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Pulmonary Abnormalities in Marfan's Syndrome
Related to Diving

Compiled by Ernest S Campbell, MD

What is Marfan's Syndrome?

It seems inevitable that someone will want to scuba dive with every disease known to man and wonder if there is some reason not to. Marfan's Syndrome is one of these conditions. It has clinical features that are uniformly fatal unless corrected surgically and include aortic aneurysmal formation and dissection due to an abnormal collagen (protein). The adventitial structure of aortas of MS patients has been noted to be composed of a certain amount of thin type III collagen fibers, and similar protein has been found in excised cysts on lungs of patients with recurrent spontaneous pneumothoraces.

How does this condition relate to diving?

Pulmonary abnormalities occur in approximately 10% of patients, the commonest being spontaneous pneumothorax and emphysema. The same abnormal protein causes cystic pulmonary abnormalities that make it extremely dangerous for a person with Marfan's syndrome to dive.  Significant pulmonary problems include restriction of lung function due to pectus excavatum (caved in chest),  or kyphoscoliosis (hunchback) and intrinsic pulmonary involvement with emphysema, bronchogenic cysts and "honeycomb lung." This leads to a significant incidence of spontaneous pneumothorax and the danger of tension pneumothorax when associated with positive pressure ventilation.

There are now many reports of cases of spontaneous pneumothorax in Marfan's syndrome patients, some occurring even with the minimal pressure changes associated with aircraft pressurization and mountain climbing. Scuba diving with compressed air would be quite hazardous considering the relatively huge pressure changes that take place during the first and last 8 feet of a dive.
 

Findings on chest x-ray

Chest radiographs typically show bilateral bullae (blisters) in the upper lung zones and pulmonary function tests are consistent with mild emphysema. There were decreases in forced expiratory flow rates at low lung volumes, carbon monoxide transfer factor, and lung elastic recoil. It is felt that pneumothorax and bullous emphysema in this syndrome are caused by a weakness in the pulmonary connective tissue framework.

These pulmonary changes require excision of the bullae and blebs and obliteration procedures (abrasion) to prevent future lung collapse and fluid formation.

Possible nasal and Eustachian tube problems

Also possibly inimical to the diver is the congenital nasopharyngeal constriction of the maxillae with nasal airway restriction (NAR). NAR is a common feature of Marfan's syndrome causing problems with sleep apnea. Eustachian tube function would be certainly endangered but has not been reported. Middle ear barotrauma, sinus squeeze and tympanic membrane rupture would be certain consequences.


References: (From Medline)
 


Cystic lung in Marfan's syndrome.

Sharma BK, Talukdar B, Kapoor R.

Thorax. 1989 Nov;44(11):978-9.

PMID:
2595640
[PubMed - indexed for MEDLINE]
Free PMC Article
2.

Marfan's syndrome presenting as bilateral spontaneous pneumothorax.

Gawkrodger DJ.

Postgrad Med J. 1981 Apr;57(666):240-1.

PMID:
7291104
[PubMed - indexed for MEDLINE]
Free PMC Article
3.

[Surgically treated case of spontaneous pneumothorax with Marfan's syndrome].

Takahashi K, Hanai K, Sasaki S, Yamaguchi T.

Kyobu Geka. 1970 Oct;23(10):760-3. Japanese. No abstract available.

PMID:
5466564
[PubMed - indexed for MEDLINE]
4.

Bronchiectasis and spontaneous pneumothorax in Marfan's syndrome.

Teoh PC.

Chest. 1977 Nov;72(5):672-3.

PMID:
913156
[PubMed - indexed for MEDLINE]
Free Article
5.

Persistent spontaneous pneumothorax in an adolescent with Marfan's syndrome and pulmonary bullous dysplasia.

Rigante D, Segni G, Bush A.

Respiration. 2001;68(6):621-4.

PMID:
11786720
[PubMed - indexed for MEDLINE]
Free Article
6.

Myelopathy in Marfan's syndrome.

Newman PK, Tilley PJ.

J Neurol Neurosurg Psychiatry. 1979 Feb;42(2):176-8. No abstract available.

PMID:
422966
[PubMed - indexed for MEDLINE]
Free PMC Article
7.

[Condition of the lungs in Marfan's syndrome].

Lisichenko OV, Poliakova-Selivanova ND.

Sov Med. 1973 Dec;36(12):127. Russian. No abstract available.

PMID:
4795218
[PubMed - indexed for MEDLINE]
8.

[A case report of spontaneous pneumothorax in Marfan's syndrome and an immunohistochemical study of type I collagen in lung tissue].

Takanami I, Morota A, Shikata J.

Nihon Kyobu Shikkan Gakkai Zasshi. 1991 Sep;29(9):1186-9. Japanese.

PMID:
1753546
[PubMed - indexed for MEDLINE]
9.

Marfan's syndrome and spontaneous pneumothorax.

Sensenig DM, LaMarche P.

Am J Surg. 1980 Apr;139(4):602-4. No abstract available.

PMID:
7369469
[PubMed - indexed for MEDLINE]



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