Clinical and Radiographic Poland Syndrome Classification: A Proposal

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494 • Volume 29 • Number 6 • November/December 2009 Aesthetic Surgery Journal Breast Surgery P oland syndrome (PS) is a rare abnormality with a sporadic presentation characterized by congenital malformations of the chest wall, with or without alterations to the ipsilateral superior limbs and hands. 1,2 Classically, it consists of a combination of unilateral aplasia of the sternocostal portion of the pectoralis major muscle (PMM) and hypoplasia of the ipsilateral hand, with syndactyly 3,4 and synbrachydactyly. 5 Only 400 cases of PS were reported by 1990; in these cases, several degrees of chest wall malformations extending to the superior limbs were seen. 1 The incidence of PS has a male-to-female ratio of 3:1 and its frequency is estimated at one in 30,000 live births, 6–11 with the right side being affected twice as often as the left side. 12,13 The etiology of PS is still unknown. Several studies have suggested that a genetic factor or, even more likely, extrinsic factors between the sixth and eighth weeks of pregnancy may interfere with PMM migration and the separation of the digits (chirodactile) that occurs in this period. 6–12 The recent vascular hypothesis for the etiology of PS implies that hypoplasia of the ipsilateral subclavian artery is the origin of this birth anomaly. 13–16 The main deformities associated with PS are as follows: (1) Muscles—Absence of the pectoralis minor muscle (PMM), absence of the clavicular and sternal seg- ments of the PMM, 17 and hypoplasia of the serratus anterior, 18,19 latissimus dorsi, deltoid, and infraspina- tus and supraspinatus muscles. 20 (2) Mammary glands—Areolar abnormalities, mammary absences, or hypoplasia. 17,18–21 Background: Many chest wall deformities have a characteristic radiologic appearance that can be the basis for a definitive diagnosis. Consequently, imaging techniques have fundamental roles in the detection, location, and characterization of these disorders. Objective: The authors propose a clinical and radiographic Poland syndrome (CRPS) classification system and possible treatment algorithm for the thoracic manifestations of Poland syndrome (PS) in women, based on both clinical examinations and imaging studies. Methods: A retrospective study was conducted of 28 female patients evaluated over 17 years in the 28th Infirmary, Plastic and Reconstructive Surgery Division of the Hospital Santa Casa da Misericórdia do Rio de Janeiro, Rio de Janeiro, Brazil. After clinical examination, all patients underwent radiographic examination with chest radiographs, conventional computed tomography scans, magnetic resonance imaging and, in some cases, additional imaging studies. All clinical and radiologic variables were compiled in a database and used in the classification system, which included three levels of disease severity. Results: Based on the CRPS classification of the 28 female patients, 10 patients had first-degree PS, 14 patients had second-degree PS, and four patients had third-degree PS. Eighteen patients underwent surgical correc- tion; a total of 39 surgical procedures were performed using the CRPS algorithm. Conclusions: Identification of the severity of PS using the proposed classification system provided an accu- rate study of each patient and enabled better planning for the surgical correction of functional and aesthetic deformities. (Aesthet Surg J; 29:494-504.) Dr. Cavalcanti Ribeiro is a Professor and Coordinator of the Post Graduation Course in the 28th Infirmary, Dr. Saltz is Associate Professor of the Post Graduation Course in the 28th Infirmary, and Dr. Moreira Mangles is a postgraduate fellow in the 28th Infirmary, Division of Plastic and Reconstructive Surgery, Hospital Santa Casa da Misericórdia do Rio de Janeiro. Dr. Koch is a Professor in the Department of Radiology, School of Medicine of the Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil. Drs. Ribeiro and Saltz are members of the Brazilian Society of Plastic Surgery. Clinical and Radiographic Poland Syndrome Classification: A Proposal Ricardo Cavalcanti Ribeiro, MD; Renato Saltz, MD; M. Gabriela Moreira Mangles, MD; and Hilton Koch, MD I N T E R N A T I O N A L C O N T R I B U T I O N

Transcript of Clinical and Radiographic Poland Syndrome Classification: A Proposal

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Breast Surgery

Poland syndrome (PS) is a rare abnormality with asporadic presentation characterized by congenitalmalformations of the chest wall, with or without

alterations to the ipsilateral superior limbs and hands.1,2

Classically, it consists of a combination of unilateralaplasia of the sternocostal portion of the pectoralis majormuscle (PMM) and hypoplasia of the ipsilateral hand,with syndactyly3,4 and synbrachydactyly.5 Only 400 casesof PS were reported by 1990; in these cases, severaldegrees of chest wall malformations extending to thesuperior limbs were seen.1 The incidence of PS has a

male-to-female ratio of 3:1 and its frequency is estimatedat one in 30,000 live births,6–11 with the right side beingaffected twice as often as the left side.12,13 The etiologyof PS is still unknown.

Several studies have suggested that a genetic factoror, even more likely, extrinsic factors between the sixthand eighth weeks of pregnancy may interfere with PMMmigration and the separation of the digits (chirodactile)that occurs in this period.6–12 The recent vascularhypothesis for the etiology of PS implies that hypoplasiaof the ipsilateral subclavian artery is the origin of thisbirth anomaly.13–16

The main deformities associated with PS are as follows:(1) Muscles—Absence of the pectoralis minor muscle

(PMM), absence of the clavicular and sternal seg-ments of the PMM,17 and hypoplasia of the serratusanterior,18,19 latissimus dorsi, deltoid, and infraspina-tus and supraspinatus muscles.20

(2) Mammary glands—Areolar abnormalities, mammaryabsences, or hypoplasia.17,18–21

Background: Many chest wall deformities have a characteristic radiologic appearance that can be the basisfor a definitive diagnosis. Consequently, imaging techniques have fundamental roles in the detection, location,and characterization of these disorders.Objective: The authors propose a clinical and radiographic Poland syndrome (CRPS) classification system andpossible treatment algorithm for the thoracic manifestations of Poland syndrome (PS) in women, based onboth clinical examinations and imaging studies.Methods: A retrospective study was conducted of 28 female patients evaluated over 17 years in the 28thInfirmary, Plastic and Reconstructive Surgery Division of the Hospital Santa Casa da Misericórdia do Rio deJaneiro, Rio de Janeiro, Brazil. After clinical examination, all patients underwent radiographic examination withchest radiographs, conventional computed tomography scans, magnetic resonance imaging and, in somecases, additional imaging studies. All clinical and radiologic variables were compiled in a database and used inthe classification system, which included three levels of disease severity.Results: Based on the CRPS classification of the 28 female patients, 10 patients had first-degree PS, 14 patientshad second-degree PS, and four patients had third-degree PS. Eighteen patients underwent surgical correc-tion; a total of 39 surgical procedures were performed using the CRPS algorithm.Conclusions: Identification of the severity of PS using the proposed classification system provided an accu-rate study of each patient and enabled better planning for the surgical correction of functional and aestheticdeformities. (Aesthet Surg J; 29:494-504.)

Dr. Cavalcanti Ribeiro is a Professor and Coordinator of the PostGraduation Course in the 28th Infirmary, Dr. Saltz is AssociateProfessor of the Post Graduation Course in the 28th Infirmary, andDr. Moreira Mangles is a postgraduate fellow in the 28th Infirmary,Division of Plastic and Reconstructive Surgery, Hospital Santa Casada Misericórdia do Rio de Janeiro. Dr. Koch is a Professor in theDepartment of Radiology, School of Medicine of the UniversidadeFederal do Rio de Janeiro, Rio de Janeiro, Brazil. Drs. Ribeiro andSaltz are members of the Brazilian Society of Plastic Surgery.

Clinical and RadiographicPoland Syndrome Classification:A ProposalRicardo Cavalcanti Ribeiro, MD; Renato Saltz, MD; M. Gabriela Moreira Mangles, MD; andHilton Koch, MD

INTE

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IONAL CONTRIBUTION

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(3) Thorax—Pulmonary herniation, hypoplasia of theribs, or deformities of the hemithorax, depending onseverity.22

(4) Ipsilateral superior limb—Skeletal hypoplasia affectingthe hands,19 forearms, upper arms, and scapula; syn-dactyly, symbrachydactyly,17 polydactyly, brachysyn-dactyly, and axillary constrictions.1–3,23

Other abnormalities associated with PS include dextro-cardia, kidney hypoplasia,18–24 neurofibromatosis,9 myas-thenia gravis,8 peroneal atrophy (Charcot-Marie-ToothDisease), microcephaly, craniofacial dysplasias, and Möbiussyndrome.25,26 There have also been reports of associatedcoagulation disturbances, psoriasis, systemic lupus erythe-matosus, Baselow–Graves disease,27–29 leukemia, and non-Hodgkin lymphoma.30 All of these components are notnecessarily always present, but the full syndrome consistsof an assortment of these abnormalities.14

PS is characterized by different degrees of mammaryhypoplasia affecting the subcutaneous tissue and the adja-cent muscles, moreso when costal arc deformities and/orsyndactyly are also present.22 The spectrum of the defor-mities varies and may involve the simple absence of thesternocostal segment of the PMM or the total absence ofall the chest wall components except for the skin and thepleural membranes,22 a condition that is associated withthe risk of lung herniation during respiration.31 Unilateralhypoplasia of the breast and PMM without upper limbinvolvement are seen more often than other forms ofPS4–29 and are not uncommon conditions in women seek-ing mammary augmentation for breast asymmetry.13

Imaging techniques have a fundamental role in thedetection, location, and characterization of chest walldisorders, many of which have characteristic radiologicappearances that allow definitive diagnosis. Sternaldeformities can be seen with radiographic examinationsand their severity quantified with computed tomography(CT) scans,32 which clearly show many abnormalities.31

For chest wall disorders, the chest radiograph remainsthe basic imaging study for evaluating pathology. Chestradiographs generally show a hyperlucency on theaffected side, which mimics a radical mastectomy.22–29,31

A mammogram is useful in showing breast asymmetryor the absence of the PMM. PS has been diagnosed inone of 19,000 mammograms,33 according to Perez Aznaret al.34 Failure to show the PMM on a mammogram ismost often related to errors in positioning. Ultra -

sonography also has a complementary role in diagnosingthoracic pathology.34

The advent of cross-sectional imaging techniques,such as CT and magnetic resonance imaging (MRI), hasenabled the precise localization of chest wall lesionsand, in some cases, definitive diagnoses.32 The MRIshows a better tissue contrast, but is less useful than CTin the study of bone pathologies.25 CT also depicts moreclearly the absence of the PMM and allows for the betterappreciation of nearby associated musculoskeletal anom-alies,32 such as mediastinal widening.35 CT and MRItogether define, with precision, the muscular abnormali-ties, making these tools useful in the presurgical period.Hurwitz et al22 justify the use of three-dimensional (3-D)CT among all available imaging studies for the planningof surgical procedures to assure the best possible recon-struction. This imaging technique can offer an accuraterepresentation of tissue deficits and asymmetry, therebyhelping clinicians make decisions regarding the transpo-sition of muscular flaps and reconstructive techniques.

In the current retrospective study, 28 female patientswere evaluated over a 17-year period. Male patientswere excluded because of differences in mammary glanddevelopment and to facilitate a standard classificationand treatment algorithm. Patients had different levels ofthoracic and superior limb compromise.

Based on the analysis of the imaging examinationsand a clinical evaluation, the cases were divided intogroups based on a new classification system. This pro-posed clinical and radiographic PS (CRPS) classificationwas designed to facilitate an accurate and practical studyof female patients and to assist in choosing the best sur-gical approach. An algorithm for the planning of surgicaltreatment is also presented.

METHODSData were obtained from the 28 female patients with PSwho were treated in the 28th Infirmary, Plastic andReconstructive Surgery Division of the Hospital SantaCasa da Misericórdia do Rio de Janeiro. Imaging studieswere performed by the Radiology Department of theUniversidade Federal do Rio de Janeiro between February1992 and January 2009. For each patient, we determinedthe degree of severity and absence or presence of congen-ital anomalies (Table 1). Only female patients wereincluded to standardize the thoracic findings in mammary

Table 1. Clinical and radiographic Poland syndrome classification

Degree of Mammary Musculoskeletal Ipsilateral superior Otherpresentation alterations chest alterations limb alterations congenital alterations

First (mild) Amastia; hypomastia or None, or partial absence of None May be presentareolar asymmetry pectoralis major muscle

Second (severe) Hypomastia or amastia; Total absence of pectoralis No or small alterations May be presentareolar asymmetry major muscle; different alterations

of the muscles and/or bones of the ipsilateral chest

Third (very severe) Amastia; areolar asymmetry Different manifestations Present May be present

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gland development, which is a variable of the CRPS sys-tem. The degrees of severity are listed below.First Degree (Mild). The diagnosis of first-degree PSwould be made in a patient with mammary asymme-try caused by hypomastia or amastia and areolarasymmetry, with or without a partial absence of thePMM. No other musculoskeletal alterations areobserved; other congenital alterations may or may notbe present.Second Degree (Severe). Hypomastia or amastia, areolarasymmetry, total absence of the PMM, and alterations ofthe ipsilateral muscle group and/or bones of the chestresults in a diagnosis of second-degree PS; ipsilateralsuperior limb alteration and other congenital alterationsmay or may not be present.Third Degree (Very Severe). Third-degree PS would bediagnosed in patients with amastia; areolar asymmetry;

major ipsilateral musculoskeletal chest alterations, suchas total absence of the PMM, the pectoralis minor mus-cle, and/or the serratus anterior muscle; possible lungherniation; widened opening of the mediastinum; andipsilateral superior limb alteration. Other congenitalalterations may or may not be present.

Imaging Studying EvaluationAll patients underwent chest radiographs and conven-tional thoracic CT scans; MRI was performed forpatients 14 through 28 once this imaging modalitybecame available in Rio de Janeiro. Three-dimensionalreconstructions of the shape and volume of the cuta-neous tissue, bones, and internal organs were con-ducted for patients 2, 3, 6, and 18. In addition, ahelical thoracic CT was performed in patients 1, 8, 10,15, 16, and 17 (Figure 1).

C D

A B

Figure 1. A, Roentgenographic study of a 32-year-old woman with deformities in the third to fifth ribs, mediastinal right deviation, and absence ofthe left breast silhouette. B, Axial computed tomographic images with contrast from a 10-year-old female, showing asymmetry in the chest silhou-ette in the frontal view, absence of the pectoralis major muscle, and hypoplasia of the pectoralis minor muscle. C, Magnetic resonance imagingscan with 3-D reconstruction shows chest asymmetry, right-sided thoracic deformities, mammary hypoplasia, absence of the pectoralis major mus-cle, sternum rotation, and mediastinal widening. D, Magnetic resonance imaging scan with 3-D reconstruction showing the same patient in part Cafter her first-stage procedure with subglandular placement of a tissue expander (round, 400 cc).

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Surgical TechniqueAn algorithm for the planning of the surgical treat-ment for all 28 patients was created from the pro-posed CRPS classification (Table 2). A total of 55procedures were planned for surgical correction ofaesthetic and/or functional abnormalities. Such cor-rections included the placement of breast implants,

creation of customized breast implants, use of tissueexpanders, and performance of local flap as well aslatissimus dorsi flap surgery, the Ravitch procedure,and contralateral symmetrization procedures such asmammary reductions or augmentations. All patientswith second- and third-degree PS were referred to tho-racic and orthopedic specialists for evaluation before

Table 2. Suggested surgical approach for each degree of presentation in the clinical and radiographic Poland syndromeclassification

Degree of presentation Suggested surgical approach

First Breast implant; contralateral mammary reduction or augmentation (symmetrization procedure when needed)

Second Tissue expander; regional local flap; breast implants or customized breast implant; symmetrization procedure

Third Tissue expander; customized breast implant when needed; breast implants; latissimus dorsi flap or other flap, such as a TRAM flap or free flap; symmetrization procedure

TRAM, transverse rectus abdominis myocutaneous.

Table 3. Clinical and imaging deformities in 28 patients with Poland syndrome

Patient no.

1 2 3 4 5 6 7 8 9 10 11 12 13 14

Age at evaluation, years 13 16 12 15 22 37 18 21 19 53 24 30 17 13

Affected side L R R R L L R R R L R R R R

Mammary hypoplasia – + + + – + + – – + + + + +

Mammary agenesis + – – – + – – + + – – – – –

Partial absence of PMM + + +* + + + + + +* + + + + +

Pectoralis minor absence + – + + + – + + + + – – – +

Serratus anterior absence – – + – + – – – + – – – – –

Nipple–areolar complex asymmetry + + + + + + + – + + + + + +

Thoracic skeletal alterations – – + – + – – + + + + + – +

Wide opening of the mediastinum – – + – – – – – – – – – – –

Ipsilateral superior limb alterations – – + – + – – – + – – – – –

Patient no.

15 16 17 18 19 20 21 22 23 24 25 26 27 28

Age at evaluation, years 21 19 41 23 23 29 25 27 23 19 17 32 13 20

Affected side L L R R R R R R R R L R R L

Mammary hypoplasia + – + – + + + + + + + + NA NA

Mammary agenesis – + – + – – – – – – – + + +

Partial absence of PMM + + + +* + + + + + – – +* + +

Pectoralis minor absence – – – + + – – – – – – + + +

Serratus anterior absence – – – + – – – – – – – + – +

Nipple–areolar complex asymmetry + + + + + – + + + + + + + +

Thoracic skeletal alterations + – + + + + – + – – – + + +

Wide opening of the mediastinum – – – + – – – – – – – + + +

Ipsilateral superior limb alterations – – – + – – – – – – + + + –

+, present; –, absent; L, left; NA, not available; PMM, pectoralis major muscle; R, right.*These patients had a total absence of the PMM; the other patients in these rows with the “+” sign had only a partial absence of the PMM.None of the patients in the study had “other systemic alterations” or “family history of alterations”, two other categories used in the clinical and radiographicPoland syndrome classification.

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undergoing surgery. Different surgical techniques wereused according to the degree of PS as shown below.(1) First degree (mild)—Breast implantation or cus-

tomized breast implantation and contralateral mam-mary reduction or augmentation when needed(symmetrization procedure).

(2) Second degree (severe)—Tissue expander placementwhen needed; regional local flap surgery; breastimplantation or customized breast implantation;symmetrization procedure.

(3) Third degree (very severe)—Tissue expander place-ment; latissimus dorsi flap or other flap surgery, suchas a free flap or transverse rectus abdominis myocu-taneous (TRAM) flap; breast implantation or cus-tomized breast implantation as needed; othersurgeries such as the Ravitch procedure and a con-tralateral symmetrization procedure.

RESULTSTwenty-eight female patients between 12 and 53 yearsof age presented with PS of different degrees of sever-ity, with or without other congenital anomalies. Thedata for each patient contained a synopsis of theanomalies, which included demographic information,family history, concomitant morphologic or otheralterations, and the information obtained from theimaging studies (Table 3).

Ten patients had first-degree PS, 14 patients had sec-ond-degree PS, and four patients had third-degree PS.Table 4 shows the CRPS level of classification for eachcase and the distribution of these classifications. Twentypatients had their right side affected; eight were affectedon their left side.

All surgical corrections were based upon the severityof disease according to the radiographic images. Tenpatients had not yet undergone surgery at the time thatthis report was written: five were undergoing preopera-tive screening and five were too young for surgical reso-lution because their growth was not yet complete.Eighteen patients underwent thoracic surgical proce-dures for reconstruction; the types of procedures aresummarized in Table 5.

Eight patients with first-degree PS underwent surgicalcorrections, with seven receiving breast implants placed ina submuscular plane and one receiving a customized breastimplant placed similarly; three of them required reductionof the contralateral breast for symmetrization (Figure 2).

Among the seven second-degree PS patients whounderwent surgery, three received tissue expanders as

an initial approach to treatment. After implantation,the volume of the prosthesis was expanded eachweek under general anesthesia until the maximalexpansion was achieved. This process required 90postoperative days. Two of these patients underwenta second procedure involving transposition of a localflap with fixation to the sternal area and breastimplant inclusion. The other patient who underwenttissue expansion had a breast implant placed and alsounderwent surgery of the contra lateral breast toachieve symmetrization.

Of the remaining four patients, two underwent localflap transposition and breast implantation, with one alsoundergoing reduction of the contralateral breast. The othertwo patients were treated only with breast implantationfor temporal symmetrization until gland development wascomplete before the final treatment (Figures 3 and 4).

Three patients with third-degree PS underwent surgi-cal treatment, receiving tissue expanders as an initialapproach with the same protocol used in the treatmentof patients with second-degree PS. After tissue expan-sion was complete, each patient underwent a transposi-tion of the major dorsal flap with fixation to the sternalarea; two received breast implants and one had a cus-tomized implant. Symmetrization of the contralateralbreast was performed in two patients (Figure 5).

The symmetrization procedures were requiredbecause the aesthetic result was a critical factor in theseprocedures. All told, seven patients underwent contralat-eral breast reductions; four of them presented with con-tralateral mammary hypertrophy.

The chosen surgical procedure and the scheduling of theintervention depended on specific conditions for eachpatient. The final decisions were made with the aim of cor-recting both the functional and/or aesthetic deformities.

DISCUSSIONFrom the time that Poland described PS in 1841 until1990, only 400 cases of this syndrome were reported.1

Published studies included the series of seven casesby Mace et al,23 a case report by Samuels et al5 focus-ing on imaging studies, a study by Merlob et al15 ofreal-time echo-Doppler duplex scanner evaluation infour cases, the surgical reconstruction of severe casesin four patients by Haller et al,36 and a long-term fol-low-up of two cases with early correction of the tho-

Table 4. Frequency of clinical and radiographic Poland syn-drome classification for 28 cases, by degree ofpresentation

Degree of presentation Frequency of cases

First 10

Second 14

Third 4

Table 5. Surgical procedures performed in 18 femalepatients with Poland syndrome

Total no. of patients surgically treated 18

Mammary prosthesis 16

Tissue expander 6

Local flaps 3

Latissimus dorsi flap 4

Contralateral mammary reduction, symmetrization procedure 8

Customized implants 2

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racic deformity using latissimus dorsi muscle flaps byAnderl and Kerschbaumer.37 Other reports haveincluded congenital studies of a single patient, likethe one by Miller and Miller,38 and associations of thedisease with non-Hodgkin lymphoma, in three casesby Sackey et al.39

Although PS is a relatively rare disease accordingto the literature, we were able to present a large num-

ber of definitively diagnosed cases in 28 carefully-evaluated female patients. This number of patientswould be equivalent to 7% of the 400 cases reportedbefore 1990 and would also represent a significantproportion of the cases described in other publica-tions since that time.

Beals and Crawford,6 Sugiura,7 Padua-Gabriel etal,8 Alembik and Stoll,9 Freire-Maia et al,10 and Taybi

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E

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F

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Figure 2. A, C, E, Preoperative views of a 52-year-old woman with mild, first-degree Poland syndrome showing breast and nipple–areolar complexasymmetry with partial absence of the pectoralis major muscle and a contralateral macromasty. B, D, F, Six months after placement of customized,croissant-shaped, 100-cc submuscular implant and symmetrization of the contralateral breast with an inverted-T technique.

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and Lachman11 all reported a higher incidence inmales than in females. However, Samuel et al15 assert-ed that this condition occurs approximately equally inmales and females, and equally on the right and leftsides. From the publications of Perez Aznar et al,34

Parano et al,40 Azzolini et al,41 Sackey et al,39 Shahamet al,42 and Armendares,43 we found that most pub-

lished case reports describe female patients. We choseto review only female patients in our study so that wecould standardize the classification system withregard to breast anatomy. Only two male patientswere identified and excluded from this study. Futurestudies are necessary to apply a similar classificationsystem to male patients.

Figure 3. A, C, E, Preoperative views of a 13-year-old female with severe, second-degree Poland syndrome affecting the right side. Because shewas still at a developmental age, a two-stage surgery was planned. Note the asymmetry of the right hemithorax, the medial rotation of the third tosixth ribs, the absence of the pectoralis major muscle, and right hypomastia. B, D, F, One year after the first stage of reconstructive surgery basedon the clinical and radiographic Poland syndrome classification and placement of a 200-cc silicone prosthesis. Her second-stage breast reconstruc-tion with latissimus dorsi muscle flap is currently on hold until her breast development is completed.

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Hurwitz et al22 noted that PS is characterized by dif-ferent degrees of mammary hypoplasia and alterations tothe subcutaneous tissue and adjacent muscles, exceptthe skin and pleural membrane. Patients with such dis-ease have a risk of lung herniation during respiration.31

Syndactyly may also be present.22 All of these compo-nents are not necessarily present, but the full syndromeconsists of an assortment of abnormalities.14

In this series, the general characteristics of PS wereobserved in all 28 female patients. The patients haddifferent degrees of involvement of the chest wall andsuperior limbs; many had congenital abnormalitiesassociated with PS. The CRPS classification organizedthese findings into the three degrees of severitydescribed above.

The CRPS classification seems to help in the evalua-tion needed before beginning any intervention for PS.This classification defines the disease in detail; eachdegree represents specific deformities defining the maintissues requiring reconstruction, which facilitates thepractical and effective study of its pathology in femalepatients. The classification also allows for a correlationof the clinical manifestations with the imaging tests,making it useful for surgeons in deciding on the bestreconstructive procedure. In our clinical experience, it isa clear and easy system that even inexperienced sur-geons in plastic surgery departments could use. Withoutthe imaging studies, an accurate evaluation of the dis-ease would not be possible.

Perez Aznar et al34 showed how mild forms of PS aremore frequent than severe forms and may go undiag-nosed. Hypoplasia of one breast or a horizontal anterioraxillary fold may be the sole clinical manifestation ofthis syndrome.44 In our cohort, we observed the severetype (second degree) more frequently than the othertypes, as it constituted 14 cases. However, most of the 10patients with mild disease were referred to a specialistfor aesthetic reasons.

Martin and Emory45 were the first to report macro-masty associated with PS. A patient with grade III/IVmammary hypertrophy was studied using anamnesisand preoperative physical and imaging examinations. Inthis case, the surgical treatment confirmed the absenceof the PMM.45 In our cohort, four of the patients whounderwent surgery presented with contralateral mammaryhypertrophy and required surgical intervention toachieve symmetrization of the breasts.

Radiographic evaluation of the most significantdeformities usually includes a chest radiograph,stereophotogrammetry, and CT and MRI scans, the lat-ter two of which can be used to define the chest walldeformity more accurately once it has been diag-nosed.46 The radiologic imaging techniques have afundamental role in the detection, localization, andcharacterization of thoracic alterations.

Even though the chest radiograph has been the basicimaging study for PS, CT may be required to reveal thetotal absence of the PMM.1 In addition, MRI has made

the study of the syndrome much easier because of itshigh resolution and capacity to generate multiplanarimages.35 Conventional CT can allow the differentiationof PS from other entities and may facilitate the recogni-tion of various radiologic findings, improve the accuracyof diagnosis, and help optimize treatment.32 The imagesseen on the helical CT scan allow for an easier approachto the disease, even for the nonspecialist members of thereconstruction team. For these and other reasons, thisstudy included all of the available imaging examinations.MRI was indicated for the prospective part of this study;before it became available, radiography and CT scanwere the only imaging modalities that could providesuch data. Other imaging examinations, including helicalCT scan of the thorax and 3-D reconstructions, were per-formed in severe cases.

The treatment of this condition is strictly surgi-cal.22–29,31–33,44,47 The indication for surgery and thetime of intervention depend on the severity of themalformations. Fonkalsrud48 reported that if surgicalrepair was performed at an early age, there was a highrecurrence rate because of later periods of rapid bonegrowth. In this study, a cohort of teenagers was stud-ied for surgical treatment; because their developmentwas not complete, temporary interventions (such asthe use of tissue expanders) were performed. The ear-liest age for first stage treatment was 15 years. Allsevere and highly severe cases were referred to tho-racic and orthopedic specialists before any treatmentwas performed; the opinion of the specialist made thesurgical choice easier.

CONCLUSIONSThe literature indicates that radiologic diagnosis of tho-racic cage alterations is indispensable for planning thetreatment and defining a precise surgical approach forPS. Based on the analysis of the imaging studies andclinical evaluation of 28 cases using the CRPS classifica-tion, an accurate study was possible for each case,adding valuable information that could be used in decid-ing upon the surgical approach. The classification alsoincludes a treatment algorithm for each level of disease.The surgical procedure chosen and the time of interven-tion depended on the details of each case and the finaldecisions were made with the aim of correcting the func-tional and/or aesthetic alterations.

During the study, the researchers found that their pro-posed system of classification was a practical and usefultool that facilitated the choice of a surgical approach.This study only included female patients; furtherresearch is required to develop and evaluate a similarclassification system for PS in males. ◗

DISCLOSURES

The authors have no disclosures with respect to the contents ofthis article.

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A B

C D

Figure 4. A, C, E, Preoperative views of a 20-year-old woman with severe, second-degree Poland syndrome affecting the left side. Note theabsence of the pectoralis major muscle, amastia, and nipple–areolar complex asymmetry. B, D, F, Sixty days after placement of the implant, show-ing good volume, breast symmetry, and filling of the entire hemithorax.

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REFERENCES1. Cooper RA, Johnson MS. Mammographic depiction of Poland’s syn-

drome. Br J Radiol 1990;63:302–303.2. Bainbridge LC, Wright AR, Kanthan R. Computed tomography in the

preoperative assessment of Poland’s syndrome. Br J Plast Surg1991;44:604–607.

3. Mentzel HJ, Seidel J, Sauner D, et al. Radiological aspects of thePoland syndrome and implications for treatment: a case study andreview. Eur J Pediatr 2002;161:455–459.

4. Poland A. Deficiency of the pectoralis muscle. Guy’s Hospital Reports1841;6:191–193.

5. Samuels TH, Haider MA, Kirkbride P. Poland’s syndrome: a mammo-graphic presentation. AJR Am J Roentgenol 1996;166:347–348.

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syndrome. A report of 2 cases. J Hand Surg Br 1994;19:659–661.

A

C

B

D

Figure 5. A, C, Preoperative views of a 13-year-old woman with very severe, third-degree Poland syndrome affecting the right side. Note thesevere deformity, breast and nipple–areolar complex asymmetry, total absence of the pectoralis major and minor muscles, sternum rotation,absence of the axillary fold, and osteoarticular deformity in the thoracic cage. B, D, Postoperative views of the same patient in parts A and C at age28, after tissue expander placement (round, 400 cc), later replaced with a round, high-profile 260-cc cohesive silicone gel–filled implant using alatissimus dorsi muscle flap for coverage. An areola graft was used from the left areola. Contralateral reduction with an inverted-T incision wasperformed in order to achieve symmetry.

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20. Smith DW. Recognizable patterns of human malformation: genetic,embryologic, and clinical aspects. Major problems in clinical pediatrics,vol 7. Philadelphia: WB Saunders, 1982.

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Accepted for publication April 21, 2009.

Reprint requests: Ricardo Cavalcanti Ribeiro, MD, Plastic and ReconstructiveSurgery Division, Hospital Santa Casa da Misericórdia do RJ, Clinica Vitee,Av. Fernando Matto, Barra da Tijuca, Rio de Janeiro, RJCEP 22621-090,Brazil. E-mail: [email protected].

Copyright © 2009 by The American Society for Aesthetic Plastic Surgery, Inc.

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doi:10.1016/j.asj.2009.09.015