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Unilateral Hypoplasia or Absence of Pectoralis Major Muscle

Presentation

[…] chest No or small alterations May be present Third (very severe) Amastia; areolar asymmetry Different manifestations Present May be present Degree of presentation Mammary alterations Musculoskeletal chest alterations Ipsilateral superior limb alterations [academic.oup.com]

Discussion The PS case presented here is one of the few cases described from Bangladesh and had peculiar presentation as it involved only the unilateral aplasia of the pectoralis major muscle without any other associated defects. 2,4 It presented in the [jpma.org.pk]

This feature can present alone, or with ipsilateral chest wall and upper extremity deformities. [thoracickey.com]

The syndrome has since evolved to encompass a myriad of anomalies, which rarely present collectively in the same individual. [ucdavis.pure.elsevier.com]

Stage of breast development Status of the latissimus dorsi muscle – Present or absent or hypoplastic Extraocular movements – to look for associated Mobius syndrome. [boneandspine.com]

Skin

  • Angiomatosis

    Syndrome 168 518 Progeria HutchinsonGilford Syndrome 169 519 Tarlov Cyst 171 520 Madelung Deformity and Dyschondrosteosis LériWeill Syndrome 173 Phakomatoses Neurocutaneous Syndromes 175 61 Neurofibromatosis 176 62 SturgeWeber Syndrome Encephalotrigeminal Angiomatosis [books.google.de]

Treatment

Treatment of Poland Syndrome Many patients may not seek he treatment. The treatment of Poland Syndrome would depend on the specific symptoms. Generally, the patients present for treatment of the chest deformity and breast asymmetry. [boneandspine.com]

There are various opinions on the timing to start treatment of patients with Poland syndrome. [journals.eco-vector.com]

Each of the succinct chapters represents the personal treatment protocols of leading experts. The Advanced Therapy concept is famous for conveying therapeutic information in an efficient, quick-review fashion. [books.google.de]

Edelweiss Aesthetic improvements in Poland's syndrome treatment with omentum flap [12] A.E. Seyfer, J.P. Fox, C.G. [archbronconeumol.org]

An algorithm for the planning of surgical treatment is also presented. [academic.oup.com]

Prognosis

Treatment and prognosis The condition is benign and depending on the degree of involvement of the upper extremity may be asymptomatic. Cosmetic reconstructive surgery may be considered (e.g. breast implant). [radiopaedia.org]

PMID: 8915289 Prognosis Chalak LF, Parham DM Pediatr Dev Pathol 2007 Mar-Apr;10(2):134-7. doi: 10.2350/06-04-00801.1. PMID: 17378683 Beals RK, Crawford S Clin Orthop Relat Res 1976 Sep;(119):166-71. [ncbi.nlm.nih.gov]

Etiology

The etiology is unknown, but speculation includes hypoplasia of the subclavian artery or its branches between the 6th and 7th week of gestation. [humpath.com]

This non-genetic and non-teratogenic etiology suggests that Poland syndrome is entirely sporadic. [annsaudimed.net]

Subclavian artery supply disruption sequence: Hypothesis of a vascular etiology for Poland, Klippel-Feil, and Moebius anomalies. Am J Med Genet 1986;23:903-18. 6. David TJ. Nature and etiology of the Poland anomaly. [thoracicmedicine.org]

Discussion The exact etiology of Poland's sequence is unknown. The male-female ratio is 3:1. In 75% of cases, right side is affected. [ijdvl.com]

The etiology of this syndrome is obscure, although two pathogenic mechanisms have been proposed. [aafp.org]

Epidemiology

This is mainly due to its ability to achieve previously unachievable quality of reconstruction with minimal scarring. [14] Epidemiology [ edit ] Poland syndrome affects males three times as often as females and affects the right side of the body twice [en.wikipedia.org]

[…] of serratus, external oblique, pectoralis minor, latissimus dorsi, [1] infraspinatus, and supraspinatus muscles Total absence of anterolateral ribs and herniation of lung Symphalangism with syndactyly and hypoplasia or aplasia of the middle phalanges Epidemiology [emedicine.medscape.com]

Epidemiology The true incidence of Poland’s Syndrome is difficult to estimate due to the variability with which it presents and the differences that occur in the specific patient cohorts. [thoracickey.com]

Pathophysiology

We hypothesise that the HK1 located on the chromosome 10 encoding hexokinase-1 is possibly linked to the pathophysiology of CHI. Learning points: Congenital hyperinsulinism (CHI) is known to be associated with various syndromes. [edmcasereports.com]

Prevention

This surgical procedure is recommended for large anterior chest wall defects, and it prevents paradoxical movement. Moreover it provides for individual remodeling of the defect depending on the shape of the patient's chest. [archbronconeumol.org]

The disturbance of the blood supply is due to thrombus or embolus, which prevent the blood to arrive at the developing tissue. [mjdrdypu.org]

Some authors believe that surgery should be performed at the end of adolescence, which will enable maximum symmetry and prevent subsequent interventions [2, 29], while others show good results in treatment of children at an earlier age [30, 31]. [journals.eco-vector.com]

The interruption of the blood supply is caused by thrombus or embolus, which prevent the blood from reaching the developing tissue. Another cause of blood supply interruption is the maldevelopment of vessels. [jpma.org.pk]

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