[Case of lymphangioleiomyomatosis (LAM) discovered during cesarean section under spinal anesthesia].

We experienced a case of scheduled cesarean section under spinal anesthesia in a patient with LAM which had been missed in spite of preoperative medical examination and consultation with specialists but discovered because of perioperative hypoxia A 35-year-old woman, Gravida 1 Para 0, with breech presentation was scheduled to undergo cesarean section under spinal anesthesia at 38 weeks of gestation. She had no history of asthma or abnormal findings at annual medical examination. She had suffered from dry cough and nocturnal dyspnea for 7 weeks and an inhaled bronchodilator was administered with diagnosis of inflammatory airway disease by her respiratory physicians. Spinal anesthesia was performed with bupivacaine 12.5 mg. At the beginning of anesthesia SPO2 was 97% in supine position, but it rapidly decreased to less than 90% and 3 l x min(-1) oxygen was supplied with a facial mask. The anesthetic level was thoracal 4 bilaterally and her breathing was stable. The circulatory state, Apgar score and other vital signs were within normal ranges. Postoperative chest X-ray showed bilateral numerous grained spots and computed tomography scans showed multiple thin-walled cysts. The characteristic history and the fluoroscopic data gave her clinical diagnosis of LAM.

Resection of Extrapulmonary Lymphangiomyoma and Post-Operative Management Considerations.

Background: Lymphangiomyomatosis is a rare disease involving the lymph vessels, causing obstruction and cystic formation with an incidence of 3-8 per million women. The disease might be sporadic or inherited. Lymphangiomyomatosis mostly affects the pulmonary system, whereas extrapulmonary Lymphangiomyomatosis may present in various site, occasionally as a localized abdominal mass. The diagnostic process might entail surgical resection to obtain a specimen for pathology that may also help to achieve a long-term control of the disease. Methods: Herein, we present a case of a 45 years old female, who suffered from pulmonary symptoms, and during her workup an abdominal mass was found. The patient underwent exploratory laparotomy with resection of a left retroperitoneal bilobar mass. Results: Histopathological report revealed Lymphangiomyoma. She had a complication of a lymphatic leakage that required a second laparotomy with satisfactory clinical outcome. Conclusions: Surgeons should be aware of the pathological lymphatics and manage post-operative complications by a trial of conservative.

Chylopericardium Due to Residual Lymphangiomyoma Detected on 99mTc-Sulfur Colloid Lymphoscintigraphy.

ABSTRACT: Lymphangiomyomas are relatively rare, benign neoplasms. Many patients present with symptoms including effusions, and some cases are incidentally detected. Surgical excision is the treatment of choice, but because of its location, complete surgical resection of a lymphangioma can be technically difficult, and recurrent cases can present with symptoms including effusions. 99mTc-sulfur colloid scan can be used to confirm the leak and nature of the effusion fluid. Here, we present an 8-year-old girl with recurrent pleural and pericardial effusions after lymphocele excision and total pericardiectomy. 99mTc-sulfur colloid lymphoscintigraphy was done to rule out secondary chylopericardium.

[Acute abdominal pain and fever in a young woman as the first symptoms of lymphangioleiomyomatosis]. / Acute buikpijn en koorts bij een jonge vrouw als eerste verschijnselen van lymfangioleiomyomatose.

A previously healthy 28-year-old woman presented to the emergency clinic with acute severe abdominal pain and high fever. A diagnostic laparoscopy was performed, during which a large retroperitoneal tumour was found. A CT-scan of the abdomen and thorax confirmed the presence of a retroperitoneal mass but also revealed multiple renal angiomyolipomas and extensive cystic lesions in all lung fields. Based on these findings, the diagnosis lymphangioleiomyomatosis (LAM) was suspected, and later confirmed by histological examination of a biopsy specimen. The acute abdomen and fever appeared to have been caused by a Streptococcus agalactiae infection of the retroperitoneal lymphangiomyoma, which was treated with intravenous antibiotics. LAM is a very rare disease affecting mostly women of childbearing age and presenting almost exclusively with pulmonary symptoms. This is the first description of LAM presenting with an acute surgical abdomen and fever due to infection of a lymphangiomyoma.

Effect of fasting on the size of lymphangioleiomyomas in patients with lymphangioleiomyomatosis.

BACKGROUND: Lymphangioleiomyomas occur in 38% of patients with sporadic lymphangioleiomyomatosis (LAM) and may cause pain and increased abdominal girth, mimicking the presence of a malignancy. Lymphatic involvement in LAM is closely associated with elevated serum levels of vascular endothelium growth factor-D (VEGF-D). Because lymphangioleiomyomas undergo diurnal variation in volume, we hypothesized that daytime ingestion of food, by increasing chyle formation and lymphatic flow, is the cause of an increase in lymphangioleiomyoma volume. METHODS: Subjects had abdominopelvic sonograms and blood drawn for measurement of serum VEGF-D levels under nonfasting (day 1) and fasting (day 2) conditions. The size of the lymphangioleiomyomas was determined by a radiologist who was blinded to the subjects' status. The Wilcoxon signed rank test was used to determine whether the nonfasting tumor size was different from the fasting tumor size. RESULTS: Thirty-five women were studied (aged 45.2 ± 8.5 years; FEV1, 82% ± 25%; diffusing capacity of the lung for carbon monoxide, 64% ± 25% predicted). Images suitable for volume measurements were obtained in 30 subjects. Fasting decreased the tumor size by 20.7 ± 39.3 cm3 (24% ± 40%, P < .001). Fasting VEGF-D levels (10,650 ± 900 pg/mL) were not significantly different from nonfasting values (12,100 ± 800 pg/mL, P = .56). CONCLUSIONS: Lymphangioleiomyoma volume decreased during the fasting state. Conversely, a combination of food intake and decreased chyle flow through lymphatics partially obstructed by LAM cells may account for increases in lymphangioleiomyoma size. Imaging studies performed under fasting conditions may help in determining whether an abdominal tumor is a result of LAM or malignancy.

Causes of death in patients with tuberous sclerosis.

Of the 355 patients with tuberous sclerosis complex (TSC) examined at the Mayo Clinic, 49 had died (9 of causes other than TSC). We attempted to determine what pattern of organ involvement occurred most often in the 40 patients who died of TSC. One baby died of cardiac failure due to cardiac rhabdomyomas, and one child died of rupture of an aneurysm of the thoracic aorta. Eleven patients died of renal disease, which was the commonest cause of death. Ten patients died as a result of brain tumors, and four patients (who were 40 years of age or older) died of lymphangiomyomatosis of the lung. Thirteen patients with severe mental handicaps died of either status epilepticus or bronchopneumonia; in all but one of these patients, the only source of information was the death certificate. Survival curves show a decreased survival for patients with TSC in comparison with that for the general population. Patients with TSC need lifelong follow-up for early detection of potentially life-threatening complications.

Exacerbation of pulmonary lymphangioleiomyomatosis by exogenous estrogens.

A 48-year-old woman with profound, rapidly progressive dyspnea requested a second opinion regarding the diagnosis and management of an undiagnosed interstitial process. One year prior to this evaluation, she had been placed on therapy with exogenous estrogens for the treatment of osteoporosis. During this therapy, she had a marked deterioration of her pulmonary status. Review of her open lung biopsy, which was obtained five years previously, revealed lymphangioleiomyomatosis. Discontinuation of estrogen therapy and treatment with tamoxifen were successful in stopping the progressive course. This patient's clinical course suggested an association between estrogen therapy and clinical deterioration during the menopause.

Failure of buserelin-induced medical castration to control pulmonary lymphangiomyomatosis in two patients.

Two women, aged 44 and 29 years, respectively, were admitted to the hospital in early 1987 for recurrent pneumothorax, dyspnea and a diffuse reticulonodular pattern evidenced on the chest x-ray film. Lung biopsy confirmed LAM in both patients. Both were treated sequentially with medroxyprogesterone and a LHRH agonist (buserelin) to achieve reversible medical castration. Neither subjective nor objective improvement was noted after 13 and 5 months, respectively, of buserelin therapy (900 micrograms/day, nasal spray) despite an effective suppression of the pituitary-gonadal axis. Medroxyprogesterone also was ineffective. Buserelin thus failed to control pulmonary LAM in these two patients, in spite of effective medical castration.

Pulmonary lymphangioleiomyomatosis associated with pulmonary parenchymal, hilar, and mediastinal noncaseating granulomas.

A young nonsmoking woman presented with severe dyspnea, exercise desaturation, and chest discomfort. Pathologic and histochemical findings revealed pulmonary lymphangioleiomyomatosis (LAM) as the primary abnormality. In addition, there were multiple noncaseating granulomas with special stains and cultures negative for organisms. This highly unusual combination of pathologic findings might suggest the presence of coexistent sarcoidosis in our patient with LAM.

Lung transplantation for respiratory failure resulting from systemic disease.

BACKGROUND: Lung transplantation for pulmonary failure resulting from systemic disease is controversial. We reviewed our transplant experience in patients with sarcoidosis, scleroderma, lymphangioleiomyomatosis, and graft-versus-host disease. METHODS: This retrospective review examined the outcome of 23 patients who underwent pulmonary transplantation for these systemic diseases. Group 1 included 15 patients with pulmonary hypertension who underwent transplantation (9 for sarcoidosis, 6 for scleroderma), and group 2 included 8 patients with normal pulmonary artery pressures who underwent transplantation (5 for lymphangioleiomyomatosis, 3 for graft-versus-host disease). The incidences of infection and rejection, pulmonary function, and survival were measured and compared with those of patients who underwent transplantation for isolated pulmonary disease. RESULTS: Although there were no differences in the rate of infection between patients who underwent transplantation for systemic versus isolated disease, patients with pulmonary hypertension who underwent transplantation for systemic disease had significantly lower rates of rejection. Four patients with sarcoidosis and 2 with lymphangioleiomyomatosis demonstrated recurrence in the allograft. Survival was similar between patients who underwent transplantation for systemic versus isolated disease. CONCLUSIONS: Patients with respiratory failure resulting from these systemic diseases can undergo transplantation with outcomes comparable to those obtained in patients who undergo transplantation for isolated pulmonary disease.

Pulmonary lymphangiomyomatosis causing bilateral pneumothorax during pregnancy.

A 32-year-old woman, 12 weeks pregnant, presented with bilateral spontaneous pneumothorax that did not heal with tube thoracostomy. At right and left thoracotomy, lymphangiomyomatosis of the lung was found and treated by pleurodesis. Lymphangiomyomatosis has been infrequently reported in the surgical literature, even though it may befall the thoracic surgeon to establish the diagnosis and aid in treatment.

Lymphoedema of the legs as a result of lymphangiomyomatosis. A case report and review of the literature.

Leg lymphoedema is described as an unusual presenting feature of lymphangiomyomatosis. MRI scanning revealed typical lesions. Possible sex hormonal manipulation as a therapy for pulmonary or abdominal involvement is reviewed.

Two cases of hamartoangiomyomatosis with characteristic scintigraphic findings.

Hamartoangiomyomatosis is a rather rare pulmonary disease which occurs in young to middle-aged women. The chief complaint of this disease is dyspnea. The chest X-ray findings of this disease are such complicated features as reticular, reticulogranular, miliary and honeycomb-like shadow. A pulmonary perfusion scintigram was prepared with a scinticamera after intravenous injection of 10 mCi of Tc-99m MAA. The anterior image showed a remarkably reduced bilateral pulmonary blood flow, especially in the middle and lower areas of the lungs. In the upper portions, the blood flow remained relatively sufficient. The posterior and lateral images also gave similar findings. In two cases of hamartoangiomyomatosis, pulmonary perfusion scintigraphy was successful in identifying the characteristic findings of the disease.

Pulmonary lymphangiomyomatosis complicating pregnancy. A case report.

A 32-year-old primigravida presented with cough and dyspnea at 16 weeks' gestation. Chest roentgenogram revealed a large pleural effusion and diffuse interstitial infiltrates. Moderate arterial hypoxemia and a significant reduction in vital capacity were present. Thoracentesis revealed sterile chyle with no evidence of malignancy. Spontaneous delivery of a healthy infant occurred at 38 weeks, but no change was seen in either the pulmonary infiltrates or chylothorax. Open lung biopsy confirmed the clinical impression of pulmonary lymphangiomyomatosis, and a pleurodesis was performed. Progesterone and estrogen receptor assays on the lung biopsy material revealed only minimal binding. Following two years of therapy with tamoxifen citrate and megestrol acetate, the chylothorax has not recurred, and there has been no other appreciable change in pulmonary function.

Retroperitioneal lymphangiomyomatosis with lymphedema of the legs.

Retropertioneal tumor is demonstrated by lymphangiography in a 28 year old woman with lymphedema of both legs. Retroperitioneal tumor was subtotally extirpated, with subsequent subsidence of the swelling of both legs. According to the pathologic examination of the extirpated specimens, lymphangiomyomatosis was confirmed. This is the fisrt case reported in the literature of lymphangiomatosis presenting with lymphedema of the leg in the absence of pulmonary involvement, pleural or abdominal effusion.

Early chylopneumothorax in a patient with pulmonary lymphangioleiomyomatosis.

A 50-year-old woman was admitted with the complaint of cough and dyspnea on exertion for the previous two months. A radiograph of the chest showed a right-sided hydropneumothorax, which was proven to be a chylous effusion by lipoprotein electrophoresis and was very refractory to tetracycline-pleurodesis. The chylopneumothorax was cured by ligation of the thoracic duct and surgical pleurodesis. After an open lung biopsy, lymphangioleiomyomatosis (LAM) was diagnosed histopathologically with smooth muscle nodules scattered throughout the lungs, obstructing the small airways, venules and lymphatics. An immunohistochemistry study using the avidin biotin complex method with monoclonal antibodies for actin and desmin showed the small nodules to be of muscle origin. During the past two years, the patient has remained stable both in respiratory status and roentgenographically without hormonal manipulation or oophorectomy. We present this case to illustrate the heterogeneous nature of this condition. While our patient's initial presentation was acute and associated with chylothorax, her postoperative course has shown no progression despite withholding of hormonal therapy.

Intraabdominal lymphangiomyoma in an infant with protein-losing enteropathy and hemihypertrophy.

Lymphangiomyoma is an extremely rare tumor occurring exclusively in women of reproductive age. The tumor is characterized by proliferation of immature smooth muscle along the lymphatic vessels of the abdomen, thorax and lung. Although lymphangiomyoma has been reported in a young girl and a girl infant, none has been reported in boys. We report herein a case of lymphangiomyoma in a two-year-old boy. The unusual presentation in this patient was that the tumor arose from the small bowel mesentery without any evidence of lung involvement. The tumor was extirpated and lymphangiomyomatosis was confirmed pathologically.

[Differential diagnosis of spontaneous pneumothorax--pulmonary lymphangioleiomyomatosis. Clinical aspects, diagnosis and therapy]. / Zur Differentialdiagnose des Spontanpneumothorax--die pulmonale Lymphangioleiomyomatose. Klinik, Diagnostik und Therapie.

As a case report, lymphangioleiomyomatosis is presented--a rare disease that only affects women and up to now with a poor prognosis. It results from a tumorlike proliferation of smooth muscle in the thoracic and retroperitoneal lymphatics. Prominent clinical findings are dyspnea, spontaneous pneumothorax, chylous pleural effusions and chylous ascites. The diagnosis results from typical chest X-rays and is established by open lung biopsy. Treatment should primarily start with medroxyprogesteronacetat (respectively tamoxifen), if necessary with consideration of the hormonal receptors. In an early stage the oophorectomy can be helpful. Surgical intervention is necessary in complications (thoracic drainage, pleurectomy for recurrent pneumothorax, Le Veen-shunt for chylous ascites). If hormonal therapy is not successful, a combined heart and lung transplantation should be attempted in ultima ratio.