You are in: eMedicine Specialties > Plastic Surgery > BREAST Breast, Poland SyndromeArticle Last Updated: Jun 28, 2006AUTHOR AND EDITOR INFORMATIONSection 1 of 9
  Author: Bradon J Wilhelmi, MD, Endowed Leonard Weiner, MD, Professor and Chief of Division of Plastic Surgery, Residency Program Director, University of Louisville School of Medicine Bradon J Wilhelmi is a member of the following medical societies: Alpha Omega Alpha, American Association for Hand Surgery, American Association of Clinical Anatomists, American Association of Plastic Surgeons, American Burn Association, American College of Surgeons, American Society for Reconstructive Microsurgery, American Society for Surgery of the Hand, American Society of Aesthetic Plastic Surgery, American Society of Plastic Surgeons, Association for Surgical Education, Plastic Surgery Research Council, and Wound Healing Society Coauthor(s): Paige Bryant Cornette, MD, Staff Physician, Department of Plastic Surgery, Southern Illinois University School of Medicine; Michael Neumeister, MD, FRCSC, FACS, Program Director, Assistant Professor, Department of Surgery, Division of Plastic Surgery, Southern Illinois University School of Medicine Editors: Christian Paletta, MD, FACS, Professor, Division Chief and Program Director, Department of Plastic and Reconstructive Surgery, St Louis University School of Medicine; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; Saleh M Shenaq, MD†, Former Director and Founder, The International Brachial Plexus Institute; Former Chief, Section of Plastic Surgery, Methodist Hospital, Houston; Nicolas (Nick) G Slenkovich, MD, Practice Director, Colorado Plastic Surgery Center at Swedish Medical Center; Lars M Vistnes, MD, FRCSC, FACS, Professor of Surgery, Emeritus, Stanford University Medical Center Author and Editor Disclosure Synonyms and related keywords: poland syndrome, ipsilateral absence of sternal head of the pectoralis major, breast hypoplasia, breast aplasia, nipple aplasia, nipple hypoplasia, subcutaneous fat deficiency, axillary hair deficiency, rib cage abnormalities, upper extremity anomalies, short upper arm, short forearm, short fingers, symphalangism INTRODUCTIONSection 2 of 9
  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, 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, and hypoplastic serratus and external oblique muscles. Poland did not outline the breast hypoplasia or hand deformities in his original description. ProblemPoland syndrome can present with ipsilateral involvement of the chest muscles, skin and subcutaneous tissues, bones, and upper extremity. 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 the 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 nipple is often smaller and higher in both males and females and the breast is generally hypoplastic in females. 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, termed Sprengel deformity. 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 also can be found in patients with Poland syndrome. The classic ipsilateral features of Poland syndrome include the following:
Additional features of Poland syndrome include the following:
FrequencyPoland syndrome is uncommon but not rare. While plastic surgeons encounter more females than males with this deformity (because they 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. EtiologyAlthough several theories have been advanced to explain the etiology of Poland syndrome, most evidence indicates that it results from a vascular event 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. ClinicalBecause 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. During the examination, note the stage of breast development and status of the latissimus dorsi muscle. The development of the latissimus dorsi muscle can be ascertained 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. Chest wall abnormalities and determining the presence of the latissimus muscle may require evaluation with CT scan. INDICATIONSSection 3 of 9
Patients with Poland syndrome present for treatment of the chest deformity and breast asymmetry. 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. Once the breasts are developed, the latissimus muscle can be used. In males, 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 should be treated to optimize the eventual outcome. CONTRAINDICATIONSSection 4 of 9
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. Integrated port tissue expanders should not be used in young patients because the expander can be deflated in attempting to access the port in a moving target. WORKUPSection 5 of 9
Lab Studies
Imaging Studies
TREATMENTSection 6 of 9
Surgical therapyIn women, abnormal breast development can influence the psychological growth of a teenager. However, if the reconstruction is performed too early, asymmetry may result with continued growth of the breasts, and a secondary revision procedure may be required once breast development is completed. When the young female patient experiences intense anxiety associated with the breast deformity, consider early treatment. During adolescence, a subcutaneous tissue expander can be placed in the affected side. The expander is placed in the subcutaneous plane since the pectoralis muscle is absent. This expander can be inflated at intervals to match the development of the unaffected breast. Placement of the expander during breast development allows for expansion of the skin to accommodate the eventual permanent implant and latissimus muscle. Moreover, this expansion of the breast skin may enlarge the hypoplastic nipple-areolar complex (NAC) often present in these patients. Tissue expansion also can correct the eccentric and elevated NAC observed in patients with Poland syndrome through strategic expander placement to lower it. Occasionally, with long periods of implant inactivity between expansions, a capsule contracture develops, which can restrict expansion, and an open capsulotomy and implant repositioning may be required. Finally, upon completion of breast development (when the patient is aged 18-19 y), the tissue expander can be removed and the breast can be reconstructed with the latissimus muscle transposed over a permanent implant. The latissimus muscle is used to correct the absence of the axillary line, correct infraclavicular flattening, and provide subcutaneous filler to cover the implant, thus preventing rippling. Occasionally, a de-epithelialized skin paddle may be required with the latissimus muscle to reconstruct the axillary line. The latissimus muscle is harvested through a small axillary incision and a transverse incision in the back, which can be concealed in the bra line. The muscle is transposed anteriorly over the breast implant and sutured to the pectoralis fascia superiorly, medially, and inferiorly. The use of the latissimus is saved until the completion of breast development and tissue expansion; if used earlier over the tissue expander, the muscle is attenuated with expansion. When striving to achieve breast symmetry at the final operation, treatment of the contralateral breast by reduction, mastopexy, or augmentation may be indicated. Furthermore, over time, the patient may develop a unilateral contracture to the breast prosthesis, resulting in loss of symmetry. Accordingly, long-term symmetry may be optimized by placement of an implant in the unaffected breast as well. If the unaffected breast is excessively large, it may require a reduction or mastopexy as an adjunct to implant insertion. If tissue expansion does not correct nipple-areolar asymmetry, additional procedures may be needed to correct nipple and areolar size and location. Consider nipple-sharing composite grafts if the unaffected nipple is of adequate size, or consider nipple reconstruction with local flaps. Areolar discrepancies can be corrected with crescent excisions, strategic tattooing, or relocation by transposing through a new skin opening and skin closure of old location. These nipple-areolar reconstruction procedures are performed at a separate stage. If the latissimus is absent as part of the Poland syndrome complex, other options for reconstruction include free latissimus muscle from the unaffected side, the transverse rectus abdominus muscle (TRAM) flap, Rubens flap, gluteus maximus flap, thigh flap, or free perforator TRAM or gluteus flaps. Another described reconstructive option is a customized prosthetic implant for the chest wall placed under the breast implant. Because thoracic vascular anomalies also can be encountered in patients with Poland syndrome, assessment of recipient vessels with duplex or angiogram is required. The TRAM flap should not be considered for those who intend to become pregnant. The gluteus and Rubens flaps have short pedicles, often requiring grafts for microanastomoses, and result in significant donor site asymmetry. In general, these other reconstructive options provide less aesthetically acceptable results than the latissimus muscle over implant procedure; however, treatment must be individualized. For males with Poland syndrome who have an intact latissimus muscle, consider reconstruction when they are aged 12-13 years. The ipsilateral latissimus muscle is harvested through a small incision in the back and axilla and transposed to fill the void of the absent pectoralis major muscle. The latissimus is folded along the sternal and inferior borders to resemble the contour of the pectoralis major muscle. The humeral insertion of the latissimus muscle must be detached, anteriorly transposed, and sutured to the bicipital groove of the humerus. Reconstruction with prosthesis also has been described for males but with disappointing results. If the latissimus is absent on the affected side, the contralateral latissimus can be used as a free flap to the axillary or internal mammary vessels after appropriate evaluation for recipient vessels with duplex or angiogram. Intraoperative detailsSee Surgical therapy. Postoperative detailsInsertion of the tissue expander is performed on an outpatient basis. The patient's follow-up care is initially for wound evaluation. Then, patients return for suture removal at 2 weeks and the initial expansion if wound healing is suitable. Tissue expansion then is performed at weekly intervals until the affected breast matches the contralateral side. The affected breast is expanded further as the unaffected breast grows. The second procedure, involving expander exchange to permanent implant and latissimus muscle reconstruction, involves placement of a drain in the donor site and breast pocket. An Ace wrap is used to minimize potential space at donor site and risk of hematoma and/or seroma. Drain care can be performed at home. These drains are removed when outputs are less than 25 mL over 24 hours. Expansion exercises over the implants are begun at 4 weeks. COMPLICATIONSSection 7 of 9
Any of the complications described for breast reconstruction with implants and autogenous tissue can be experienced in the reconstruction of patients with Poland syndrome, including implant infection, seroma or hematoma around the implant, implant displacement, deflation, exposure, contracture, pain, flap loss, muscle atrophy, progressive change in symmetry, latissimus donor site seroma and hematoma, back asymmetry from latissimus transposition, and need for additional revisions. OUTCOME AND PROGNOSISSection 8 of 9
With appropriate timing, procedure selection, and preoperative evaluation, excellent results and high patient satisfaction can be achieved in the treatment of chest and breast deformities resulting from Poland syndrome. Through the early use of tissue expansion, breast growth can be achieved to match the normal development on the unaffected side. Tissue expansion prepares the breast envelope for the autogenous reconstruction and permanent implant and can correct nipple and areolar irregularities as well. Finally, upon completion of breast development, the latissimus muscle can be used to reconstruct the axillary line, reconstruct the infraclavicular hollow, and provide implant coverage. REFERENCESSection 9 of 9
Breast, Poland Syndrome excerpt Article Last Updated: Jun 28, 2006 |
