Interactive Cardiovascular and Thoracic Surgery 3:272-273(2004)
© 2004 European Association of Cardio-Thoracic Surgery
Case report - Thoracic general |
Spontaneous pneumothorax: are we missing something?
I.A. Kouerinis,
P.A. Hountis,
A.K. Loutsidis and
I.P. Bellenis*
Department of Thoracic and Vascular Surgery, Evangelismos Hospital, Athens, Greece
* Corresponding author. Address: King George 24, 11635 Athens, Greece. Tel.: +30-210-720-1506 (Off.)/210-721-0451 (Res.); fax: +30-210-722-4449
ionbellenis{at}hotmail.com
Received October 9, 2003;
received in revised form November 17, 2003;
accepted November 28, 2003
 |
Abstract
|
|---|
Most thoracic surgeons overlook the fact that spontaneous pneumothorax is one of the pulmonary complications of connective tissue disorders such as Marfan or Ehlers-Danlos syndromes, and they consider it as primary. In the following report we describe a unique case of spontaneous rupture of the sigmoid secondary to a spontaneous recurrent contralateral pneumothorax in a young patient with undiagnosed Marfan syndrome. The aim of this presentation is to raise a high index of suspicion of every thoracic surgeon to include in his differential diagnosis the connective tissue disorders in any case of spontaneous pneumothorax and if so, to follow further diagnostic procedures to anticipate any other visceral complications.
Key Words: Spontaneous; Pneumothorax; Visceral; Rupture; Marfan; Ehlers-Danlos
|
1. Case report
|
|---|
A 17-year-old male was admitted to our department stating that he had experienced a severe, acute thoracic pain followed by dyspnea several hours ago. He was about 185 cm tall, slim, with prominent eyes with no past history of similar pathology. Both physical and radiographic findings were compatible with pneumothorax, and thoracostomy tube drainage was performed as soon as the diagnosis was set. Six days later, since there was no sign of amelioration, we performed pleural abrasion with complete success (Fig. 1). The patient was discharged at the fourth postoperative day, but he was readmitted a few days later with the diagnosis of controlateral pneumothorax. Upon follow-up thoracostomy tube drainage, he complained of an acute abdominal pain. As there was marked rigidity, absence of bowel sounds and abdominal X-rays were suggestive of air under the left diaphragm, an urgent laparotomy was performed. At operation the peritoneal cavity contained blood mixed with feces and the sigmoid colon had a 2 cm tear on the anterior aspect of the mesenteric border. An edge biopsy was taken and the tear was repaired in two layers with absorbable suture. The postoperative period progressed uneventfully and the thoracostomy tube drainage was removed at the second postoperative day. CT chest, abdominal and brain scans with contrast were mandatory, as connective tissue disease was then included in our differential diagnosis, in order to rule out the co-existence of vascular disorders. The findings were unremarkable and at 6 months following surgery the patient was free of symptoms.
View larger version (91K):
[in this window]
[in a new window]
|
Fig. 1 The Marfan patient of our presentation. Notice the flexibility of his joints, the pectus carinatum and his features. The scars from the midline incision and thoracostomy tube drainage are also distinguishable.
|
|
|
2. Discussion
|
|---|
Connective tissue diseases are characterized by gene mutations, which affect the structure, and the synthesis of elastin and type III collagen resulting in various system disorders concerning pulmonary, cardiovascular, skeletal, and skin abnormalities (Table 1).
Among the above-mentioned respiratory abnormalities, the incidence of spontaneous pneumothorax is about 5% in such cases [1], while colon perforation is reported to be a rare complication of a non-typical undiagnosed syndrome [2,3]. In these cases despite the meticulous exploration during laparotomy, a causing factor—diverticulitis, luminal bowel obstruction, incarcerated hernia, intussusception or volvulus—cannot be identified.
We must focus our attention on the fact that skeletal manifestations that most usually set the diagnosis in Marfan patients, may be slightly distinguishable from the common tall, asthenic, healthy subjects that present spontaneous pneumothorax and as a result many of them go unrecognised. One must always have in mind that the frequency of this syndrome is reported to be 6 persons per 100,000 inhabitants [4], although in China the incidence is higher and appears to be 17 per 100,000 [5]. Undoubtedly the real prevalences exceed these numbers, as mild cases usually remain undiagnosed. Additionally, pneumothorax is also present in Marfanoid hypermobility syndrome in which patients have features of both Marfan and Ehlers-Danlos syndromes.
In conclusion we refer that when a suspicious subject is admitted with spontaneous pneumothorax further evaluation is obligatory. Past medical or strong family history in accordance with specific anthropometric measures are useful diagnostic tools. These include (a) arm span: 8 cm or more in excess of height; (b) lower truncal segment (pubis to heel, LS): 5 cm longer than upper segment (US, pubis to crown); (c) US/LS ratio <0.85; (d) arachodactyly—positive thumb (Steinberg) test, positive wrist (Walker-Murdoch) sign, metacarpial index >8,4 [6].
The diagnosis of connective tissue disorder can be established histologically, by skin fibroblast cultures, by electron microscopic examination and by electrophoresis analysis of skin biopsies that reveal lack of type III collagen and elastin.
When the diagnosis has been set further evaluation including CT scans and angiography should be pursued as rupture or dissection of the ascending aorta remain the most lethal complications. Nevertheless, the cardiothoracic surgeon must have in mind that surgical repair of ascending aorta aneurysms may prove extremely difficult due to tissue friability. Intraluninal stent grafts might prove helpful in cases of descending thoracic aortic aneurysms but more experience is needed to determine their role in such arterial complications of connective tissue disorders.
doi:10.1016/j.icvts.2003.11.015
|
References
|
|---|
- Murray RA, Poulton TB, Sartarelli MG, Dweik RA, Litwin DK, Kirby TJ, Kirby TJ, Meziane MA, O'Donovan PB. Rare pulmonary manifestation of Ehlers-Danlos syndrome. J Thorac Imaging. 1995;10:138–141[Medline]
- Olnick HM, Harris CM. Spontaneous perforation of the colon. South Med J. 1980;73(3):326–328[Medline]
- Gierson ED, Storm KF, Shaw W, Coyne SK. Caecal rupture due to colonic ileus. Br J Surg. 1975;62:383–386[Medline]
- Pyeritz RE. Editorial: Marfan syndrome. N Engl J Med. 1990;323:987–989[Medline]
- Sun QB, Zhang KZ, Cheng TO, Li SL, Lu BX, Zhang ZB, Wang W. Marfan syndrome in China: a collective review of 564 cases among 98 families. Am Heart J. 1990;120:934–948[Medline]
- Streeten EA, Murphy EA, Pyeritz RE. Pulmonary function in the Marfan syndrome. Chest. 1987;91(3):408–412[Abstract/Free Full Text]
|
|
Top
Abstract
1. Case report