Teaching Case

was admitted to the hospital because of severe acute pain in the lower abdomen. On physical examination, she had a diffusely tender abdomen without rebound tenderness. Her medical history revealed pulmonary lymphangioleiomyomatosis which had been known for 4 years and diagnosed on open lung biopsy. Blood tests were normal on admission. Conventional radiographs and CT scan of the lungs were performed, as well as lymphography, CT scan and MRI of the abdomen.

A 54-year-old woman was admitted to the hospital because of severe acute pain in the lower abdomen. On physical examination, she had a diffusely tender abdomen without rebound tenderness. Her medical history revealed pulmonary lymphangioleiomyomatosis which had been known for 4 years and diagnosed on open lung biopsy. Blood tests were normal on admission. Conventional radiographs and CT scan of the lungs were performed, as well as lymphography, CT scan and MRI of the abdomen. Based on medical history, clinical data and imaging findings, the diagnosis of retroperitoneal localization of lymphangioleiomyomatosis (LALM) was suggested.

Lymphangioleiomyomatosis (LALM) is a rare disease, almost exclusively observed in women of childbearing age. Pulmonary findings, such as interstitial lung disease, chylothorax, pneumothorax, haemoptysis and symptoms of obstructive lung disease predominate, although mediastinal and retroperitoneal localizations of LALM are well recognized parts of the disease. Acute abdominal pain may be a manifestation of the disease. This pain probably results from distortion of normal lymph routes, secondary to proliferation of smooth muscle within the lymphatic channels, thereby causing development of collaterals and large lymph cysts which overdistention may cause rupture and chyloperitoneum. In the lungs, the smooth muscle proliferation is seen along the airways, blood vessels and lymphatic channels. Air trapping, increased intra-alveolar pressure and the resulting alveolar disruption cause thin-walled cystic deformation of the lung parenchyma. Tuberous sclerosis and LALM have been linked with each other. However family history and other manifestations of tuberous sclerosis are often absent in LALM. Diagnosis of LALM is made on open lung biopsy, as there is still debate whether the diagnosis may be ruled out on a normal CT scan. Most patients die from respiratory insufficiency within an average of 8.5 years after the onset of the disease. Medical treatment with medroxyprogesterone or ovariectomy seems ineffective for relief of symptoms. Lung transplantation can prolong survival. In the presented patient, the retroperitoneal LALM was not considered to be a contraindication for lung transplantation, for it was thought to have only minor prognostic significance.