Correction of Poland Syndrome (Chest Hypoplasia) Using Polymethylmethacrylate Implant

Roberto Chacur*1, Honório Sampaio Menezes2, Nívea Maria Bordin da Silva Chacur3, Danuza Dias Alves4, Rodrigo Cadore Mafaldo5, Leandro Dias Gomes6, Alexandre Peruzzo7 and Gisele Dos Santos Barreto8 1Surgeon, Leger Clinic, Rio de Janeiro, Brazil 2PhD in medicine, Leger Clinic, São Paulo, Brazil 3MD, Leger Clinic, Rio de Janeiro, Brazil 4MD, Leger Clinic, Porto Alegre, Brazil 5Surgeon, Leger Clinic, Brazil 6Surgeon, Leger Clinic, Porto Alegre, Brazil 7Plastic Surgeon, Porto Alegre, Brazil 8Biomedical Scientist, Leger Clinic, Porto Alegre, Brazil *Corresponding author: Roberto Chacur, Surgeon, Leger Clinic, Brazil DOI: 10.26717.BJSTR.2019.14.002493


Introduction
Poland anomaly (PA), also known as Poland syndrome, was first described by Alfred Poland as a unilateral underdevelopment or absence of the chest muscle, often associated with deformities such as syndactyly and chest wall deformity [1,2] Poland syndrome is characterized by a variable clinical presentation. The classical deformity consists of unilateral hypoplasia or aplasia of the sternocostal head of pectoralis major muscle and ipsilateral hand malformations. In addition, hypoplasia or aplasia of the breast is observed on the affected side in women. This condition is rare and present since birth [3,4]. The defect affects primarily the sternal portion of the pectoral muscles, and the clavicular portion of the muscle remains often unchanged [1,3] According to the National Human Genome Research Institute, the incidence is three times higher in males, and twice more frequent on the right side, and may even be associated with ipsilateral brachydactyly [3] Absence or decrease of hair, absence of the mammary gland, decreased nipple, more retractable or adherent skin and less subcutaneous tissue are characteristics that may also be associated with less muscle development [3]. smoking during pregnancy, occurs more precisely in the fourth month of pregnancy, when the vascular and lymphatic systems are formed. A decrease in subclavian artery blood flow can cause the anomalies that characterize the syndrome, such as atrophy of the affected region [1,3]. The aesthetic aspect is in general the most severe problem of the syndrome, but does not affect cognition, although there is relationship with other morphological changes [1,3]. Some associated systemic effects contribute with the correct diagnosis of the syndrome. Such changes can be observed and should be investigated during anamnesis, namely: gastrointestinal abnormalities, dextrocardia, diaphragmatic defect, change in the ipsilateral arm (size of the humerus, radius and ulna), biliary abnormality, oligodactyly (absence of fingers), syndactyly, costal arch abnormality, upper limb asymmetry, abnormal scapula, renal alteration or agenesis, encephalocele, microcefalia, vesicoureteral reflux and vertebral change [1,3].
The deformities in the hands are more evident at birth than chest wall abnormalities [5]. It is important to stress that, although the changes described above must be investigated, Poland syndrome is generally characterized only by underdeveloped muscles [5]. The aesthetic problem is important and becomes more uncomfortable during adolescence. In female patients, chest deformity becomes more evident during adolescence as the absence or asymmetry of the developing breasts occurs. In male patients, although the chest does not develop in adolescence, the chest wall deformity causes great suffering, having a negative effect in social situations, including the use of swim and sports suits.

Case Report
The patient P.C.R., 37 years of age, was received at Clínica LEGER, in Porto Alegre, on February 01 2011 at 1:30 pm. The patient presented total agenesis of the right pectoral muscle, but other systemic changes or symptoms were not reported. After diagnosis of Poland syndrome, a treatment based on bioplasty combined with ipsilateral tissue detachment, permanent epilation on the left side was suggested. A plastic surgeon was indicated to perform mastopexy to correct the contralateral gynecomastia presented by the patient.

Treatment
The treatment was conducted by bioplasty of the pectoral muscles using a total volume of 420 ml of 30% polymethylmethacrylate

Discussion
Although Poland syndrome is diagnosed mainly by clinical criteria, complementary analyses such as radiography, computed tomography and/or magnetic resonance imaging allow the investigation of the involvement of other organs and the mapping of the region before defining the treatment [1,3]. In the present case, no further tests were conducted and the patient did not present other complaints. The treatment can combine different methods, where appropriate, which are able to treat both the breasts and the chest wall (breast implants, muscle flaps). More recently, autologous fat cells have been implanted in these cases, according to the Coleman method [2]. In female patients, the treatment can be started only after mammary development has been completed, whereas in males it should be conducted as early as possible. The most common type of surgery used to treat female patients has been breast reconstruction with silicone implant [1,3,6]. In a study of the radiological appearance of normal and pathological results of mammary implantation of autologous fat, Constantini et al. [4] observed fatty cysts and fat necrosis, with and without calcification, related to the volume of injected fat. The product used in the present implant in a male patient, as in this case, it is not always feasible because of the difficulty of finding a donor site. Costa et al [5] observed that the deformity caused by the absence of sternocostal part of pectoralis major muscle can be corrected, with aesthetic purpose, by a latissimus dorsi pedicled flap or myocutaneous flap, in case of breast implant surgery [1,3].
Expanders and implants, transposition of latissimus muscle flaps (when not affected by the syndrome), and rectus abdominis muscle flaps, in case of absence of the latissimus dorsi, are alternatives that have been proposed to restore the breast volume [7][8][9]. These techniques can achieve excellent results depending on the degree of deformation, but the aesthetic results of reconstruction of the anterior axillary pillar and the filling of the infraclavicular fossa have been disappointing [10]. Furthermore, an is the inability to accurately determine the final volume available of omentum, at the time of planning the surgery. In the case reported here, there was no need of hospitalization or postprandial discomfort, and different from the study reported by Costa et al. [5] there was no difficulty in calculating the final volume. According to the classification of Foucras et al. [9], four patients presented grade II Poland syndrome, three had grade I and two patients had grade III. Three of the patients (two with grade II and one with grade III Poland syndrome) received silicone implants.
One teenage male patient, who was too embarrassed by his deformity, was treated with autologous fat injection. Three patients with moderate breast asymmetry were treated by contralateral liposuction, and the last patient, by resection of the contralateral breast. Intra-or postoperative complications were observed in eight patients. The aesthetic results were in general satisfactory.
PMMA filling in large volumes has been reported even for replacement of gluteal prosthesis without adverse effect observed after five years of follow-up [13] and also in other regions of the body such as arm, chest and back for both aesthetic and reconstructive purposes in patients traumatized and with sequelae [14]. The results include more attractive contours and volumetric repositioning compromised by the action of time.

Conclusion
In conclusion, our results show that the treatment of Poland syndrome using polymethylmethacrylate implant, in addition to being an effective alternative for aesthetic correction of the deformity, reduced the risk of scarring and had a satisfactory aesthetic result.