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KMID : 0371319730150040063
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Journal of the Korean Surgical Society
1973 Volume.15 No. 4 p.63 ~ p.69
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The Marfan Syndratne Associated with Suhpleural Blebs
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金炯默/Kim, H.M.
金仁洙/李勝鎭/朴光勳/徐舜圭/崔信義/Kim, I.S>/Lee, S.J./Park, K.H./Suh, S.K./Choi, S.E.
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Abstract
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A case of the Marfan Syndrome associated with tension pneumothorax, left, due to rupture of the subpleural blebs is presented as the first case in Korea.
The case was 15 year old middle school girl with severe respiratory distress, and her family pedigree demonstrated heritable nature and its transmission as an autcscmal dcmirart hereditary trait.
The girl showed typical arachncdactyly with abrcrrral skeletal ratio, high arched palate, pigeon. chest and mild kyphescolicsis, and moderate dilataticn cf the thoracic aorta, tut there was no distinct optical involvement. The, extremities were excessively thin and Icrg, and the measurement of the low segment was in excess of these of the upper segment, and the arm span in excess of the highest. Also there was loose jointness of the small joints of the hand and feet.
Thoracotomy, left, was performed to remove the ruptured subpleural blebs at the upper margin cf
the apico-posterior segment of the left upper lobe of the lung as a wedge resection including regicral lymph node at the pulmonary hilum, and the patient recovered without any complications, and discharged from hospital on 9th postoperative day.
Pathological section slide of the lung tissue revealed cystic dilatation of the subpleural blebs lined by thin fibrous membrane with chronic nonspecific inflammatory cell infiltrations through the wall composed of loose fibrous tissue, and lymphnede sections revealed nothing, specific abnormal findings.
Follow-up check of the patient for 6 months revealed some increase of the intensity of the systolic blowing murmur at the aortic area with increased palpitaticn, but there was no more recurrence of the pneumothorax.
Regarding the optic involvement, the case revealed no remarkable optic involvement such as dislocation of the lens or retinal detachment, but the diagnosis of the Marfan Syndrome were considered to be confirmative under the clinical observation of the characteristic family heredity, typical skeletal involvement (arachnodactyly, excessive long extremities, high arched palate, pigeon chest and loose jointness), cardiovascular involvement and rare but distinct association with recurrent spontaneous tension pneumothorax due to rupture of the subpleural blebs.
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