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Unilateral Hypoplasia or Absence of Pectoralis Major Muscle


Presentation

  • This work presents state-of-the-art techniques in thoracic surgery from the investigators who have developed them. Each of the succinct chapters represents the personal treatment protocols of leading experts.[books.google.de]
  • […] 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]
Pain
  • New chapters to this second edition include: New Techniques to Manage Pain, Advances in Diagnostic Imaging, Management of Germ Cell Tumors of the Mediastinum, Updates on Diaphragmatic Pacing and Preoperative Evaluation of Neuro-Motor Diseases of the Esophagus[books.google.de]
  • A handful of those cases have involved rib defects and pain on the left side. 1,4–7 Until now, there have been no reports of right-sided chest pain in the setting of left-sided Poland syndrome.[nzma.org.nz]
  • He denied any previous injury to the chest, surgical treatment to his chest, or pain/weakness to the right side. Physical Exam: The patient was well-appearing and in no acute distress.[amssm.org]
  • Rimmed vacuoles Tibialis muscle weakness Edema Gait disturbance Neck flexor weakness Pain Hepatomegaly Calf muscle hypertrophy Axonal loss Necrotizing myopathy Paralysis Agenesis of corpus callosum Oxycephaly Hyperlordosis Seizures Respiratory failure[mendelian.co]
  • He reported retrosternal pain on deep inspiration, fatigue and mild dyspnea on exertion. Cardiopulmonary auscultation revealed a non-displaced apex beat.[archbronconeumol.org]
Surgical Procedure
  • 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 functional significance of the RA and its increasing usage in surgical procedures makes it necessary to be aware of the variations in the morphology of RA.[pulsus.com]
  • Eighteen patients underwent surgical correction; a total of 39 surgical procedures were performed using the CRPS algorithm.[academic.oup.com]
  • Most patients with Poland syndrome do not require surgical procedures to correct muscle or chest wall deformities. Breast reconstruction and augmentation should be considered after full development for women with amastia or hypoplasia of the breast.[jamanetwork.com]
Down Syndrome
  • Neonatal bilateral vocal fold paralysis may be caused by birth trauma, asphyxia, or central nervous system (CNS) anomalies. 2 Other recognizable syndromes such as Down syndrome, 22q deletion syndrome, and Robinow syndrome can present with bilateral vocal[annsaudimed.net]
Pneumonia
  • Consult new chapters covering Ultrasound, Innate Immunity, Adaptive Immunity, Deposition and Clearance, Ventilator-Associated Pneumonia.[books.google.de]
  • […] acidosis Abnormality of movement Severe global developmental delay Pallor Irritability Developmental regression Abnormality of the eye Clonus Mental deterioration Retinopathy Myalgia Coma Hypoglycemia Feeding difficulties in infancy Acidosis Myoclonus Pneumonia[mendelian.co]
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]
Fear
  • Parents of Poland Syndrome svidaho.net [Support Groups] Our Questions & Fears The 1st Surgery POLAND SYNDROME Victor A. McKusick, OMIM, Johns Hopkins University, February 27, 2006 CLINICAL SYNOPSIS ...[ibis-birthdefects.org]
Steppage Gait
  • gait Distal sensory impairment Respiratory distress Respiratory insufficiency Micrognathia EMG: myopathic abnormalities Diaphragmatic weakness Intellectual disability Areflexia Macrocephaly Low-set ears Kyphoscoliosis Cleft palate Cryptorchidism Muscular[mendelian.co]
Cranial Nerve Involvement
  • The most common cranial nerve involved is the 6 th cranial nerve (abducent). The oculomotor and trochlear can also be involved.[annsaudimed.net]

Treatment

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

Prognosis

  • 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]
  • 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]

Etiology

  • Etiology J Pediatr Orthop 2012 Oct-Nov;32(7):727-31. doi: 10.1097/BPO.0b013e318269c898. PMID: 22955538 Seyfer AE, Fox JP, Hamilton CG Plast Reconstr Surg 2010 Sep;126(3):902-11. doi: 10.1097/PRS.0b013e3181e60435.[ncbi.nlm.nih.gov]
  • 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]
  • The etiology of this syndrome is obscure, although two pathogenic mechanisms have been proposed.[aafp.org]

Epidemiology

  • […] 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]
Sex distribution
Age distribution

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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