects. Respiratory pressures and electromyography of scalene and sternocleidomastoid muscles were continuously recorded during incremental maximal cardiopulmonary exercise testing until symptom limitation. Dyspnoea was assessed at rest, every 2 min during exercise and at the end of exercise with a modified Borg scale. MAIN RESULTS: Inspiratory muscle strength measurements were significantly lower for patients in comparison to controls (all p<0.05). Patients achieved lower peak of exercise (lower oxygen consumption) compared to controls, with both gastric (-9.8±4.6 cmH2O versus 8.9±6.0 cmH2O) and transdiaphragmatic (6.5±5.5 cmH2O versus 26.9±10.9 cmH2O) pressures significantly lower, along with larger activation of both scalene (40±22% EMGmax versus 18±14% EMGmax) and sternocleidomastoid (34±22% EMGmax versus 14±8% EMGmax). In addition, the paralysis group presented significant differences in breathing pattern during exercise (lower tidal volume and higher respiratory rate) with more dyspnoea symptoms compared to the control group. CONCLUSION: The paralysis group presented with exercise limitation accompanied by impairment in transdiaphragmatic pressure generation and larger accessory inspiratory muscles activation compared to controls, thereby contributing to a neuromechanical dissociation and increased dyspnoea perception.

Unilateral diaphragm paralysis: a dysfunction restricted not just to one hemidiaphragm.

BACKGROUND: Most patients with unilateral diaphragm paralysis (UDP) have unexplained dyspnea, exercise limitations, and reduction in inspiratory muscle capacity. We aimed to evaluate the generation of pressure in each hemidiaphragm separately and its contribution to overall inspiratory strength. METHODS: Twenty-seven patients, 9 in right paralysis group (RP) and 18 in left paralysis group (LP), with forced vital capacity (FVC) < 80% pred, and 20 healthy controls (CG), with forced expiratory volume in 1 s (FEV1) > 80% pred and FVC > 80% pred, were evaluated for lung function, maximal inspiratory (MIP) and expiratory (MEP) pressure measurements, diaphragm ultrasound, and transdiaphragmatic pressure during magnetic phrenic nerve stimulation (PdiTw). RESULTS: RP and LP had significant inspiratory muscle weakness compared to controls, detected by MIP (- 57.4 ± 16.9 for RP; - 67.1 ± 28.5 for LP and - 103.1 ± 30.4 cmH2O for CG) and also by PdiTW (5.7 ± 4 for RP; 4.8 ± 2.3 for LP and 15.3 ± 5.7 cmH2O for CG). The PdiTw was reduced even when the non-paralyzed hemidiaphragm was stimulated, mainly due to the low contribution of gastric pressure (around 30%), regardless of whether the paralysis was in the right or left hemidiaphragm. On the other hand, in CG, esophagic and gastric pressures had similar contribution to the overall Pdi (around 50%). Comparing both paralyzed and non-paralyzed hemidiaphragms, the mobility during quiet and deep breathing, and thickness at functional residual capacity (FRC) and total lung capacity (TLC), were significantly reduced in paralyzed hemidiaphragm. In addition, thickness fraction was extremely diminished when contrasted with the non-paralyzed hemidiaphragm. CONCLUSIONS: In symptomatic patients with UDP, global inspiratory strength is reduced not only due to weakness in the paralyzed hemidiaphragm but also to impairment in the pressure generated by the non-paralyzed hemidiaphragm.

Low-grade Endometrial Stromal Sarcoma with Unusual Metastasis to the Skull

Background Endometrial stromal sarcomas (ESSs) are the second most common uterine sarcomas. Although ESSs are often indolent, they have metastatic potential. To the best of our knowledge, there are only three reports of brain metastasis, and the present report is the first to describe a late skull metastasis of an ESS. Case Report We describe the case of a 51-year-old woman who presented abnormal vaginal bleeding 14 years ago; she was diagnosed with an uterine mass and submitted to a hysterectomy. One year ago she presented ESS lung metastasis followed by a left parietal calvarial metastasis. The optimal treatment for metastatic ESS is controversial, but the use of progesterone and aromatase inhibitors is advisable.

Introdução Sarcoma endometrial estromal (SEE) é a segunda lesão mais frequente dentre os sarcomas uterinos. Geralmente são lesões indolentes, mas com potencial de desenvolver metástase. Até o momento há apenas três relatos de metástase cerebral, sendo este o primeiro estudo a descrever uma metástase craniana tardia dessas lesões. Relato de caso Nós descrevemos o caso de uma paciente de 51 anos de idade que apresentou há 14 anos um quadro de sangramento vaginal anormal, sendo diagnosticada uma massa uterina; a paciente foi submetida a uma histerectomia. Há um ano ela evoluiu com metástase pulmonar, seguida por metástase craniana parietal esquerda. O tratamento ideal do SEE metastático ainda é controverso, mas o uso de inibidores de aromatase é aconselhável.

Thoracoscopic approach of the internal thoracic lymphatic chain.

OBJECTIVE: Few studies to date have evaluated the videothoracoscopic approach of the internal thoracic lymphatic chain. However, the histological evaluation of lymph nodes is essential for patients with breast cancer who show lymph node uptake at scintigraphy in the preoperative period and also for patients with lymphoma who have exclusive uptake in these lymph nodes at positron emission tomography for recurrence assessment. Our goal was to evaluate the safety and the change in oncologic approach through this minimally invasive technique. METHODS: This is a review of the prospectively collected data in a group of patients undergoing thoracoscopic biopsy of the thoracic lymphatic chain in patients with breast cancer and lymphoma carried out in our institution between September 2010 and June 2012. The analyzed variables include age, histological type, operated hemithorax, number of resected lymph nodes, neoplastic involvement of the resected lymph nodes, duration of chest tube drainage, length of hospital stay, and perioperative mortality. Complications such as subcutaneous emphysema and necessity for blood transfusion were also evaluated. RESULTS: Thoracoscopic biopsy was successfully performed in 16 patients, of whom 14 had breast cancer and 2 had lymphoma. The surgical biopsy results changed the treatment in the two patients with lymphoma and avoided radiotherapy in the eight patients with breast cancer who did not have metastases. CONCLUSIONS: The use of the thoracoscopic assessment of the internal thoracic lymphatic chain seems safe and resulted in therapy modification in more than half of our patients, suggesting that it is an effective technique for staging in selected breast cancer and lymphoma cases.

Pleural effusion following ovarian hyperstimulation.

Ovarian hyperstimulation syndrome (OHSS) is an iatrogenic complication that occurs in the luteal phase of an induced hormonal cycle. In most cases, the symptoms are self-limited and spontaneous regression occurs. However, severe cases are typically accompanied by acute respiratory distress. The objective of the present study was to describe the clinical presentation, treatment, and outcome of pleural effusion associated with OHSS in three patients undergoing in vitro fertilization. The patients ranged in age from 27 to 33 years. The onset of symptomatic pleural effusion (bilateral in all cases) occurred, on average, 43 days (range, 27-60 days) after initiation of hormone therapy for ovulation induction. All three patients required hospitalization for massive fluid resuscitation, and two required noninvasive mechanical ventilation. Although all three patients initially underwent thoracentesis, early recurrence of symptoms and pleural effusion prompted the use of drainage with a pigtail catheter. Despite the high output from the pleural drain (mean, 1,000 mL/day in the first week) and prolonged drainage (for 9-22 days), the outcomes were excellent: all three patients were discharged from hospital. Although pleural effusion secondary to OHSS is probably underdiagnosed, the associated morbidity should not be underestimated, especially because it affects potentially pregnant patients. In this study, early diagnosis and appropriate supportive measures yielded favorable results, limiting the surgical approach to adequate pleural drainage.

Derrame pleural secundário à hiperestimulação ovariana / Pleural effusion following ovarian hyperstimulation

A síndrome de hiperestimulação ovariana (SHEO) é uma complicação iatrogênica que ocorre na fase lútea de um ciclo hormonal induzido. Na maioria dos casos, os sintomas são autolimitados e regridem espontaneamente. Entretanto, casos graves comumente cursam com desconforto respiratório agudo. O objetivo deste estudo foi descrever a apresentação clínica, o tratamento e os desfechos de derrame pleural associado a SHEO em três pacientes submetidas a fertilização in vitro. A idade das pacientes variou de 27 a 33 anos, e o aparecimento do derrame pleural sintomático (bilateral em todos os casos) ocorreu, em média, 43 dias (variação: 27-60 dias) após o início da terapia hormonal para a indução da ovulação. Todas as pacientes necessitaram de internação hospitalar para reposição volêmica maciça, e duas delas necessitaram de ventilação mecânica não invasiva. Embora todas as pacientes tenham sido inicialmente submetidas à toracocentese, a recidiva precoce dos sintomas e do derrame pleural fez com que se optasse pela drenagem pleural com cateter do tipo pigtail. Apesar do alto débito de drenagem (média de 1.000 mL/dia na primeira semana) e do tempo de drenagem prolongado (9-22 dias), os desfechos foram excelentes (alta hospitalar). Embora o derrame pleural secundário a SHEO seja provavelmente subdiagnosticado, a morbidade associada não deve ser subestimada, principalmente devido a seus efeitos em pacientes potencialmente gestantes. Nesta série de casos, o diagnóstico precoce e as medidas de suporte clínico adequadas permitiram uma evolução favorável, limitando a abordagem cirúrgica a uma drenagem pleural adequada.

Ovarian hyperstimulation syndrome (OHSS) is an iatrogenic complication that occurs in the luteal phase of an induced hormonal cycle. In most cases, the symptoms are self-limited and spontaneous regression occurs. However, severe cases are typically accompanied by acute respiratory distress. The objective of the present study was to describe the clinical presentation, treatment, and outcome of pleural effusion associated with OHSS in three patients undergoing in vitro fertilization. The patients ranged in age from 27 to 33 years. The onset of symptomatic pleural effusion (bilateral in all cases) occurred, on average, 43 days (range, 27-60 days) after initiation of hormone therapy for ovulation induction. All three patients required hospitalization for massive fluid resuscitation, and two required noninvasive mechanical ventilation. Although all three patients initially underwent thoracentesis, early recurrence of symptoms and pleural effusion prompted the use of drainage with a pigtail catheter. Despite the high output from the pleural drain (mean, 1,000 mL/day in the first week) and prolonged drainage (for 9-22 days), the outcomes were excellent: all three patients were discharged from hospital. Although pleural effusion secondary to OHSS is probably underdiagnosed, the associated morbidity should not be underestimated, especially because it affects potentially pregnant patients. In this study, early diagnosis and appropriate supportive measures yielded favorable results, limiting the surgical approach to adequate pleural drainage.

Giant multilocular thymic cyst in an HIV-infected adolescent.

A girl with vertically acquired HIV infection presented with a 6-month history of dyspnea and chest pain. Computed tomography of the thorax showed a heterogenous mass measuring 13 × 9 × 17 cm located in the anterior mediastinum. Complete surgical resection was accomplished with no complications. The final diagnosis was multilocular thymic cyst, a distinct pathologic entity that is morphologically distinguishable and unrelated to congenital thymic cyst.

Hemangioma of the rib.

An asymptomatic 48-year-old woman presented to our hospital with a tumor of the rib incidentally diagnosed on a chest roentgenogram. The patient was investigated and underwent tumor resection of the chest wall. The pathologic study revealed that it was cavernous hemangioma. This tumor of the bone is a distinctly uncommon benign vascular tumor, generally occurring in the spine or skull. Hemangiomas involving the rib are even more rare, with only 22 cases described in the literature. However, we suggest that this tumor of the rib should be considered in the differential diagnosis, principally in asymptomatic patients.

Leiomyomatous hamartoma of the posterior mediastinum.

A posterior mediastinal mass was found in a 72-year-old man who complained of progressive shortness of breath. Computed tomography of the thorax showed a well-defined, heterogenous mass measuring 9.5 x 4.0 x 3.0 cm, located in the right costovertebral angle. Complete surgical resection was accomplished through a right posterolateral thoracotomy. The final diagnosis was a leiomyomatous hamartoma of the posterior mediastinum. This is an unusual location for this type of tumor, and it has rarely been reported in the literature.

Surgical treatment of congenital tracheal stenoses.

OBJECTIVE: To analyze the outcomes of patients undergoing repair of congenital tracheal stenosis. METHODS: This was a retrospective review of congenital tracheal stenosis patients treated between 2001 and 2007 at the University of São Paulo School of Medicine Hospital das Clínicas in São Paulo, Brazil. RESULTS: Six boys and one girl (age at diagnosis ranging from 28 days to 3 years) were included. Five of the patients also had cardiac or major vessel malformations. The stenosis length was short in three patients, medium in one and long in three. The techniques used were pericardial patch tracheoplasty in three patients, resection and anastomosis in two, slide tracheoplasty in one and vascular ring correction in one. One patient died during surgery due to hypoxia and hemodynamic instability, and one died from septic shock on postoperative day 11. Other complications included pneumonia, arrhythmia, stenosis at the anastomosis level, residual stenosis, granuloma formation and malacia. The mean follow-up period was 31 months; four patients were cured, and one required the use of a T tube to maintain airway patency. CONCLUSIONS: Congenital tracheal stenosis is a curable disease. However, its repair is complex and is associated with high rates of morbidity and mortality.

Tratamento cirúrgico das estenoses traqueais congênitas / Surgical treatment of congenital tracheal stenoses

OBJETIVO: Analisar os desfechos dos pacientes submetidos ao reparo de estenose congênita de traqueia. MÉTODOS: Análise retrospectiva dos pacientes com estenose traqueal congênita tratados no Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo entre 2001 e 2007. RESULTADOS: Seis meninos e uma menina (idade ao diagnóstico entre 28 dias e 3 anos) foram incluídos. Cinco pacientes apresentavam malformações intracardíacas e/ou de grandes vasos associadas. A extensão das estenoses foi curta em três pacientes, média em um e longa em três. As técnicas utilizadas foram traqueoplastia com enxerto de pericárdio em três pacientes, ressecção e anastomose em dois, traqueoplastia em bisel em um e correção de anel vascular em um. Um paciente morreu no intraoperatório por hipóxia e instabilidade hemodinâmica e outro no 11º dia pós-operatório por choque séptico. Outras complicações observadas foram pneumonia, arritmia, estenose na anastomose e estenose residual, malácia e formação de granulomas. O tempo médio de seguimento pós-operatório foi de 31 meses; quatro pacientes ficaram livres da doença e um necessitou de tubo T para manter a via aérea pérvia. CONCLUSÕES: A estenose congênita de traqueia é uma doença curável. Entretanto, seu reparo é complexo e está associado a taxas de morbidade e mortalidade significativas.

OBJECTIVE: To analyze the outcomes of patients undergoing repair of congenital tracheal stenosis. METHODS: This was a retrospective review of congenital tracheal stenosis patients treated between 2001 and 2007 at the University of São Paulo School of Medicine Hospital das Clínicas in São Paulo, Brazil. RESULTS: Six boys and one girl (age at diagnosis ranging from 28 days to 3 years) were included. Five of the patients also had cardiac or major vessel malformations. The stenosis length was short in three patients, medium in one and long in three. The techniques used were pericardial patch tracheoplasty in three patients, resection and anastomosis in two, slide tracheoplasty in one and vascular ring correction in one. One patient died during surgery due to hypoxia and hemodynamic instability, and one died from septic shock on postoperative day 11. Other complications included pneumonia, arrhythmia, stenosis at the anastomosis level, residual stenosis, granuloma formation and malacia. The mean follow-up period was 31 months; four patients were cured, and one required the use of a T tube to maintain airway patency. CONCLUSIONS: Congenital tracheal stenosis is a curable disease. However, its repair is complex and is associated with high rates of morbidity and mortality.

Pulmonary artery sarcoma mimicking a pulmonary artery aneurysm.

Pulmonary artery sarcoma is an uncommon neoplasm, and its clinical and radiological presentation usually simulates chronic thromboembolic disease. We present the case of a 77-year-old woman admitted with dyspnea, chest pain, and hemoptysis. A chest computed tomographic scan showed moderate right-sided pleural effusion and a saccular dilatation of the interlobar portion of the right pulmonary artery, which was filled with contrast and surrounded by an irregular soft-tissue attenuation mass, suggesting a ruptured pulmonary artery aneurysm. The patient was operated on. Intraoperatively, a pseudoaneurysm and a solid mass were identified within the oblique fissure around the interlobar artery. Therefore, a right pneumonectomy was performed. Definitive pathologic examination was consistent with pulmonary artery sarcoma. The patient had a good outcome and is free of disease 2 years after surgery.