Affiliation:
1. Department of Pediatric Surgery
2. Department of Surgery, Nepal Medical College and Teaching Hospital, Kathmandu, Nepal
Abstract
Introduction:
Poland syndrome is a rare congenital syndrome that mostly includes the absence of unilateral pectoralis major muscle and digit anomalies like symbrachydactyly. It can also present with other varied manifestations like the absence of ribs, bilateral absence of pectoralis major muscle, urogenital anomalies, dextrocardia, etc.
Case Presentation:
We herein present a case of a 6-year-old boy with Poland syndrome as an incidental finding, the second one reported from Nepal, 11 years after the first report. The syndrome was diagnosed after the patient came to the hospital for treatment of phimosis. On examination, the sternocostal head of the right pectoralis major muscle was absent with a palpable clavicular head with symbrachydactyly of the ipsilateral side.
Discussion:
Poland syndrome is mostly diagnosed clinically. Its differential diagnosis includes other chest wall anomalies, nipple anomalies, isolated thoracic lipoatrophy, and isolated hand/upper limb anomalies without pectoralis major muscle involvement. Computed tomography and MRI scans can help delineate the exact chest wall abnormality. It also helps to find other manifestations of the syndrome and for the treatment plans.
Conclusion:
Poland syndrome is a rare syndrome that has the propensity to be missed in clinical settings. Treatment, mainly sought for cosmetic reasons, includes breast augmentation procedures or myocutaneous flap coverage.
Publisher
Ovid Technologies (Wolters Kluwer Health)
Reference13 articles.
1. Deficiency of the pectoral muscles;Poland;Guys Hosp Rep,1841
2. Poland’s syndactyly;Clarkson;Guys Hosp Rep,1962
3. Poland’s syndrome: a mammographic presentation;Samuels;AJR Am J Roentgenol,1996
4. The SCARE 2020 guideline: updating consensus Surgical CAse REport (SCARE) guidelines;Agha;Int J Surg,2020
5. A variant of Poland’s syndrome – case report and review of literature;Rauniyar;NJR,2011