Linfagioleomiomatose pulmonar em paciente pediátrico: relato de caso / Pulmonar lymphagioleiomatosis in a pediatric patient: a case report

A linfangioleiomiomatose pulmonar (LAM) é considerada uma doença rara, com predileção de acometimento em mulheres durante período reprodutivo, principalmente entre a terceira e quarta década de vida. A doença é atualmente reconhecida como uma neoplasia de baixo grau e se caracteriza por proliferação de células musculares lisas atípicas no parênquima pulmonar. Dispneia, pneumotórax recorrente e tosse seca são as manifestações clínicas mais comuns. Em exames de imagem, é notada comumente a existência de hiperinsuflação, infiltrado intersticial reticulonodular e cistos de diferentes tamanhos em topografia pulmonar. A presença concomitante de angiomiolipomas pode ser verificada em alguns pacientes. Tais achados radiológicos, quando associados ao quadro clínico característico , falam a favor do diagnóstico de LAM, sendo possível a confirmação pela histopatologia. A despeito das diferentes formas de tratamento existentes para a LAM, ainda não existe medida curativa para a doença. Em geral, os pacientes evoluem com contínua deterioração da função pulmonar, com predomínio de um distúrbio ventilatório obstrutivo que deve ser diferenciado de outras afecções mais prevalentes, como asma e doença pulmonar obstrutiva crônica. Neste trabalho, relata-se um caso de paciente em idade pediátrica, com presença de achados clínicos e radiológicos compatíveis com o diagnóstico de LAM, ainda que fora da faixa etária usual de acometimento da doença.

Pulmonary Lymphangioleiomyomatosis (LAM) is a rare disease which affects women during reproductive age mainly between thirdies and forties. Currently the disease is recognized with a low grade neoplasm and it is characterized by proliferation of atypical smooth muscle cells in the lung parenchyma. Dyspnea, recurrent pneumothorax and dry cough are the most common clinical manifestations. On imaging studies, the presence of hyperinsuflation, interstitial infiltrates reticulonodular and cysts of different sizes in lung topography is common. The concomitant presence of angiomyolipoma can be recorded in some patients. When associated with the usual clinical condition, the radiological findings suggest the diagnosis of LAM and it can be confirmed by histopathology. Despite of different forms of treatment for LAM, there is still no curative method for it. In general, the patients develop continuous deterioration of lung function with a predominance of obstructive lung disorder which must be distinguished from other more prevalent diseases, such as asthma and chronic obstructive pulmonary disease. In this paper, we report a case of a child with the presence of clinical and radiological findings compatible with the diagnosis of LAM although it is outside the usual age of onset of the disease.

Recurrent spontaneous pneumothorax during pregnancy managed conservatively: a case report

A 36-year-old lady presented with four episodes of right sided pneumothorax during pregnancy requiring multiple chest drain insertion. It was complicated with persistent air leak despite low pressure high volume suction applied to the chest drainage. She delivered safely through spontaneous vaginal delivery with chest drainage. Further imaging by high resolution computed tomography (HRCT) scan of thorax done revealed bilateral scattered pulmonary cysts and sub pleural bullae and was later followed up with respiratory unit. She had no further episodes of pneumothorax postpartum. This case highlights the vital importance of prompt recognition and management of pneumothorax in pregnancy as the patient involved is at higher risk for acute respiratory failure leading to maternal and/or foetal mortality. It is essential for early involvement of obstetric team and to expedite the delivery for a better perinatal and maternal outcome.

Surgical management of recurrent pneumothorax in the second trimester of pregnancy / 대한주산의학회잡지

Spontaneous pneumothorax in pregnancy is generally regarded as an unusual disorder, with only approximately 44 cases having been reported in the world literature. The most common cause is the rupture of a subpleural apical bulla or bleb, due to increased respiratory demand of the peripartum period. Pneumothorax should be considered in any pregnant woman with chest pain and/or dyspnea and must be confirmed radiographically. Treatment of simple pneumothorax during pregnancy is controversal. Admission and close observation of the patient is usually done with small pneumothorax. Other treatment options are needle aspiration, needle decompression (eg, intension pneumothorax), pleurodesis, tube thoracostomy, thoracotomy, and thoracoscopy. We report a recent experience of a 34 years-old pregnant woman with recurrent pneumothorax, who was treated with thoracotomy during the 28th weeks of pregnancy. She had previously been well during pregnancy and all antenatal investigations, including ultrasound scan, were normal. Cardiovascular examination did not reveal any abnormality. On chest auscultation, air entry was reduced on the left side of the chest. Chest X-ray revealed significantly expanded left lung with a large pneumothorax. After successfully treated with surgical approach, the patient had vaginal delivery of a healthy male infant, weighing 2.93 kg, safely during 39th weeks of pregnancy.

Preliminary remark about the value of thoracoscopy in diagnosis and treatment recurrent pleurorrhea and pneumothorax

Thoracoscopy is a new techique and it has not been widenly applied yet in Vietnam. During a period of time from 09/2003 to 08/2004, the researcher had thoracoscopied for 17 patients. Among them, 14 patients with pleural effusion (6males, 8 females, the ages from 20 to 80). The patients were bronchoscopy and pleural biopsy by Castelain needles for 2 to 3 times but not yet definitive diagnosis. Thoracoscopy biopsy has effective in histological diagnosis on 10/14 (72%) patients. Among the patients, cancer patients was 57%, tuberculars were 15%. There isn’t any complication.

The Effect of Pleurodesis with Doxycycline in the Rabbit / 결핵

BACKGROUND: The intrapleural instillation of tetracycline for pleural sclerosis had been most commonly used in patients with symptomatic malignant pleural effusion or recurrent pneumothorax for a long time. Unfortunately, at a time of expanding use of this agent, the production of injectable tetracycline hydrochloride used for pleurodesis was discontinued by its sole manufacturer in mid-1991 because the manufacturer was unable to meet US Food and Drug Administration purity standards. So we performed a preliminary study of doxycycline, as a alternative pleural sclerosant on rabbit pleura and compared its efficacy with that of tetracycline. METHOD: Eighteen New Zealand white rabbits weighing 2 to 3kg(mean 2.6kg) were divided into three groups. In each groups, one tetracycline(20 mg/ml/kg) and two doxycycline solutions(7 mg/ml/kg and 20 mg/ml/kg) instillated into the right pleural space through an 18-gauge angiocath with care to prevent pneumothorax. All rabbits were sacrificed after 28 days. RESULTS: 1) In the group of tetracycline 20 mg/ml/kg(six rabbits), five rabbits showed partial pleural symphysis with several fibrous bands, and one rabbit died on 22th day. 2) In the group of doxycycline 7 mg/ml/kg(six rabbits), three rabbits showed partial pleural symphysis and the other three rabbits showed complete pleural symphysis without necrosis of underlying parenchymal lung tissue. 3) In the group of doxycycline 20 mg/ml/kg(six rabbits), two rabbits showed complete pleural symphysis without lung necrosis, another two rabbits showed complete pleural symphysis with lung necrosis, and the other two rabbits died on 4th and 13th day, respectively. CONCLUSION: We concluded that doxycycline is a highly effective sclerosing agent having stronger pleurodesis effect with that of tetracycline by dose base and its optimal dosage was considered as 7 mg/ml/kg with minimal complications.