Ann Thorac Surg 2010;89:599-601. doi:10.1016/j.athoracsur.2009.06.091
© 2010 The Society of Thoracic Surgeons
Case Reports
Restrictive Chest Wall Deformity as a Complication of Surgical Repair for Pectus Excavatum
Chih-Hao Chen, MDa,b,*,
Hung-Chang Liu, MDa,b,
Tzu-Ti Hung, BNa,b,
Chao-Hung Chen, MDa
a Department of Thoracic Surgery and Pathology, Mackay Memorial Hospital, Taipei City, Taiwan
b Mackay Medicine, Nursing and Management College, Taipei City, Taiwan
Accepted for publication June 16, 2009.
* Address correspondence to Dr Chen, Department of Thoracic Surgery and Pathology, Mackay Memorial Hospital, No. 92, Section 2, Chung Shan N Rd, Taipei City, Taiwan 10449 (Email: musclenet2003{at}yahoo.com.tw).
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Abstract
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The Ravitch operation is frequently performed to correct pectus excavatum. However, extensive destruction of the perichondrium and rib growth centers may lead to failure of subsequent chest wall development. A 29-year-old man who underwent a Ravitch operation 26 years previously developed a restrictive chest wall deformity, which resulted in severe pulmonary hypertension and restrictive lung disease.
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Introduction
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Restrictive chest wall deformity may result from extensive destruction of the perichondrium and rib growth centers during pectus repair. Failure of chest wall growth can lead to pulmonary hypoplasia, pulmonary hypertension and eventually cardiac failure.
A 29-year-old man was evaluated. When he was 3 years old, he underwent a conventional Ravitch operation. According to the operation records, the patient had a moderate pectus excavatum. The surgeon resected all grossly deformed cartilage, preserving the perichondral sheath. Struts were not used. The initial functional and cosmetic results were excellent. He recovered well during the postoperative period. The only persistent problems were mild dyspnea and mild exercise intolerance in childhood. In his adolescent period, the patient noticed that his chest wall did not grow proportionately with the rest of his body. At times he experienced dyspnea, especially during vigorous exercise. Rest was able to alleviate the symptoms most of the time. However, in recent years, the patient complained of marked dyspnea even after walking for a short distance. With time the symptoms worsened, which led the patient to seek medical attention. On presentation, the patient's vital signs were stable. Physical examination found a small and restricted thoracic development, especially in the upper half of the thorax (Fig 1). A chest roentgenogram revealed a long and narrow thoracic cage, especially in the upper half of the chest, and severe enlargement of the heart (Fig 2). A subsequent computed tomographic scan of the chest confirmed small lung volumes and prominent pulmonary vessels at the level of aortic arch (Fig 3A). At the level of the heart, a computed tomographic scan revealed absence of costal cartilage and aberrant growth of the anterior chest wall, with concomitant enlargement of the cardiac chambers, as well as some pericardial effusion (Fig 3B). Pulmonary hypertension, estimated to be 75 mm Hg, impaired cardiac diastolic compliance, and severe tricuspid regurgitation was found on cardiac ultrasound. Lung function test showed decreased forced vital capacity (0.71 L), forced expiratory volume in 1 second (0.44 L), and decreased total lung capacity (1.88 L). Due to progressive dyspnea, the patient was evaluated for surgical intervention. The planned surgical procedure was a lateral thoracic expansion by means of both titanium struts and bone grafts. However, due to the higher risk of mortality and morbidity, the patient declined the operation and was discharged against medical advice. He is regularly followed in the outpatient department with medical control of pulmonary hypertension. Intermittent oxygen therapy is also required in his daily life.
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Fig 1. The gross appearance of the thorax showing the inappropriate chest wall growth in the upper half compared with the rest of the body.
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Fig 2. A long and narrow thoracic cage is an apparent radiographic feature of restrictive chest wall deformity. The size of the heart is exceedingly large and occupies the already narrowed space of the chest.
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Fig 3. A computed tomographic scan of the chest. (A) At the aortic arch level, the pulmonary vessels are highly engorged (even larger than the aorta) due to severe pulmonary hypertension. (B) At the heart level, aberrant anterior chest wall growth, cardiomegaly, and pericardial effusion are evident.
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Comment
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The Ravitch operation was previously the procedure of choice for correcting pectus excavatum, and more recently the Nuss procedure has become more prevalent [1]. The Ravitch operation, however, is still frequently performed by thoracic surgeons worldwide. Knowledge of the potential long-term complications is mandatory for every surgeon performing this procedure.
For severe pectus excavatum deformity, the Ravitch operation can be performed at any age. For less severe examples, some surgeons advocate surgical correction before a child attends school to avoid adverse social consequences because of their appearance, whereas others delay the procedure until later [2].
Although the operating surgeon described preservation of the perichondrium, the growth centers of the ribs were likely being damaged during the procedure. This may explain the reason that the patient's chest wall development was so restrictive. If the growth centers are damaged at a young age, impaired chest wall deformity may ensue; the severe consequences of which are amply demonstrated and described in this patient.
With respect to treatment, lateral thoracic expansion using titanium struts and rib splitting, as described for the congenital Jeune syndrome, was suggested to the patient [3–5]. Unfortunately, surgery was declined, and demise in this patient may be hastened.
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References
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- Nuss D, Kelly Jr RE, Croitoru DP, et al. A 10-year review of a minimally invasive technique for the correction of pectus excavatum J Pediatr Surg 1998;33:545-552.[Medline]
- Haller Jr JA, Colombani PM, Humphries CT, et al. Chest wall constriction after too extensive and too early operations for pectus excavatum Ann Thorac Surg 1996;61:1618-1625.[Abstract/Free Full Text]
- Kaddoura IL, Obeid MY, Mroueh DM, et al. Dynamic thoracoplasty for asphyxiating thoracic dystrophy Ann Thorac Surg 2001;72:1755-1758.[Abstract/Free Full Text]
- Davis JT, Long FR, Adler BH, et al. Lateral thoracic expansion for Jeune syndrome: evidence of rib healing and new bone formation Ann Thorac Surg 2004;77:445-448.[Abstract/Free Full Text]
- Weber TR. Further experience with the operative management of asphyxiating thoracic dystrophy after pectus repair J Pediatr Surg 2005;40:170-173.[Medline]
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Abstract
Introduction
