Presentation and Treatment of Poland Anomaly

Abstract

Background: Poland anomaly is a sporadic, phenotypically variable congenital condition usually characterized by unilateral pectoral muscle agenesis and ipsilateral hand deformity. Methods: A comprehensive review of the medical literature on Poland anomaly was performed using a Medline search. Results: Poland anomaly is a sporadic, phenotypically variable congenital condition usually characterized by unilateral, simple syndactyly with ipsilateral limb hypoplasia and pectoralis muscle agenesis. Operative management of syndactyly in Poland anomaly is determined by the severity of hand involvement and the resulting anatomical dysfunction. Syndactyly reconstruction is recommended in all but the mildest cases because most patients with Poland anomaly have notable brachydactyly, and digital separation can improve functional length. Conclusions: Improved understanding the etiology and presentation of Poland anomaly can improve clinician recognition and management of this rare congenital condition.

Keywords: Poland anomaly; Poland syndrome; congenital hand; pectoral agenesis; symbrachydactyly.

Figure 1.

Chest wall deformity in Poland anomaly. Source. Photograph A, courtesy of Adrian E. Flatt, MD. Note. (A) A 16-month-old male with right chest wall deformity. (B) An 18-month-old female with right chest wall deformity. Note the hypoplasia of the pectoralis musculature and rib anomalies present in A and underdevelopment of the right areola in B, highlighting the variability in clinical presentation.

Figure 2.

Symbrachydactyly in 18-month-old female with Poland anomaly. Note. (A) Dorsal and (B) volar views of simple incomplete syndactyly of a right hand’s second and fourth webspaces. Note concomitant brachydactyly with complete absence of the middle phalanx in the index and small fingers, which corresponds to the presence of a single interphalangeal crease. (C) Anteroposterior hand radiograph demonstrates the absence of index and small finger middle phalanges that reflects clinical examination.

Figure 3.

Symbrachydactyly in Poland anomaly. Source. Photograph courtesy of Adrian E. Flatt, MD. Note. (A) Dorsal and (B) volar views of left symbrachydactyly in a 5-year-old male with Poland anomaly. (C) Dorsal and (D) volar views of left symbrachydactyly in a 4-year-old male with Poland anomaly.

Figure 4.

Syndactyly reconstruction in Poland anomaly. Note. (A) Volar and (B) dorsal views demonstrating surgical markings for planned syndactyly reconstruction of second and fourth webspaces of right hand in a 18-month-old female. Three-flap web-plasty planned for fourth webspace, obviating need for skin grafting. (C) Intraoperative image demonstrating distal bifurcation of the common digital artery in the second webspace. The radial digital artery of the long finger was ligated after confirming perfusion with temporary application of a small vessel clamp. (D) Postoperative image demonstrating final flap position in the fourth webspace.

Figure 5.

Two-year follow-up after syndactyly reconstruction for Poland anomaly. Source. Photograph courtesy of Adrian E. Flatt, MD. Note. (A) Preoperative dorsal and (B) volar views demonstrating symbrachydactyly of right hand in a 16-month-old male. (C) Postoperative dorsal and (D) volar views 2 years following third webspace reconstruction.