Source: Annual Congress 2002 - Chest trauma, airways and miscellaneous

Abstract

Poland's syndrome is a non-genetic congenital abnormality characterized by unilateral aplasia of pectoralis muscles and ribs III-V. Males are affected more often and the right side of the chest is involved in 60% of the cases.
Cardio-respiratory impairment results from reduction of the thoracic volume due to depression deformity of the chest and from the lack of costal support for the heart and lung. An isolated dextrocardia in Poland's syndrome is seen in 7% of the cases, and is not associated with other cardiovascular anomalies. Lung herniation occurs in 8% of the cases. Radiography confirms unilateral hyperlucent lung and shift of the heart towards the unaffected side. Our experience includes 27 patients, 20 of whom we operated upon.
Surgical intervention is indicated due to depression of the chest and the possibility of its progression, lack of adequate protection of the heart and lung, paradoxical movement of the chest wall, and hypoplasia of the breast in females. Correction in usually carried out in children in two stages, while in adults, it should be done in one. During surgery, unilateral ventilation of the opposite lung is recommended, as well as epidural anesthesia, which is continued postoperatively. The defect and lung herniation are corrected by bony allo- or autografts or split rib grafts taken from the unaffected side of the chest. It may also be repaired with a prosthetic mesh patch sutured to the aplastic rib stumps, in combination with the latissimus dorsi musculocutaneous flap. In female adult patients, prosthetic breast implantation is advisable.

Rating:

Share or cite this content

Citations should be made in the following way:
A. A. Fokin, F. Robicsek, T. N. Masters (Charlotte, United States Of America). Cardio-respiratory impairment in patients with Poland's syndrome. Eur Respir J 2002; 20: Suppl. 38, 975

Member's Comments

No comment yet.

Related content which might interest you: Imunnopathologic aspects of the bronchoobstructive syndrome in children Source: Annual Congress 2009 - Host factors in paediatric bacterial respiratory infection Year: 2009 Pulmonary rehabilitation in patients with postpolio syndrome Source: Eur Respir J 2001; 18: Suppl. 33, 224s Year: 2001 Neurocognitive profile of children with congenital central hypoventilation syndrome (CCHS) Source: Annual Congress 2011 - Obstructive sleep apnoea: the heart and the brain Year: 2011 Birt Hogg-Dubé syndrome in Poland Source: Virtual Congress 2020 – Addressing unresolved questions in rare diffuse parenchymal lung diseases: interdisciplinary research is the key Year: 2020 Exercise performance of children and adolescents affected by severe acute respiratory syndrome (SARS) Source: Eur Respir J 2004; 24: Suppl. 48, 393s Year: 2004 Pulmonary manifestations of Sjögren's syndrome Source: Eur Respir Rev 2016; 25: 110-123 Year: 2016 Prevalence and clinical characteristics of obesity – hypoventilation syndrome in Greece Source: Annual Congress 2008 - Socioepidemiological studies in obstructive sleep apnoea Year: 2008 Assessment and treatment of congenital central hypoventilation syndrome in children Source: Sleep and Breathing Conference 2021 Year: 2021 Respiratory evaluation in Down's syndrome Source: Eur Respir J 2002; 20: Suppl. 38, 321s Year: 2002 Respiratory assessment in young subjects with Down syndrome Source: Annual Congress 2010 - Distortions of the respiratory system in the lung function Year: 2010 A new syndrome or bilateral Poland’s syndrome Source: Annual Congress 2013 –Chest wall and pleura Year: 2013 Pulmonary function in children with hyperventilation syndrome Source: Eur Respir J 2002; 20: Suppl. 38, 140s Year: 2002 Respiratory system impairments in children with mucopolysaccharidoses: Hurler and Hunter syndromes Source: International Congress 2017 – Assessing the impact of respiratory and sleep problems in children Year: 2017 The prevalence of hyperventilation syndrome among children Source: Eur Respir J 2003; 22: Suppl. 45, 319s Year: 2003 Sleep-related hypoventilation in children with Prader-Willi syndrome: Experience from two UK paediatric sleep centres Source: International Congress 2016 – Latest advances in paediatric sleep medicine Year: 2016 Experience of 10-years with churg-strauss syndrome: is this an accompaniment or a transition from analgesic-induced asthma? Source: Eur Respir J 2001; 18: Suppl. 33, 433s Year: 2001 Presence of Kartagener syndrome reduces exercise capacity in primary ciliary dyskinesia Source: International Congress 2016 – Effects of exercise training interventions in chronic respiratory disease Year: 2016 Severe persistent asthma in children with Ehlers-Danlos’ syndrome Source: Eur Respir J 2004; 24: Suppl. 48, 162s Year: 2004 Prevalence and diagnosis of obesity hypoventilation syndrome (OHS) in ambulatory obese patients Source: International Congress 2016 – Epidemiological aspects of obstructive sleep apnoea Year: 2016 Is widal syndrome usually a severe disease? Source: Eur Respir J 2004; 24: Suppl. 48, 47s Year: 2004