[Pulmonary lymphangioleiomyomatosis]. / Pulmonal lymfangioleiomyomatose.

Pulmonary lymphangioleiomyomatosis is a rare disease with a haphazard proliferation of smooth muscle throughout the lung. Little knowledge about the disease, its clinical presentation and the diagnostic methods used may be a reason for underdiagnosing the disease. In Ostfold county, Norway, with only 250,000 inhabitants, we have seen four patients with this disease in the last few years. The diagnosis was based on histological examination of transbronchial biopsy material in three patients who underwent bronchoscopy the presence of lymphangioleiomyomatosis was not recognized at the initial pathological examination. The disease can be misinterpreted as fibrosis at histological examination. Specific procedures for detecting smooth muscle can be used, eradicating the need for open lung biopsy. Transbronchial biopsy is a valid and useful method for confirming the diagnosis of lymphangioleiomyomatosis. High resolution CT has also shown to be an important diagnostic tool. We emphasize the importance of raising the question of lymphangioleiomyomatosis with the pathologist when this rare, but probably underdiagnosed disease, is suspected by the clinician.

Acute abdominal pain as presenting symptom in lymphangiomyomatosis. Case report.

A case of lymphangiomyomatosis presenting with acute abdominal pain is described. Laparotomy revealed a multiloculated cystic mass on the posterior abdominal wall with typical histological pattern. In the ensuing 3 years the respiratory manifestations of this rare disorder have slowly progressed. Possible causal factors and management are discussed.