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Poland Syndrome

  • Author: Bradon J Wilhelmi, MD; Chief Editor: James Neal Long, MD, FACS  more...
 
Updated: Feb 22, 2016
 

Background

Named after Sir Alfred Poland, Poland syndrome includes the features of ipsilateral breast and nipple hypoplasia and/or aplasia, deficiency of subcutaneous fat and axillary hair, absence of the sternal head of the pectoralis major muscle, hypoplasia of the rib cage, and hypoplasia of the upper extremity. In 1841, Sir Alfred Poland described this chest wall anomaly in the Guy's Hospital Gazette while still a medical student based on findings of one cadaver dissection. In his original description, titled "Deficiency of the pectoral muscles," he specifically noted absence of the sternocostal portion of the pectoralis major muscle with an intact clavicular origin, absence of the pectoralis minor muscle, and hypoplastic serratus and external oblique muscles. Poland did not outline the breast hypoplasia or hand deformities in his original description.

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Problem

Poland syndrome can present with ipsilateral involvement of the chest muscles, skin and subcutaneous tissues, bones, and upper extremity.[1, 2] The absence of the sternal head of the pectoralis major muscle is considered the minimal expression of this syndrome. Involvement of adjacent muscles, including the pectoralis minor, serratus, latissimus dorsi, and external oblique, also has been described.

The skin of the area is hypoplastic with a thinned subcutaneous layer, and the axillary hair may be absent. The ipsilateral nipple is often smaller and higher in both male and female patients, and the breast is generally hypoplastic in female patients.

Skeletal deformities may involve absence of portions of the ribs or costal cartilages anteriorly. In severe cases, anterior lung herniation may be present. The scapula may be smaller with winging; this is termed Sprengel deformity. (A study by Yiyit et al of 113 patients with Poland syndrome found Sprengel deformity to be the most frequent concurrently existing anomaly, with the condition present in 18 patients [16%].[3] ) The upper extremity also may be hypoplastic. The upper arm, forearm, and fingers may be shortened, which is termed brachysymphalangism. Simple, complete, or incomplete syndactyly can also be found in patients with Poland syndrome.

The classic ipsilateral features of Poland syndrome include the following:

  • Absence of sternal head of the pectoralis major muscle
  • Hypoplasia and/or aplasia of breast or nipple (athelia)
  • Deficiency of subcutaneous fat and axillary hair
  • Abnormalities of rib cage
  • Upper extremity anomalies; short upper arm, forearm, or fingers (brachysymphalangism)

Additional features of Poland syndrome include the following:

  • Hypoplasia or aplasia of serratus, external oblique, pectoralis minor, latissimus dorsi, [1] infraspinatus, and supraspinatus muscles
  • Total absence of anterolateral ribs and herniation of lung
  • Symphalangism with syndactyly and hypoplasia or aplasia of the middle phalanges
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Epidemiology

Frequency

Poland syndrome is uncommon but not rare. While plastic surgeons encounter more female patients than male patients with this deformity (because the female patients seek out treatment of breast asymmetry), no gender predilection is exhibited. Many men remain undiagnosed unless they seek attention for the treatment of associated hand anomalies. Because Poland syndrome is underreported and infrequently diagnosed, the exact incidence is difficult to determine. In one review, the incidence of Poland syndrome was estimated at 1 in 30,000. The right side is affected twice as often as the left.

Most Poland syndrome cases arise sporadically. However, several reports exist of family members and twins with the same diagnosis, suggesting some degree of genetic transmission. Poland syndrome has been associated with other syndromes, including Möbius syndrome (congenital bilateral facial paralysis with inability to abduct the eyes) and Klippel-Feil syndrome. Hematopoietic malignancies, including leukemia and non-Hodgkin lymphoma, have been described in patients with Poland syndrome.

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Etiology

Although several theories have been advanced to explain the etiology of Poland syndrome, most evidence indicates that it results from a vascular developmental anomaly during the critical sixth week of gestation, with hypoplasia of the subclavian artery causing musculoskeletal malformations. The critical vascular event, known as subclavian artery supply disruption sequence (SASDS), occurs when the medial and forward growth of the ribs forces the subclavian vessel into a U-shaped configuration. The specific region of vessel involvement dictates the clinical manifestation (ie, Poland syndrome, Möbius syndrome, Klippel-Feil syndrome), and more proximal occlusions result in more severe syndromes.

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Presentation

Because the functional disability in Poland syndrome is mild, patients usually present later for evaluation and discussion on aesthetic options. Preoperative evaluation should include a thorough history and examination.[4]

During the examination, the stage of breast development and status of the latissimus dorsi muscle should be noted. The presence and development of the latissimus dorsi muscle can be determined with the provocative maneuver of downward strain by the patient with his or her hand on the hip. Furthermore, extraocular muscle motion must be confirmed in excluding an associated Möbius syndrome. Examination of lymph nodes and complete blood cell count should assist with evaluation for associated leukemia and non-Hodgkin lymphoma. Determining chest wall abnormalities and the presence of the latissimus muscle may require evaluation with CT scan.[5]

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Indications

Patients with Poland syndrome present for treatment of the chest deformity and breast asymmetry.[6] Three main determinants influence the timing and options for reconstruction: breast development, existence of a latissimus dorsi muscle, and degree of chest wall deformity.

If the breasts are not fully developed, use of autologous tissues for reconstruction is delayed until such time. During breast development, females may benefit from provisional breast reconstruction with tissue expansion. The hypoplastic breast can be expanded incrementally to match breast development on the unaffected side with a temporary tissue expander breast implant.[7] This helps stretch the contracted skin and potentially lowers the raised nipple in preparation for the latissimus flap reconstruction. Once the breasts are developed and the breast envelope is appropriately stretched, the latissimus muscle can be used with or without a permanent implant, depending on the desired size.[8, 9]

A patient with minor breast asymmetry may be a candidate for correction with a permanent implant expander. An implant expander has a port that can be used to incrementally fill the implant over time postoperatively until symmetry is achieved; the port is then removed through a separate incision and the implant is left in place permanently. The most popular breast implant expander is the Becker implant, which has silicone gel and is filled with saline.

In male patients, the chest deformity can be reconstructed with the latissimus dorsi muscle as early as age 13 years. When rib abnormalities are mild, reconstruction with the latissimus dorsi muscle affords satisfactory chest wall symmetry. However, if severe, associated rib abnormalities may need to be treated to optimize the eventual outcome.

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Contraindications

Breast implants and expanders should not be placed in patients who are too young to maturely accept the responsibility of a foreign body and to have the ability to deal with the potential complications. Patients must be able to tolerate occasional expansion procedures. Remote port tissue expanders may be preferred for younger patients. Integrated port tissue expanders can be used in young patients with an increased risk of deflation because the actual implant can be punctured in attempting to access the port in a moving target.

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Contributor Information and Disclosures
Author

Bradon J Wilhelmi, MD Leonard J Weiner Professor and Chief of Plastic Surgery, Plastic Surgery Residency Program Director, Hiram C Polk Jr Department of Surgery, University of Louisville School of Medicine

Bradon J Wilhelmi, MD is a member of the following medical societies: Alpha Omega Alpha, American Association for Hand Surgery, American Society for Reconstructive Microsurgery, Association for Surgical Education, Plastic Surgery Research Council, American Association of Clinical Anatomists, Wound Healing Society, American Society for Aesthetic Plastic Surgery, American Burn Association, American College of Surgeons, American Society for Surgery of the Hand, American Society of Plastic Surgeons

Disclosure: Nothing to disclose.

Coauthor(s)

Michael Neumeister, MD, FRCSC, FACS Chairman, Professor, Division of Plastic Surgery, Director of Hand/Microsurgery Fellowship Program, Chief of Microsurgery and Research, Institute of Plastic and Reconstructive Surgery, Southern Illinois University School of Medicine

Michael Neumeister, MD, FRCSC, FACS is a member of the following medical societies: American Association for Hand Surgery, American Burn Association, American College of Surgeons, American Medical Association, American Society for Surgery of the Hand, American Society of Plastic Surgeons, American Society for Reconstructive Microsurgery, Canadian Society of Plastic Surgeons, Illinois State Medical Society, Illinois State Medical Society, Ontario Medical Association, Plastic Surgery Research Council, Royal College of Physicians and Surgeons of Canada, Society of University Surgeons, American Council of Academic Plastic Surgeons

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

James Neal Long, MD, FACS Founder of Magnolia Plastic Surgery; Former Associate Professor of Plastic and Reconstructive Surgery, Division of Plastic Surgery, Children's Hospital and Kirklin Clinics, University of Alabama at Birmingham School of Medicine; Section Chief of Plastic, Reconstructive, Hand, and Microsurgery, Birmingham Veterans Affairs Medical Center

James Neal Long, MD, FACS is a member of the following medical societies: Alpha Omega Alpha, American College of Surgeons, American Medical Association, American Society of Plastic Surgeons, Plastic Surgery Research Council, Sigma Xi, Southeastern Society of Plastic and Reconstructive Surgeons, Southeastern Surgical Congress

Disclosure: Nothing to disclose.

Additional Contributors

Pankaj Tiwari, MD Assistant Professor, Division of Plastic Surgery, Ohio State University College of Medicine

Disclosure: Nothing to disclose.

Acknowledgements

Paige Bryant Cornette, MD Staff Physician, Department of Plastic Surgery, Southern Illinois University School of Medicine

Disclosure: Nothing to disclose.

References
  1. Cochran JH Jr, Pauly TJ, Edstrom LE, Dibbell DG. Hypoplasia of the latissimus dorsi muscle complicating breast reconstruction in Poland's syndrome. Ann Plast Surg. 1981 May. 6(5):402-4. [Medline].

  2. Longaker MT, Glat PM, Colen LB, Siebert JW. Reconstruction of breast asymmetry in Poland's chest-wall deformity using microvascular free flaps. Plast Reconstr Surg. 1997 Feb. 99(2):429-36. [Medline].

  3. Yiyit N, Isıtmangil T, Oksuz S. Clinical analysis of 113 patients with Poland syndrome. Ann Thorac Surg. 2015 Mar. 99 (3):999-1004. [Medline].

  4. Seyfer AE, Fox JP, Hamilton CG. Poland syndrome: evaluation and treatment of the chest wall in 63 patients. Plast Reconstr Surg. 2010 Sep. 126(3):902-11. [Medline].

  5. Bainbridge LC, Wright AR, Kanthan R. Computed tomography in the preoperative assessment of Poland's syndrome. Br J Plast Surg. 1991 Nov-Dec. 44(8):604-7. [Medline].

  6. Moir CR, Johnson CH. Poland's syndrome. Semin Pediatr Surg. 2008 Aug. 17(3):161-6. [Medline].

  7. Argenta LC, VanderKolk C, Friedman RJ, Marks M. Refinements in reconstruction of congenital breast deformities. Plast Reconstr Surg. 1985 Jul. 76(1):73-82. [Medline].

  8. Hester TR Jr, Bostwick J 3rd. Poland's syndrome: correction with latissimus muscle transposition. Plast Reconstr Surg. 1982 Feb. 69(2):226-33. [Medline].

  9. Moelleken BR, Mathes SA, Chang N. Latissimus dorsi muscle-musculocutaneous flap in chest-wall reconstruction. Surg Clin North Am. 1989 Oct. 69(5):977-90. [Medline].

  10. Gatti JE. Poland's deformity reconstructions with a customized, extrasoft silicone prosthesis. Ann Plast Surg. 1997 Aug. 39(2):122-30. [Medline].

  11. Hochberg J, Ardenghy M, Graeber GM, Murray GF. Complex reconstruction of the chest wall and breast utilizing a customized silicone implant. Ann Plast Surg. 1994 May. 32(5):524-8. [Medline].

  12. Marks MW, Iacobucci J. Reconstruction of congenital chest wall deformities using solid silicone onlay prostheses. Chest Surg Clin N Am. 2000 May. 10(2):341-55, vii. [Medline].

  13. Manzano Surroca M, Ribo Cruz JM, Parri Ferrandis F, et al. [Poland's syndrome and free autologous fat grafts]. Cir Pediatr. 2014 Jan. 27(1):43-8. [Medline].

  14. Seifarth FG, Cruz Pico CX, Stromberg J, Recinos VM, Burdjalov VF, Karakas SP. Poland syndrome with extracorporeal intercostal liver herniation and thoracic myelomeningocele. J Pediatr Surg. 2012 Jan. 47(1):e13-7. [Medline].

  15. Mojallal A, La Marca S, Shipkov C, Sinna R, Braye F. Poland syndrome and breast tumor: a case report and review of the literature. Aesthet Surg J. 2012 Jan 1. 32(1):77-83. [Medline].

  16. Li W, Zhang L, Zhang Q, Du J, Zhang S, Liu X. Poland syndrome associated with ipsilateral lipoma and dextrocardia. Ann Thorac Surg. 2011 Dec. 92(6):2250-2. [Medline].

 
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