Abstract
Poland syndrome, tuberous breast deformity, gynecomastia, and macromastia are among the most common breast disorders affecting adolescents. While the etiology and physical manifestations may differ between each condition, they all may impose psychosocial stress necessitating evaluation and treatment. A multidisciplinary team is essential to providing comprehensive care specific to an adolescent population. The purpose of this chapter is to discuss common adolescent breast deformities necessitating plastic surgeon involvement, and the components of a successful multidisciplinary adolescent breast care team.
Keywords: adolescent, breast, multidisciplinary care, plastic surgery, macromastia, Poland syndrome, tuberous breast deformity, gynecomastia
Adolescents with congenital and acquired breast deformities require more complicated treatment algorithms compared with the adult population due to the complex interaction of psychosocial, sexual, and growth characteristics. A multidisciplinary team (MDT) is integral to providing comprehensive perioperative care in this unique population. The objectives of this chapter are (1) to discuss the multidisciplinary adolescent breast care team members and their roles, (2) to highlight the most commonly encountered breast conditions including Poland syndrome (PS), tuberous breast deformity, gynecomastia, and macromastia, and (3) to present our institution's multidisciplinary experience.
Multidisciplinary Team Members
The MDT for adolescent breast conditions may consist of plastic surgeons, pediatric surgeons, endocrinologists, psychologists, pediatricians, physical therapists, dieticians, and patient care coordinator/social workers. A subset of core providers is often needed for all patients. In our institution, we have found many benefits from shared medical appointments (SMAs) involving a single clinic visit with the most common providers involved in these patients' care ( Fig. 1 ). 1 Patients may require referral to other specialties within the MDT on a case-by-case basis.
Fig. 1.

Standard workflow for Multidisciplinary Breast Condition Clinic.
Pediatrician
Pediatricians are often the initial providers who recognize breast abnormalities, namely, gynecomastia, macromastia, or breast lumps/masses. 2 Thus, it is important for pediatricians within the community to have familiarity in diagnosing these conditions and with the referral process for the Multidisciplinary Breast Condition Clinic.
Obstetrician–Gynecologist
While obstetrician–gynecologists (OB-GYNs) are traditionally not members of multidisciplinary breast care teams, a bidirectional relationship exists between OB-GYNs and plastic/breast surgeons. A positive association has been shown to exist between gynecologic complaints and severe macromastia. 3 The American College of Obstetricians and Gynecologists has set standards in place for practicing OB-GYNs to address these concerns including knowledge of nonsurgical suggestions for breast comfort and appearance as well as an understanding of the options for surgical intervention to treat the condition and, if necessary, referral to a multidisciplinary breast clinic. 4
Plastic Surgery
Plastic surgeons comprise the core of the MDT and should be comfortable evaluating and treating a host of adolescent breast conditions such as PS, tuberous breast deformity, gynecomastia, and macromastia. 5 Their expertise helps provide patients and their families with the variety of treatment options available, fostering patient-centered care. Other members of the MDT provide crucial support for evaluating and optimizing surgical candidacy and success, and with their recommendations, the plastic surgeon will be well-equipped when deciding upon whom to operate.
Pediatric Surgery
Depending on the institution, pediatric surgeons may be equipped to treat gynecomastia or may be the frontline for evaluation of a breast condition, ultimately guiding the patient to a Multidisciplinary Breast Condition Clinic for definitive treatment. 6 Pediatric surgeons often also treat patients with chest wall abnormalities such as pectus carinatum or excavatum or skeletal anomalies associated with PS.
Endocrinologist
Endocrine system dysfunction may affect breast development in both adolescent males and females, thus, certain conditions (especially gynecomastia) may benefit from an endocrinology consultation. Additionally, endocrine complaints were shown to be an independent risk factor for severe macromastia. 3 The endocrinologist should have an understanding of the appropriate referral pattern to a multidisciplinary breast clinic, and conversely, the plastic surgeon should have a relationship with a knowledgeable endocrinologist.
Psychologists
A strong association exists between adolescent patients with breast anomalies and subsequent psychosocial manifestations. Patients with macromastia can have a lower quality of life and self-esteem compared with their peers and are more likely to develop concurrent eating disorders, such as bulimia nervosa, in an effort to reduce breast size. 7 8 A similar psychological burden also affects males with gynecomastia as studies report significantly higher rates of anxiety, depression, and social phobia among adolescent males with gynecomastia. 9 Psychologists can help recognize the presence of psychological dysfunction before or after surgery and provide counseling and support to improve self-image and manage social and emotional challenges.
Physical Therapist
The physical therapist may provide regimens for back and neck pain relief in those hoping to delay surgery for macromastia, although results show temporary or no benefit. 10 Still, some insurers require that patients seeking a breast reduction for macromastia first undergo evaluation and treatment from a physical therapist prior to authorizing surgical intervention. 10 Physical therapists are also especially important in providing an individualized exercise regimen for those with concurrent obesity, enabling obese patients to lower their surgical complication risk.
Clinic Coordinator
Clinic coordinators (or care coordinators) are responsible for directing care among various specialties, serving both as a patient advocate and as the primary point of contact. 11 Furthermore, Clinic Coordinators have been shown to lower costs, decrease hospital stays, and improve patient satisfaction. 12
Social Workers
Social workers connect the patient with outside resources, help with financial and insurance issues, and assist with the complex family dynamics between family members or guardians.
Dieticians
Dieticians optimize caloric and nutritional intake, as obesity may cause or exacerbate the patient's breast disorder. For males, a strong link was found between gynecomastia and obesity as over 60% of patients with gynecomastia are either overweight or obese. 13 In females, obesity was similarly found to be the strongest risk factor in adolescent macromastia. 3 With proper weight management and nutrition, severe breast hypertrophy can be improved and although most patients may still require surgical correction of excess tissue and/or skin, a body mass index (BMI) close to normal is associated with a lower risk for complications and a subjectively better cosmetic outcome. 14 15 16
Our Experience with the Multidisciplinary Breast Clinic
Our multidisciplinary breast condition clinic is comprised of plastic surgeons, physical therapists, dieticians, psychologists, and social workers. Our clinic coordinator ensures the clear and continuous flow of providers, arranges referrals, and helps to maintain continuity as a primary point of contact for the patients ( Fig. 1 ). Since 2016, patient–physician visits transpire in a group setting on specific days of the month. 1 These SMAs have generated high provider and patient satisfaction, as they decrease the complexity of seeing providers in separate clinics and on separate days and even allow groups of patients to empathize, learn from, and support one another. 1 All patients presenting for an initial consultation will be seen by the plastic surgeon. In order to maximize efficiency, patients are prescreened by chief complaint to determine which providers they will see, and patients may see providers in a different order to minimize provider bottlenecks. Those who present with macromastia will be screened for pain (neck pain, back pain, shoulder grooving), stabilization of breast size, BMI, exercise tolerance, psychosocial burden, other additional symptoms, and will be seen by a physical therapist. Patients are also evaluated by a psychologist to determine their psychological state and surgical candidacy and to prepare them for the significant change in body appearance and shape associated with treatment.
Common Adolescent Breast Conditions
Poland Syndrome
PS may manifest as a combination of chest and upper extremity abnormalities including hypoplasia of mammary tissue and the nipple–areola complex (NAC) ( Fig. 2 ), hypoplasia or absence of the pectoralis muscles ( Fig. 3 ), chest wall deformities of the ribs, shortening of the ulna/radius, and other hand anomalies. 17 Pediatricians or gynecologists are often the first providers to recognize early signs of PS and should make referrals to the multidisciplinary breast condition clinic and hand surgeons, as indicated. Physical therapists also are necessary in evaluating and managing patients with PS due to their muscular hypoplasia and possible posturing difficulties or concomitant scoliosis. Patients with skeletal chest wall abnormalities associated with PS should be evaluated and treated by an appropriate surgeon (often pediatric surgery).
Fig. 2.

A 16-year-old female patient with right-sided Poland syndrome and hypoplasia of her right breast. Right anterior axillary fold and pectoralis is present in this patient. Reproduced with permission from Texas Children's Hospital.
Fig. 3.

A 17-year-old male with right-sided Poland syndrome, with complete absence of the right pectoralis major muscle. Reproduced with permission from Texas Children's Hospital.
Definitive surgical intervention is often deferred until the patient reaches stable breast size which typically occurs at 16 years of age or older. We (and others) advocate for earlier intervention with placement of a tissue expander and periodic expansion as the patient's peers begin breast development, decreasing the stigma of PS and improving psychological outcomes. 5
Goals of surgical management for PS in females most importantly include recreation of the breast mound, and secondarily, creation of an anterior axillary fold. This is often accomplished in two stages through the placement of a tissue expander in preparation for a future exchange with a breast implant. Despite Food and Drug Administration approval limitations for silicone or saline implants for the purpose of augmentation (ages 22 and 18 respectively), in this unique population, either silicone or saline implants may be used at any age for the purpose of reconstruction. 18 For all individuals, appropriate informed consent must be obtained regarding the risks of infection, extrusion, rupture, capsular contracture, as well as the evolving understanding of the risk of breast implant-associated anaplastic large cell lymphoma and breast implant illness. Fat grafting may be useful in those with a less severe morphology of PS in an effort to avoid implant use 19 ; however, this may require multiple stages and may be limited by adequate donor sites. Whether implant-based or autologous fat grafting reconstruction is performed, in cases of asymmetry, contralateral reduction or mastopexy may be warranted and can be performed simultaneously with implant exchange or in a separate stage. Although not crucial to all patients, a pedicled latissimus dorsi muscle flap may be utilized in those with hypoplastic or absent pectoralis major muscle to recreate the anterior axillary fold. The muscle is transpositioned to the chest, the tendon released and repositioned near the bicipital groove, and a tissue expander or implant may be placed submuscularly in a single stage. Sacrifice of the latissimus may cause some impaired function and adds a significant scar (unless performed via a transaxillary incision and endoscope-assisted approach). 20 21
Male patients with PS also may benefit from reconstruction, with the goals of restoring symmetrical pectoralis bulk and the anterior axillary fold. Often solid structure off-the-shelf or customized silicone implants are used, and a latissimus may be transferred if desired. In our experience, male patients with PS also substantially benefit from surgical correction of chest asymmetry, mitigating psychosocial distress; however, insurance companies may determine that the procedure is an elective cosmetic operation, necessitating patient self-pay.
Tuberous Breast Deformity
Tuberous breast deformity stigmata include constricted base width, raised inframammary fold, and herniation of the breast parenchyma toward the NAC with areola enlargement. 22 This condition can be unilateral or bilateral. A constricting fibrous ring, thought to be originated from abnormal superficial fascia, can be found at the level of the periphery of the NAC. 22 This ring inhibits the normal development of the breast and prevents expansion of the breast during puberty. Principles of surgical management for tuberous breast anomalies include release of the radial constricting band, lowering of the inframammary fold, expansion and augmentation of the breast volume, and nipple areolar reduction. 22 The first stage generally involves radial scoring and tissue expander placement through a periareolar or inframammary incision. Implant exchange is later performed. A periareolar mastopexy is often necessary to correct the large size of the NAC. Surgical intervention is generally performed after cessation of breast growth.
Gynecomastia
Gynecomastia is abnormal hypertrophy of breast tissue in males and manifests as persistent firm tissue beneath the NAC ( Figs. 4 and 5 ). This condition affects up to 65% of pubertal males due to hormonal shifts, but usually spontaneously resolves within a year, with over 90% of patients' pubertal gynecomastia resolving within 3 years. 23 There are numerous secondary causes of gynecomastia including but not limited to Klinefelter's, testicular cancer, and pharmacologic causes. Therefore, it is important to differentiate between physiologic and pathologic gynecomastia.
Fig. 4.

A 16-year-old male with right-sided Simon Grade IIA gynecomastia. Reproduced with permission from Texas Children's Hospital.
Fig. 5.

A 16-year-old male with bilateral Simon Grade III gynecomastia with a large component of lipomastia. Reproduced with permission from Texas Children's Hospital.
A detailed endocrinologic, medication, and substance use history should be conducted. Substances that can induce gynecomastia include anabolic steroids, alcohol, herbal supplements, and marijuana. Further assessment includes genital and testicular examination, Tanner staging, and physical examination of breast tissue to differentiate between lipomastia (which also known as adipomastia or pseudogynecomastia) and true gynecomastia, as well as to rule out any breast masses. 23 24 Identifying gynecomastia secondary to substance use can prevent unnecessary workup and subsequent strain on the patient and the health care system. A trial of discontinuation of offending substances must be completed prior to surgical intervention. One retrospective review found that an endocrine workup was administered in 87% of patients presenting with gynecomastia, but only 1.7% of patients had positive findings and over two-thirds of cases were found to be drug-induced. 25 However, referral to endocrinology may be necessary if galactorrhea is present, the testicular exam is abnormal, or if the gynecomastia is rapidly progressing. 25 Endocrinologic studies are usually indicated, namely, thyroid hormone levels and function tests, dehydroepiandrosterone sulfate, testosterone, estradiol, human chorionic gonadotropin, follicle-stimulating hormone, and luteinizing hormone levels. 17 Additionally, if feminizing characteristics are present, evaluation for Klinefelter's syndrome via karyotype is indicated.
Since most cases of gynecomastia will self-resolve, treatment typically consists of reassurance or avoidance of the causative agent. However, surgery is indicated when there is an absence of secondary causes, persistence of the breast enlargement for over 1 year, and/or severe social distress. 26 Surgical management of gynecomastia typically depends on the severity of the gynecomastia most often classified by the Simon system 27 :
Grade I: Minor breast enlargement without skin redundancy.
Grade IIa: Moderate breast enlargement without skin redundancy ( Fig. 4 ).
Grade IIb: Moderate breast enlargement with skin redundancy.
Grade III: Marked breast enlargement with significant skin redundancy ( Fig. 5 ).
For grade I/II, a combination of liposuction and glandular tissue resection through a periareolar incision is associated with minimal complications and excellent patient satisfaction. 28 For grade IIb cases where the skin has poor elasticity, resection may be performed while maintaining the NAC on a dermal pedicle, avoiding the complications of free nipple grafting. For grade III gynecomastia, subcutaneous mastectomy and free nipple grafting are necessary to remove a large amount of excess skin and correct NAC ptosis. 29 Of note, combined techniques consisting of surgical resection followed by aspiration demonstrated the lowest complication rates overall (when considering hematoma, seroma, over/underresection, dehiscence, infection, pathologic scar, irregularities/redundant skin, NAC necrosis [partial or total]/abrasion, and revision/recurrence). 30 Overall, gynecomastia surgery in adolescents is effective with a majority of patients satisfied with the outcomes and reporting increased self-esteem and quality of life, postoperatively. 26 31
Despite the clear advantages to surgical intervention, many insurers do not see gynecomastia surgery as a covered benefit and patients are often forced to pay out of pocket. Providers must frequently advocate for their patients through peer-to-peer discussions and appeals. It is the authors' hope that this condition will eventually be universally recognized by insurers for the morbidity it causes young men and approve surgical intervention for these patients.
Macromastia
Adolescent macromastia involves symptomatic, usually bilateral, enlargement of the breasts ( Fig. 6 ). Although often idiopathic, some identifiable etiologies exist for this condition, including hormonal abnormalities and obesity. 32
Fig. 6.

A 18-year-old female with bilateral symptomatic macromastia, grade 3 ptosis, and shoulder grooving. Reproduced with permission from Texas Children's Hospital.
Tumors must be ruled out through a thorough history and physical examination, and advanced imaging acquired for any concerning findings. A specific subset of these patients suffer from juvenile breast hypertrophy in which very rapid, disfiguring, and continued breast growth may occur before and during puberty. This may be caused by an increase in responsiveness to estrogen, triggering the fibrous and fatty components of the breast to increase while the glandular tissue remains unchanged. 5
Macromastia causes significant postural problems and neck and back pain. It can make it difficult to perform certain physical activities, participate in sports, and find clothing, which fits correctly. Obesity may be a confounding factor contributing significantly to the presence of macromastia. Even with conservative management including diet, exercise, weight loss, and physical therapy, significant skin and breast tissue may be present, leading to persistence of symptoms. Surgical intervention is the only solution to the presence of this excess skin and breast tissue.
Reduction mammaplasty surgery necessitates preservation of the NAC on a vascularized pedicle, with the goal of maintaining the capacity for lactation and nipple sensation. Informed consent is not complete without a thorough discussion of the possible loss of one or both of these functions and even loss of the NAC or need to convert to a free nipple graft. 33 A review of over 150 patients with macromastia who underwent reduction mammoplasty found no significant differences in lactational success rates between superior, medial, and inferior pedicles. When evaluating preservation of nipple sensation postoperatively, one meta-analysis of over 2,600 patients found that the superomedial pedicle was associated with higher rates of diminished nipple/areolar sensation when compared with the inferior pedicle approach. 34 However, benefits of the superomedial pedicle approach include shorter operative times and enhanced aesthetic outcomes. The technique implemented may depend on institutional or surgeon preference, as depicted by a retrospective review of two different quaternary children's hospitals, in which over 90% of adolescent reduction mammoplasties were done using an inferior pedicle at one institution while a medial pedicle was used in over 90% of cases at the other institution. 33 35
In general, surgical candidates are patients who have stable weight for at least 3 months, as weight gain or weight loss may compromise the long-term success of the operation and lead to the recurrence of macromastia or breast deflation. Overweight patients, with a BMI greater than 30, are encouraged to lose weight and are referred to a dietician and physical therapist. If weight loss is not successful, but patients have a BMI of 35 or less, then they may still proceed with surgery as a BMI greater than 35 has been demonstrated to double the risk of postoperative complications. 14 Additionally, patients with a BMI greater than 35 who have failed weight loss may be referred to bariatric surgery. Only in exceptional circumstances should reduction mammaplasty be considered for patients with extreme obesity.
It is not uncommon for an adolescent who undergoes breast reduction surgery to require a secondary reduction later in life. Losee et al showed the ability to use a different pedicle safely for secondary reduction. 36 However, a review of over 90 patients found that unmatched secondary pedicles had higher complication rates than matched secondary pedicles; thus, if the pedicle from primary surgery is known, it is likely the safest option to conduct the procedure with the same pedicle. 37
Although, ideally, patients will have stable breast size before undergoing surgery, it is the authors' opinion that continued breast growth is only a relative contraindication to surgery. Adolescent patients with severe symptomatic macromastia, even with continuing breast growth, should be considered for reduction mammaplasty if the patient understands that breast growth may continue, necessitating a secondary reduction. This should be discussed on a case-by-case basis.
A large majority of reduction mammaplasties in adolescent patients are an insurance covered benefit. Insurance companies vary in the criteria necessary for approval, namely, descriptions of three or more symptoms, attempt at conservative therapies (such as physical therapy), and, perhaps most importantly, estimated resection weight volumes based on the Schnur scale. 38 However, the Schnur scale was created based on the adult population, and its use to determine approval for adolescents may cause unnecessary denials. A trial of physical therapy and weight loss (if indicated) is usually required. As such, physical therapists will evaluate each patient with macromastia and implement an individualized regimen. 10 38 39 Complete resolution of symptoms is uncommon, and physical therapy may be most beneficial for those symptomatic patients who are hoping to postpone breast reduction until breast growth stabilization at a later age. 39 40 Stability in breast size, ranging from 6 to 12 months is also frequently required. Notably, Figueroa et al found no significant differences in insurance approval based on the specific insurance carrier; however, they observed that insurance denied coverage for a reduction mammaplasty in 40% of patients.
Conclusion
The variety of breast disorders affecting adolescents warrants a multidisciplinary approach and team for optimal treatment, with consideration of the unique clinical and nonclinical factors affecting this patient population. A holistic and detailed evaluation is needed to fully recognize the physical, psychosocial, and sexual aspects of adolescent breast disorders. A Multidisciplinary Breast Care Clinic consisting of the core providers seeing each patient at a single visit greatly improves the ability to provide holistic care, improving the patient experience. Open and frequent communication among providers, the patient, and their families allows for timely patient-centered care.
Funding Statement
Funding None.
Footnotes
Conflict of Interest None declared.
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