Nehra, Deepika MD; Ein, Sigmund H. MD; Tlumacki, Mark CO; Masiakos, Peter T. MD
DEEPIKA NEHRA, MD, SIGMUND H. EIN, MD, MARK TLUMACKI, CO, and PETER T. MASIAKOS, MD, are affiliated with the Department of Pediatric Surgery, Massachusetts General Hospital for Children, Boston, Massachusetts.
Correspondence to: Deepika Nehra, MD, 55 Fruit St, Boston, MA 02114; e-mail: firstname.lastname@example.org
Pectus carinatum is a chest wall deformity characterized by a convex protrusion of the sternum and adjacent cartilages. Orthotic bracing has been used for correction of this deformity. In this report we provide convincing imaging that demonstrates the potential efficacy of this treatment modality and also describe in detail the process of constructing an ideal custom orthosis for the treatment of pectus carinatum deformities.
Despite the fact that there are a few published reports that describe the initial experience with orthotic bracing for pectus carinatum (PC) deformities, to date there is very little convincing imaging data to support the reported results and there is also very little information on the construction of these orthotic braces, when they should be implemented and the length of time that they should be worn. For this reason, we present here the cases of two teenaged boys treated with orthotic bracing for a PC deformity (one homemade and the other made in the orthotics facility at the Massachusetts General Hospital). This report concentrates specifically on providing convincing imaging that demonstrates the potential efficacy of this treatment and on describing the process of constructing an ideal custom orthotic brace.
J.T., a 14½-year-old white male, was first seen by one of the authors (S.H.E.) in his General Surgery Clinic at the Hospital for Sick Children, Toronto, Canada in March 2000. He and his parents presented with a Polaroid picture of a moderately severe (cosmetically), asymptomatic PC deformity from 3 months before (Figs. 1A, B). The patient was offered corrective surgery, however on hearing the operative approach described, the patient’s father constructed a very ingenious compression brace (Fig. 2), which the patient wore at all times (except for in the shower) and successfully corrected the PC deformity within 3 months. When he was first seen in Clinic (after wearing the compression brace for 3 months), his PC defect was virtually gone, except for some mild bilateral lower costal cartilage flaring, greater on the left (Figs. 1C, D). He continued to use the compression brace for 4 more months only at night. After using the compression for a total of 7 months, he decreased his nighttime use in a tapering fashion. He finally discontinued using the compression brace after 2 years, at which time the mild left lower costal cartilage flaring had also decreased. He remains well with no recurrence for 7 years. His smaller fraternal twin brother, age 15½ years, was seen in April 2001 with a mild to moderate (cosmetically) PC, which was also asymptomatic. He was treated with a similar compression brace (also made by his father) for 2 months all day and night, and then a tapering nighttime schedule for 3 more months, at which time he discontinued its use. When seen after 6 months, his PC also returned to normal. His follow-up of 6 years is also recurrence free.
A.B., a 15-year-old white male, was first seen for evaluation of his PC deformity by one of the authors (P.M.) in his General Surgery Clinic at the Massachusetts General Hospital for Children, Boston, MA in December 2006. His PC had been evident since birth; however, it became more exaggerated during his teenage years. He was generally in good health but did report dyspnea on exertion that he had noticed in the months before presentation. He was an active lacrosse player and reported feeling increasingly winded and fatigued recently. On physical examination, his chest appeared significantly disfigured with an obvious PC deformity (Fig. 3A). The patient and his parents were presented with both surgical management and orthotic bracing as treatment options and were encouraged to try a trial of orthotic bracing. The patient was fitted for a custom compression brace (Fig. 4) that he wore both during the day and at night, and it was removed only for 3 hrs a day during his lacrosse practice. The brace was adjusted at each month and more cushioning was added to help prevent irritation of the skin as the protrusion flattened. After 2 weeks of wearing the brace, the patient subjectively reported that his sternum seemed a little flatter. After wearing the brace for 9 weeks, his sternum was significantly flatter and his pectus deformity was almost indiscernible (Fig. 3B). This patient clearly experienced superlative cosmetic results from orthotic bracing for his PC deformity. He will continue to wear the brace all day for a few months and then switch to nocturnal bracing only until the age of 18 or when vertical growth slows to give him the best chance of stable results. His follow-up to this point in time is 19 months, and he remains recurrence free.
PC (also called pigeon breast) describes a range of chest wall deformities characterized by a convex protrusion of the sternum and the adjacent costal cartilages. The condition is thought to result from the abnormal growth of the costal cartilages resulting in an anterior chest wall protrusion of varying severity. The exact cause of the exaggerated growth is not yet understood. The condition is seen more commonly in families with other thoracic deformities and has also been associated with Marfan syndrome, congenital heart disease, and hand agenesis indicating that there may be a hereditary or congenital origin.
The overall incidence of PC is 0.6%, and the deformity affects males more than females (4:1).1 The deformity often progresses in severity during the accelerated growth seen with puberty. As such, the condition is not typically appreciated until after the first decade of life and most patients present for correction as teenagers.2 Although PC may be associated with significant cardiopulmonary compromise with symptoms such as palpitations, dyspnea, and wheezing because of chest wall rigidity, for most patients it is the significant cosmetic and psychosocial impact that prompts them to seek surgical reconstruction.3
Surgery is the traditional treatment for PC. Although surgical management usually produces good results, these procedures are often very debilitating. For this reason, there has recently been an increasing impetus to explore nonoperative management strategies for PC by taking advantage of the fact that the anterior chest wall is still compliant during puberty. Various different strategies for remodeling the chest wall have been described, but it is clear that some of the most convincing outcomes have been seen with orthotic bracing.
The first case report describing the use of orthotic bracing for the PC deformity was published in 1993. In this report, Mielke and Winter4 describe an excellent result in an 8-year-old girl treated with bracing for about 16 months for a cosmetically objectionable PC deformity. This first report was followed by other reports documenting similar success with orthotic bracing for PC.1,5–7 In this report, we again demonstrate good success with custom-made braces for very noticeable pectus deformities in two motivated teenage boys.
Fabrication of these orthoses is a relatively simple procedure. There are a few prefabricated PC orthoses commercially available, but we choose to custom fabricate each patient due to the asymmetry of the protrusion abnormalities. Custom fabrication most importantly insures a lower profile and more comfortable orthosis with fewer problems associated with skin breakdown. To make a custom brace, first a negative cast is taken of the patient supine using plaster splints. This is then filled and modified slightly building up the most prominent aspect of the protrusion. A rigid material such as high-density polyethylene or copolymer is thermoformed over the protrusion and lined with a soft interface. The anterior and posterior bands made from aluminum are carefully contoured and are then covered with heat shrink tubing leaving adequate clearance for compression. These are connected with ladder buckles on each side to allow adjustment of the compression. The patient can monitor the compression by the position of these buckles and ladder straps and increase it at regular intervals. This keeps a gradual increase of compression on course and provides the clinician with a progress report. Shoulder straps are added to aid with suspension of the orthoses. The completed orthoses is shown in Figure 4.
Once a custom brace has been constructed, the correct application of the brace and diligence with brace wearing on the part of the patient are of paramount importance. Application of bracing in early teenage growth phase (before age 16) seems to be the optimal time for initiating this approach. Patients are typically instructed to wear the brace for 23 hrs a day for 3 to 6 months or until the defect is flattened. After this, the brace should be worn for 16 hours a day for the next 3 to 6 months, which allows the patient to participate in school activities without the brace. After complete subjective improvement is noted, the maintenance phase is initiated with nightly brace wearing until linear growth ceases.7 All patients should undergo re-evaluation every 3 months to ensure proper fit of the orthoses.
This report clearly documents, in a pictorial format, the potential efficacy of orthotic bracing for a child with a disfiguring PC deformity. In addition, we also describe the process of creating a custom pectus orthoses to achieve the best corrective result. Although similar results have been reported previously, there are no convincing imaging data in the literature to support the documented results and there is very little data on the actual construction of a custom orthosis. Here, we provide imaging that clearly demonstrates remarkable improvement in a PC deformity after only 2 to 3 months of bracing in two very motivated teenaged boys. From the images in this report, it is clear that orthotic bracing with a carefully constructed custom prostheses has the potential to improve the PC deformity to the point where it is barely discernable (Figs. 1, 3).
The importance of patient compliance and motivation in combination with strong parental support cannot be overemphasized. It is clear that in well motivated skeletally immature individuals, bracing can be an effective treatment for cosmetically displeasing PC. We also emphasize an individual-based approach with interval refitting that has been implicit in the excellent results demonstrated in our patients (now totaling 12). This report provides imaging data that clearly demonstrates the potential efficacy of orthotic bracing for PC in strongly motivated and compliant patients.
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3. Chidambaram B, Mehta AV. Currarino-Silverman syndrome (pectus carinatum type 2 deformity) and mitral valve disease. Chest 1992;102:780–782.
4. Mielke CH, Winter RB. Pectus carinatum successfully treated with bracing. A case report. Int Orthop 1993;17: 350–352.
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6. Egan JC, DuBois JJ, Morphy M, et al. Compressive orthotics in the treatment of asymmetric pectus carinatum: a preliminary report with an objective radiographic marker. J Pediatr Surg 2000;35:1183–1186.
7. Frey AS, Garcia VF, Brown RL, et al. Nonoperative management of pectus carinatum. J Pediatr Surg 2006;41:40–45; discussion 40–45.
KEY INDEXING TERMS: pectus carinatum; orthosis; bracing