Tuberous Breasts

Updated 3/2017-- photos and all links (except to my own posts) removed as many are no longer active and it was easier than checking each one.

The latest edition of the Aesthetic Surgery Journal (Sept/Oct 2010) has a really nice article (first reference below) on this tuberous breasts. One of the best things about the article is the great photos, both of the deformity (includes this one to the right) and the corrective procedure.

Another nice thing the article has is the review of the breast’s embryology which is critical to understanding the formation of the deformity (bold emphasis is mine).

The breast originates from the mammary ridge, which develops in utero from the ectoderm during the fifth week. Shortly after its formation (in the seventh to eighth weeks), most parts of this ridge disappear, except for a small portion in the thoracic region, which persists and penetrates the underlying mesenchyme around 10 to 14 weeks. Further differentiation and development of the breast occurs during the intrauterine life and is completed by the time of birth, after which essentially no further development occurs until puberty.

During puberty, the mammary tissue beneath the areola grows with enlargement of the areola, until the age of 15 to 16, when the breast assumes its familiar shape. As a result of the ectodermal origin of the breast and its invagination into the underlying mesenchyme, the breast tissue is contained within a fascial envelope, the superficial fascia. This superficial fascia is continuous with the superficial abdominal fascia of Camper and consists of two layers: the superficial layer (which is the outer layer covering the breast parenchyma) and the deep layer (which forms the posterior boundary of the breast parenchyma and lies on the deep fascia of the pectoralis major and serratus anterior muscles). The deep layer of the superficial fascia is penetrated by fibrous attachments (suspensory ligaments of Cooper), joining the two layers of the superficial fascia and extending to the dermis of the overlying skin and the deep pectoral fascia. Of note is that the superficial layer of this fascia is absent in the area underneath the areola, as can easily be demonstrated by the invagination of the mammary bud in the mesenchyme.

Clinical experience has shown us and other authors that in cases of tuberous breasts, there is a constricting fibrous ring at the level of the periphery of the nipple-areolar complex that inhibits the normal development of the breast. This constricting ring of fibrous tissue is denser at the lower part of the breast and does not allow the developing breast parenchyma to expand during puberty. Histology confirmed the existence of such dense fibrous tissue in the area of this “constricting ring.” Specimens from two of our patients have been examined, and they showed large concentrations of collagen and elastic fibers, arranged longitudinally. We believe this ring represents a thickening of the superficial fascia, as described earlier. Perhaps the two layers of this fascia join at a higher level than usual, or the suspensory ligaments are thicker and more dense.

Tuberous breast deformity is a rare condition that becomes apparent during teenage years as the breast develop. As noted in the embryology description, the deformity is due to a constricting fibrous ring which does not allow the breast to form in a normal shape.

The deformity which was first described in 1976 by Rees and Aston, can be either unilaterally or bilaterally. When bilateral, the deformity may be vary in degree. It has many other names: tubular breasts, Snoopy breasts, herniated areolar complex, domed nipple, nipple breast, constricted breast, lower pole hypoplasia, and narrow-based breast.

A common classification of tuberous breast deformity is the one proposed by Grolleau et al (photo credit):

• Type I: deficiency of the lower medial quadrant. (A)
• Type II: deficiency of both lower quadrants. (B)
• Type III: deficiency of all four quadrants. (C,D)

The only way to correct this deformity is surgery. Even if no implant is needed or desired, the nipple/areolar complex benefits from a periareolar donut-type skin excision, reducing the areola to the desired size, usually 4 to 4.5 cm in diameter.

I agree with the authors that the constricting fibrous ring needs to be divided so the breast parenchyma can assume a more natural shape. When an implant is used, the subglandular or duel-plane position is preferred.

I would encourage reading the full article for more tips.

REFERENCES

Aesthetic Reconstruction of the Tuberous Breast Deformity: A 10-Year Experience; Mandrekas AD, Zambacos GJ; Aesthetic Surgery Journal September/October 2010 30: 680-692, doi:10.1177/1090820X10383397

The tuberous breast; Rees TD, Aston SJ; Clinics of Plastic Surgery 1976;3:339-347.

Breast Base Anomalies: Treatment Strategy for Tuberous Breasts, Minor Deformities, and Asymmetry; Grolleau, Jean-Louis; Lanfrey, Etienne; Lavigne, Bruno; Chavoin, Jean-Pierre; Costagliola, Michel; Plastic & Reconstructive Surgery. 104(7):2040-2048, December 1999.

Aesthetic Reconstruction of the Tuberous Breast Deformity; Mandrekas, Apostolos D.; Zambacos, George J.; Anastasopoulos, Anastasios; Hapsas, Dimitrios; Lambrinaki, Nektaria; Ioannidou-Mouzaka, Lydia; Plastic & Reconstructive Surgery. 112(4):1099-1108, September 15, 2003.

Madelung Deformity

Updated 3/2017-- photos and all links removed as many are no longer active and it was easier than checking each one.
 

Otto W. Madelung described the wrist deformity bearing his name in 1878 as ”Die spontane subluxation der hand nocte vorne” or “spontaneous forward subluxation of the hand.” Several authors prior to Madelung, including Dupuytren in 1834, Nelaton in 1847, and Malgaigne in 1855, had described entities termed carpus curvus, radius curvus, progressive subluxation of the wrist, manus valgus, manus furca, and idiopathic progressive curvature of the radius. However, Madelung first accurately described it clinically and proposed both an etiology and treatment. 

Madelung deformity (MD) of the wrist is characterized by a growth disturbance in the ulnar (small-finger side) and volar (palm-side) half of the distal (near the wrist) radial physis (growth plate of the radius). As the bone grows, the carpal end of the radius curves in a volar and ulnar direction rather than straight. The distal articular surface of the radius slants in a volar and ulnar direction to a greater degree than normal, the carpus (wrist) and hand shift volarward, and the ulna remains dorsally.

It occurs predominantly in adolescent females. One third of cases of MD are transmitted in an autosomal dominant fashion. The condition has a variable expression and 50% penetrance. MD is bilateral in 50% of cases. There is an associated with mesomelic dwarfism and Turner's Syndrome.

MD has been classified into four different etiologic groups:

1. Posttraumatic--has been found following repetitive trauma or following a single event that disrupts growth of the distal radial ulnar-volar physis.
2. Dysplastic--Bone dysplasias associated with MD include multiple hereditary osteochondromatosis, Ollier disease, achondroplasia, multiple epiphysial dysplasias, and the mucopolysaccharidoses (eg, Hurler and Morquio syndromes). The most important dysplasia associated with MD, however, is dyschondrosteosis
3. Chromosomal or genetic (Turner syndrome)
4. Idiopathic or primary

Symptoms usually begin during adolescence in girls aged 10-14 years. Patients experience increasing deformity and pain in the wrist with decreased range of motion. On physical examination, the hand is translated volarly to the long axis of the forearm. The ulna, being relatively unaffected, abuts the carpus and becomes prominent dorsally relative to the carpus and hand. Range of motion is decreased, with a limitation of supination, dorsiflexion, and radial deviation. Pronation and flexion usually are normal, but may be decreased.

The diagnosis is confirmed with PA and lateral X-ray views of the forearm and wrist. Several distinct radiographic features of MD exist (described here). Because the deformity is 3-dimensional, a better understanding may be obtained with tomograms or CT scans.

Nonoperative management may be helpful in skeletally mature individuals with MD and mild-to-moderate short-term wrist pain. This management may include anti-inflammatories and appropriate splinting to relieve joint irritation from overactivity.

Operative treatment can be divided into those procedures that
1. Correct the primary deformity of the radius

• Vickers Physiolysis--In 1992, Vickers and Nielsen described the lesion in the volar and ulnar distal radius as both bony and ligamentous, and they stated that it is an inherent failure of focal growth and structural tethering of further growth. They described an ulnar-volar release for MD of the physis, called physiolysis. This then allows normal and compensatory growth to correct the deformity. The deformity must be noticed early when significant growth remains.

2. Those that attempt to decrease pain and increase range of motion by making a compensatory change in the ulna

• Osteotomy of radius--If the deformity has progressed in an older child and remaining growth is insufficient, several procedures can be used to correct the position of the distal radiocarpal joint surface. They usually consist of a biplane osteotomy, either closing or opening wedge, which corrects the position of the joint surface and brings the radius and ulna into a more proper position. If a positive ulnar variance remains, an ulnar shortening procedure can be performed.
• Radioulnar length adjustment--In MD, the ulna grows normally and becomes longer than the radius. Because the radius is volar, the ulna appears to be subluxed dorsally. The incongruence at the distal radioulnar joint and the impingement of the radius on the ulna in supination may cause pain and contribute to decreased range of motion in supination. In order to allow unrestricted rotation, several ulnar procedures have been described. These include 1) ulnar shortening, 2) ulnar head resection and a DRUJ arthrodesis, and 3) ulnar pseudoarthrosis. Some have advocated both radial and ulnar procedures.
• Ulnar resection--The Darrach procedure long has been a treatment option for MD. This construct in isolation may leave the carpus unstable, especially in light of the increased ulnar and volar slope of the radial articular surface. The carpus therefore tends to slide off of the ulnar side of the wrist. Several authors have devised procedures to solve this problem. The Sauve-Kapandji (Lauenstein) procedure may be a viable option for MD as the ulnar head is preserved and there is less chance for ulnar migration of carpus.

3. Those that address both

• In 2000, Carter and Ezaki reported a combined procedure using a Vickers ligament release and a dome-shaped osteotomy of the radius to correct all of the aspects of the radial deformity, including the radial and volar translation of the distal metaphysis. It not only corrects the deformity but also decreases pain and increases range of motion.

Postoperative management will depend upon what is done surgically. Six to 8 weeks of cast immobilization is necessary following an osteotomy of the radius or ulna to allow for bony healing. Hand therapy is necessary in children who are not able to regain range of motion on their own after 2-3 months without restrictions. These children should be monitored until surgical issues are resolved and then yearly until skeletal maturity is reached. Following a physiolysis procedure, yearly radiographs can be used to document improvement in the position of the distal radial articular surface with growth.

References

• Madelung Deformity by Paul M Lamberti, MD--eMedicine Article
• Carter PR, Ezaki M: Madelung's deformity. Surgical correction through the anterior approach; Hand Clin 2000 Nov; 16(4): 713-21 [Medline]
• Vickers D, Nielsen G: Madelung deformity: surgical prophylaxis (physiolysis) during the late growth period by resection of the dyschondrosteosis lesion. J Hand Surg [Br] 1992 Aug; 17(4): 401-7 [Medline]
• Madelung's Deformity--Wheeless' Online Textbook of Orthopedics
  
• Madelung's Deformity--Children's Hospital Boston