Management of the tuberous breast represents one of the greatest surgical challenges in aesthetic breast surgery, requiring careful assessment and a methodical approach to obtain an acceptable result. The surgeon must be familiar with multiple techniques that can be performed individually or may be combined to address various aspects of the tuberous deformity. This article describes the etiology, anatomic features, identification, and classification of the tuberous breast, focusing on surgical management and potential pitfalls. Through case study, expected outcomes in the management of this complex problem are described.
Key points
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The tuberous breast is a congenital abnormality of breast development that incorporates a constricted base of the breast and 1 or more of the following: high inframammary fold, areola hypertrophy, pseudoherniation of tissue through the areola, ptosis, hypoplasia, and breast asymmetry.
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Advanced forms of tuberous breast are readily apparent clinically; however, the diagnosis of more minor forms of tuberous breast requires careful examination and a high index of suspicion.
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The principles of treatment of the tuberous breast include:
- a.
Release of the constricted base through expansion, scoring, or internal flaps.
- b.
Lowering of the inframammary fold and restoring a normal nipple to inframammary fold distance.
- c.
Correction of herniated breast tissue.
- d.
Reduction of the size of the areola.
- e.
Augmenting the breast volume, when necessary.
- f.
Correction of underlying breast asymmetry.
- a.
Introduction
Breast anomalies characterized by an abnormality or asymmetry of the breast base have been called many names including tuberous breasts, tubular breasts, herniated nipple areolar complex (NAC), Snoopy deformity, lower pole hypoplasia, and constricted breasts. These terms all represent varying degrees of the same deformity, with a multitude of different techniques described for their correction. No matter the name, it is broadly characterized by a deficiency in the vertical and horizontal dimensions of the breast, frequent underdevelopment of the breast, asymmetry, and herniation of breast tissue into the areola accompanied by expansion of the areola. Since its first description in 1976 by Rees and Aston, this deformity, now most commonly referred to as the tuberous breast, has been the subject of several classification systems and a host of surgical management options.
It has been said that no other condition of the breast presents the same type of surgical challenge as the tuberous breast deformity. Understanding its features and implementing a methodical approach to its surgical management is paramount, as the psychological and emotional effect these deformities can have on women are significant.
Despite several large reported series, the prevalence of tuberous breast deformity is not firmly established. DeLuca-Pytell and colleagues reported a prevalence of 73% in a retrospective analysis of 375 patients presenting for mammoplasty. Zambacos and Mandrekas suggested that the actual percentage of tuberous breast is unknown but is actually much lower (6%–7%) than the one reported in the study by DeLuca-Pytell and colleagues. Although some investigators consider this to be a problem of the female breast only, several recent studies have described similar features in the male breast. Asymmetry in tuberous breast deformity is almost always present
A high index of suspicion is important in recognizing all forms of a tuberous breast. The unique anatomic features require specific surgical decisions and techniques in comparison with a standard breast augmentation. Failure to recognize this will predispose the patient to an unsatisfactory outcome and increase the likelihood of problems such as implant malposition, implant edge visibility and palpability, persistence of the old inframammary fold (IMF), and secondary soft-tissue deformities.
Introduction
Breast anomalies characterized by an abnormality or asymmetry of the breast base have been called many names including tuberous breasts, tubular breasts, herniated nipple areolar complex (NAC), Snoopy deformity, lower pole hypoplasia, and constricted breasts. These terms all represent varying degrees of the same deformity, with a multitude of different techniques described for their correction. No matter the name, it is broadly characterized by a deficiency in the vertical and horizontal dimensions of the breast, frequent underdevelopment of the breast, asymmetry, and herniation of breast tissue into the areola accompanied by expansion of the areola. Since its first description in 1976 by Rees and Aston, this deformity, now most commonly referred to as the tuberous breast, has been the subject of several classification systems and a host of surgical management options.
It has been said that no other condition of the breast presents the same type of surgical challenge as the tuberous breast deformity. Understanding its features and implementing a methodical approach to its surgical management is paramount, as the psychological and emotional effect these deformities can have on women are significant.
Despite several large reported series, the prevalence of tuberous breast deformity is not firmly established. DeLuca-Pytell and colleagues reported a prevalence of 73% in a retrospective analysis of 375 patients presenting for mammoplasty. Zambacos and Mandrekas suggested that the actual percentage of tuberous breast is unknown but is actually much lower (6%–7%) than the one reported in the study by DeLuca-Pytell and colleagues. Although some investigators consider this to be a problem of the female breast only, several recent studies have described similar features in the male breast. Asymmetry in tuberous breast deformity is almost always present
A high index of suspicion is important in recognizing all forms of a tuberous breast. The unique anatomic features require specific surgical decisions and techniques in comparison with a standard breast augmentation. Failure to recognize this will predispose the patient to an unsatisfactory outcome and increase the likelihood of problems such as implant malposition, implant edge visibility and palpability, persistence of the old inframammary fold (IMF), and secondary soft-tissue deformities.
Anatomy and histopathology
The clinical features of the tuberous breast are illustrated in Fig. 1 , and include a reduced breast base diameter, a high and constricted IMF, breast hypoplasia, ptosis, areola hypertrophy, herniation of tissue into the areola, and variable asymmetry of the breast.
The etiology of this deformity is unclear. Glaesmer (1930) suggested a phylogenetic relapse and Pers (1968) postulated that there is a failure of tissue differentiation in a limited zone of the fetal thorax. These theories were effective in explaining deformities consistent with amastia and Poland syndrome, but more recent theories point to a simpler explanation that highlights the abnormal superficial fascia or weakness of the periareolar supporting tissues in the tuberous breast.
In earlier description and classification of tuberous breast deformity, Grolleau hypothesized in 1999 that the tuberous form is the result of stronger than normal adherence between the dermis and underlying muscle in the lower quadrants of the breast, which the developing breast cannot release. This adherence restricts peripheral expansion of the breast, causing it to develop in a forward direction and giving the breast its tubular appearance. In cases where the connective and muscular structure of the areola is weak, the gland herniates into the areola. These theories have been more recently expanded with Mandrekas’ description of the ring theory and Costagliola’s discussion of the role of the weakened peri-NAC skin and fascia in predisposing to herniation of tissue into the areola.
Together, these theories describe the breast as contained within a superficial fascial envelope, continuous with Camper fascia in the abdomen. The superficial layer covers the breast parenchyma, and the deep layer lies on the pectoralis fascia and forms the posterior boundary. A constricting ring at the level of the areola caused by a thickening of the superficial fascia, the joining of the 2 fascial layers at a higher level, or a thickening of the suspensory ligaments in this area inhibits normal development of the breasts. This constriction, combined with the absence of the superficial layer of the fascial envelope under the areola, allows for preferential development of the growing breast in a vertical direction with herniation through the weakened peri-NAC skin, resulting in the tuberous shape with areolar widening.
Classification
In 1996, Von Heimberg reviewed preoperative photos of 40 patients (68 breasts) with varying degrees of the tuberous breast deformity to describe a classification of 4 types ( Fig. 2 ):
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Type I: Hypoplasia of the lower medial quadrant
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Type II: Hypoplasia of the lower medial and lateral quadrants with sufficient skin in the subareolar region
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Type III: Hypoplasia of the lower medial and lateral quadrants with skin deficiency in the subareolar region
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Type IV: Severe breast constriction with minimal breast base
Grolleau later examined 37 patients with breast base deformity, and modified the Von Heimberg classification to describe only 3 groups, as no objective or clinical difference could be seen between Von Heimberg types II and III. This study graded constricted breast base as Type I (lower medial quadrant deficiency), Type II (deficiency in both lower quadrants), and Type III (deficiency of all 4 quadrants). Of his patients, 54% had type I, 26% had type II, and 18% had type III. Seventy percent had volume asymmetry of greater than 100 g. Virtually all type III were hypoplastic and required an implant to restore adequate volume; 74% of type II and 100% of type I had sufficient volume to make an implant unnecessary. This classification also noted areolar herniation to be more frequent in type III breasts but did not include this feature in the classification.
More recently, a paradigm shift in the classification of tuberous breast deformity has been suggested based on the degree of areolar herniation. Pacifico and Kang described an objective classification using the Northwood Index (NI), a ratio between the amount of forward projection of the areola divided by the areolar diameter. Fifty-five breasts were examined (20 tuberous) from photos, and NI was found to be 0.19 in normal breasts and 0.54 in tuberous breasts (on average). Tuberous breasts were then further divided into mild, moderate, and severe based on NI. Any NI higher than 0.4 was considered tuberous.
Treatment goals and planned outcomes
Principles of Treatment
A cornerstone of any successful aesthetic procedure is having a defined process for patient education and management of expectations. This tenet holds particularly true for patients with a tuberous breast. These patients dwell on the boundary between aesthetic and reconstructive surgery. Most patients appreciate that their breasts have an unusual shape or appearance, and in many cases desire an increase in volume. Patients frequently comment that they knew something was not quite right about their breasts but just did not know what it was. Often, however, patients do not have a full appreciation of the degree of anatomic abnormality, and the difficulties that will be faced by the surgeon to restore a near normal appearance to the breast. Without proper education, many patients expect outcomes similar to those seen with routine primary breast augmentation.
Surgical management of the tuberous breast is ultimately determined by the severity of presentation, and requires both the surgeon and patient to address each aspect of the deformity. The principles of treatment should include the following:
- 1.
Release of the constricted base in both a vertical and horizontal plane
- 2.
Restore a normal nipple to IMF distance through a combination of mastopexy and lowering of the IMF
- 3.
Obliterate the old IMF to avoid a double-bubble appearance
- 4.
Reduction of herniated tissue
- 5.
Correction of areola hypertrophy
- 6.
Restoration of breast volume
- 7.
Correction of breast asymmetry
In addition, the decision must be made whether to address the multiple elements of this deformity in 1 or 2 stages. Whenever possible, correction is offered in a single stage; however, in cases of type IV deformity or severe herniation of tissues into the areola, a 2-stage approach is suggested ( Fig. 3 ).
Photographs can be helpful in the process of managing expectations. Patients should be aware that in comparison with a primary augmentation, they are more likely to have residual asymmetry, require additional scars, have implant edge visibility or palpability, and may have persistence of an old inframammary scar. The complexity of these patients will result in an increased incidence of secondary surgery compared with the population undergoing primary breast augmentation. Ensuring that patients have a full understanding and acceptance of these issues before their initial treatment is a necessary component of patient selection and preparation.
Minor degrees of tuberous deformity are often seen in women presenting with concerns of ptosis, hypertrophy, or asymmetry. A high index of suspicion is necessary to appreciate the constrictive component of the presentation.
Patients with ptosis and adequate volume can be treated using mastopexy and breast-reduction techniques. Care should be taken to adjust and customize the skin patterns of excision to account for areas of tissue deficiency. For example, in a type I deformity with medial deficiency, it is important to excise skin laterally and maintain skin in the deficient medial quadrant. Tissue excision is also guided by the type of deformity, and internal breast flaps may be helpful to redistribute tissue to areas of apparent deficiency ( Fig. 4 ).
It is also necessary to appreciate the difference between the tubular breast deformity and the tuberous breast deformity. Despite the similarity in pathogenesis, tubular and tuberous breasts are distinct deformities that present with different clinical features. The tuberous breast is characterized by reduction of both vertical and horizontal diameters, alteration of areola shape and size, and moderate to severe hypoplasia of the skin and glandular tissue. By contrast, a tubular breast presents with a long vertical diameter of the upper quadrants, extending downward far beyond the IMF, and a normal or relatively reduced horizontal diameter. Volume is either normal or increased. In general, tubular breasts can be corrected with mastopexy/reduction patterns of glandular resection and reshaping, whereas tuberous breasts require more release, reshaping of lower quadrants with glandular flaps, autologous fat, or the use of an implant.
The Constricted Base
Successful correction of the tuberous breast must include adequate expansion of the constricted base of the breast. Since the first descriptions of the tuberous deformity, most investigators have highlighted techniques to release, resurface, or camouflage the constricted base ( Fig. 5 ). Adequate release can often be achieved through implant pocket dissection with radial scoring as required. In severe cases, 2-stage release with the use of tissue expansion may be indicated. More recently, fat has been considered for filling a tight lower pole in conjunction with preoperative external expansion (see later discussion). Although Rees and Aston were first to discuss radial scoring, they did not actually transect the constricting ring. Dinner and Dowden considered that the skin itself was constricting, and advocated full-thickness skin and glandular incisions with transposition of local skin and subcutaneous tissue flaps.
More recently, internal glandular reshaping has allowed for correction of the constricted base without significant external scars or contour irregularities. Internal flaps fashioned from subareolar parenchyma, based anteriorly on the subareolar tissue or posteriorly on the chest wall, can be folded down to reconstitute the constricted lower pole. The unfolded subareolar gland flap was recently described in 42 breasts (26 patients). This modified Puckett technique, which included lengthening of the subareolar flap with 2 L-shaped releasing incisions, allowed further unfolding, elongation, and homogeneity of the flap to allow it to reach the new IMF and provide full coverage of a prosthesis.
Mandrekas and Zambacos used a modified inferior pole flap in 41 breasts over 10 years. These investigators used a periareolar donut excision and inferior pole exteriorized through the periareolar incision that was then transected vertically at 6 o’clock, releasing the constriction ring and forming 2 pillars that could be redraped and sutured if necessary to reform the lower pole of the breast. Implants were used if additional volume was required. Reported complications included bruising and swelling, hematoma, capsular contracture (secondary to hematoma), and asymmetry. No reoperations were required with a minimum follow-up of 18 months.
Volume Correction
In most cases, it will be necessary to increase volume either to establish a normal breast shape or to satisfy patient expectations. Volume can be increased with either autogenous or alloplastic techniques. The use of flaps for the correction of a tuberous breast has been described (see earlier discussion), although microfat injection is becoming a common method for the use of autogenous tissue.
Fat can be used to increase breast volume, with the added benefit of being able to control distribution of volume throughout the breast. Most often, fat will be added to the lower pole of the breast. When combined with techniques such as needle band release (also known as rigottomies), fat can be used to restore contour in the inferior pole and lower the IMF. External tissue expansion using a BRAVA device is a useful adjunct to fat injection and is thought to assist in retention of the fat graft through expansion of the recipient site, increasing the graft to capacity ratio and improving recipient site vascularity ( Fig. 6 ).
