Poland Sequence: Retrospective Analysis of 66 Cases.

Area of Science:

• Congenital disorders
• Plastic surgery
• Medical classification systems

Background:

• Poland syndrome (PS) is a rare congenital disorder with variable thoracic and upper extremity malformations.
• Existing classifications may not fully capture the spectrum of PS, necessitating refinement for optimal management.
• The pathognomonic feature is the absence/aplasia of the sternocostal aspect of the pectoralis major muscle.

Purpose of the Study:

• To analyze patterns and outcomes of Poland syndrome patients diagnosed at a single institution.
• To revise the diagnosis and classification of PS based on new proposals.
• To improve assessment and initial management strategies for suspected PS cases.

Main Methods:

• Retrospective study of 66 PS patients from 1988 at a Pediatric Plastic Surgery Unit.
• Analysis of medical charts, records, photographs, and imaging.
• Systematic literature review of PubMed, Cochrane Plus, Scopus, Web of Science, and Europe PMC.

Main Results:

• The most frequent PS phenotype observed was T1B1N1S0 (hypoplasia/aplasia of pectoralis muscle, chest/breast hypoplasia, no upper extremity involvement).
• Scoliosis was the most common associated malformation.
• Literature review indicated a 1:1 male to female ratio for PS, contrary to previous reports.

Conclusions:

• Propose an updated TBN classification (TBNS) incorporating upper extremity involvement for improved PS management.
• Personalized surgical reconstruction, including implants and fat grafting, yields high satisfaction and low complication rates.
• Standardized imaging documentation is recommended for presurgery and postsurgery assessment.