Surgery for Chest Wall Deformities by Gary W. Raff & Shinjiro Hirose

Author:Gary W. Raff & Shinjiro Hirose
Language: eng
Format: epub
Publisher: Springer International Publishing, Cham

Fig. 6.5(A, B) Different degrees of Poland syndrome severity. (A) Partial PS with pectoral muscle deficit only. (B) Full-blown PS with large rib defect, extensive muscle deficit, isolated dextrocardia, and unilateral hand abnormality. (Used with permission from Fokin, AA.; Steuerwald, NM.; Ahrens, WA.; Allen, KE. Anatomical, Histologic, and Genetic Characteristics of Congenital Chest Wall Deformities. Semin Thorac Cardiovasc Surg. 2009; 21:44–57)

Diagnosis

Although the presentation of Poland’s Syndrome is quite variable, the sine qua none for the disease is the congenital absence of the sternocostal head of the pectoralis major muscle. This reflects the simplest and most common form of the syndrome; and is recognized, in either gender, as an absent anterior axillary fold. The female deformity, in its mildest form, manifests with the concomitant finding of breast hypoplasia. The simultaneous occurrence of additional ipsilateral anomalies of the thorax and upper extremity upstage the diagnosis to one of the more severe forms, rounding out the full spectrum of the syndrome.

Prenatal diagnosis is possible by ultrasonic evaluation; but the diagnosis is more commonly made after birth by the constellation of clinical findings [19]. Parents typically notice the severe form of the syndrome early in infancy due to a visibly asymmetric chest wall and brachysyndactyly. The simple form, however, even if known to the patient, may remain hidden from the family until early adolescence when the patient becomes more self-conscious about the asymmetry.

While the majority of patients with Poland’s Syndrome present primarily with an aesthetic concern, in the rare case of cardiopulmonary compromise, severe functional impairment is recognized in infancy and should be addressed with prompt surgical intervention. A thorough assessment of all patients with Poland’s Syndrome is necessary for a complete and accurate diagnosis. Those with the mild form of the syndrome may have measureable differences in the affected hemithorax and upper extremity that may otherwise go unnoticed by visual inspection alone. This also highlights the importance of a thorough examination in individuals presenting with an apparent isolated syndactyly, as up to 10 % of these patients will have Poland’s Syndrome [20].

Physical examination should involve an undraped evaluation of the entire torso and bilateral upper extremities, with inspection, palpation, and measurement of key structural components. This should include measurement of the ribs; as well the distances from sternal notch to acromion, olecranon to ulnar styloid, and lengths of all phalanges; all of which should be compared to measurements on the contralateral, unaffected side. Standard muscle testing is performed, and baseline photographs are taken in the usual fashion [2].

While not a requirement for diagnosis , various imaging modalities may assist with preoperative planning, particularly in cases where autogenous reconstruction is being considered. Computed Topography (CT), Magnetic Resonance Imaging (MRI), and Angiography are the most useful; permitting a more detailed assessment of the skeletal, soft tissue, and vascular anomalies, respectively. Beer et al., in their report of a case of Poland’s Syndrome in a 17-year-old girl, were forced to abort a pedicled latissimus dorsi flap due to severe attenuation of the muscle that was not recognized clinically on preoperative examination.

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