Monday, January 4, 2010

Clinical and Radiographic Poland Syndrome Classification: A Proposal – an Article Review

The authors of the recent Aesthetic Surgery Journal article (full reference below) have proposed a new classification of Poland Syndrome based on both clinical and radiographic presentation (CRPS). Using their CRPS classification, they present an algorithm for planning surgical treatment.

The article begins with a review of Poland Syndrome:

Classically, it consists of a combination of unilateral aplasia of the sternocostal portion of the pectoralis major muscle (PMM) and hypoplasia of the ipsilateral hand, with syndactyly and synbrachydactyly.

The reported incidence of Poland Syndrome (PS) is one in 30,000 live births. Male-to-female ratio is 3:1. The right side is affected twice as often as the left.

The etiology of PS is still unknown, but recently a vascular hypothesis suggests hypoplasia of the ipsilateral subclavian artery.

Using data obtained from 28 female patients, they classify PS patients as follows:

First Degree (Mild): The diagnosis of first-degree PS would be made in a patient with mammary asymmetry caused by hypomastia or amastia and areolar asymmetry, with or without a partial absence of the pectoralis major muscle (PMM). No other musculoskeletal alterations are observed; other congenital alterations may or may not be present.

Second Degree (Severe): Hypomastia or amastia, areolar asymmetry, total absence of the PMM, and alterations of the ipsilateral muscle group and/or bones of the chest results in a diagnosis of second-degree PS; ipsilateral superior limb alteration and other congenital alterations may or may not be present.

Third Degree (Very Severe): Third-degree PS would be diagnoses in patients with amastia; areolar asymmetry; major ipsilateral musculoskeletal chest alterations, such as total absence of the PMM, the pectoralis minor muscle, and /or the serratus anterior muscle; possible lung herniation; widened opening of the mediastinum; and ipsilateral superior limb alteration. Other congenital alterations may or may not be present.

Suggested surgical approach for each degree of presentation:

First Degree (mild) – Breast implantation or customized breast implantation and contralateral mammary reduction or augmentation when needed (symmetrization procedure).

Second Degree (severe) – Tissue expander placement when needed; regional local flap surgery; breast implantation or customized breast implantation; symmetrization procedure.

Third Degree (very severe) – Tissue expander placement; latissimus dorsi flap or other flap surgery, such as a free flap or transverse rectus abdominis myocutaneous (TRAM) flap; breast implantation or customized breast implantation as needed; other surgeries such as the Ravitch procedure and a contralateral symmetrization procedure.

REFERENCE

Clinical and Radiographic Poland Syndrome Classification: A Proposal; Aesthetic Surgery Journal, Volume 29, Issue 6, Pages 494-504 (November 2009); Ricardo Cavalcanti Ribeiro, Renato Saltz, M. Gabriela Moreira Mangles, Hilton Koch (subscription required)

1 comment:

StorytellERdoc said...

Great reading about Poland Syndrome, which I've never seen in the ER, and Roosevelt's big secret. Awesome reading and learning from my part. Thanks. Stay warm.