Correction of Poland Syndrome (Chest Hypoplasia) Using Polymethylmethacrylate Implant
Journal Title: Biomedical Journal of Scientific & Technical Research (BJSTR) - Year 2019, Vol 14, Issue 1
Abstract
Poland syndrome is a rare disease characterized by unilateral underdevelopment or absence of the chest muscle. Several techniques are used to correct the chest deformity. Objective: To describe a minimally invasive treatment option, with the use of polymethylmethacrylate (PMMA), for difficult-to-treat deformities caused by Poland Syndrome. Methods: PMMA was percutaneously implanted using a microcannula, after local anesthesia and with the patient awake. The volume of the implant was administered gradually, until the deformity was corrected. The patient returned to normal life immediately after the procedure, without the need for hospitalization. Conclusion: In addition to being an effective option in aesthetic correction of the deformity, the use of PMMA reduced the risk of scarring and obtained satisfactory aesthetic result. 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]. The etiology is unknown, but the condition is most likely due to a decrease in blood flow in the region during embryonic development. Several studies suggest that a vascular change, caused by maternal 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.
Authors and Affiliations
Roberto Chacur, Honório Sampaio Menezes, Nívea Maria Bordin da Silva Chacur, Danuza Dias Alves, Rodrigo Cadore Mafaldo, Leandro Dias Gomes, Alexandre Peruzzo, Gisele Dos Santos Barreto
Keywords
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- EP ID EP594307
- DOI 10.26717/BJSTR.2019.14.002493
- Views 134
- Downloads 0
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