Presentation and Treatment of Poland Anomaly

Buckwalter, J. A.; Shah, Apurva S.

doi:10.1177/1558944716647355

Cited by 23 publications

(19 citation statements)

References 44 publications

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“…Corrective surgery should be planned at an optimal time for symbrachydactyly before flexion contractures and angular deformity sets in. Rarely, corrective osteotomies are required for misaligned joints of the upper extremity to give a functional hand (85,86).…”

Section: Surgical Intervention and Reconstructionmentioning

confidence: 99%

A narrative review of Poland’s syndrome: theories of its genesis, evolution and its diagnosis and treatment

Hashim¹,

Quek²,

Chandran³

2021

Transl Pediatr

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Poland's syndrome (PS) is a rare musculoskeletal congenital anomaly with a wide spectrum of presentations. It is typically characterized by hypoplasia or aplasia of pectoral muscles, mammary hypoplasia and variably associated ipsilateral limb anomalies. Limb defects can vary in severity, ranging from syndactyly to phocomelia. Most cases are sporadic but familial cases with intrafamilial variability have been reported.Several theories have been proposed regarding the genesis of PS. Vascular disruption theory, "the subclavian artery supply disruption sequence" (SASDS) remains the most accepted pathogenic mechanism. Clinical presentations can vary in severity from syndactyly to phocomelia in the limbs and in the thorax, rib defects to severe chest wall anomalies with impaired lung function. Most patients have subtle presentation at birth and milder forms in childhood. Functional limitations due to PS are usually minimal. Surgical treatment aims to improve pulmonary functions arising from severe thoracic deformities but is more often done to enhance the cosmesis. The use of adipose-derived mesenchymal stem cells and fat transfer have shown promising results in recent times for correction of chest defects and breast augmentation. Gaining deeper insights into the etiopathogenesis and clinical presentation of PS will improve the clinical recognition and management of this rare condition. In this review article, we aim to outline the details of this syndrome including its etiopathogenesis, evolution, spectrum of clinical manifestations, other systemic associations, diagnostic modalities, and recent advances in treatment.

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Section: Surgical Intervention and Reconstructionmentioning

confidence: 99%

A narrative review of Poland’s syndrome: theories of its genesis, evolution and its diagnosis and treatment

Hashim¹,

Quek²,

Chandran³

2021

Transl Pediatr

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“…It is mostly unilateral and more commonly involves the musculoskeletal system of right side of the thorax and the right upper limb. 4 The condition has also been called as ipsilateral thorax syndrome, limb/body-wall defect, fissure thoracis lateralis, pectoral-aplasia dysdactylia syndrome or unilateral chesthand deformity in view of the body parts affected. 5 In addition to these typical characteristics of this syndrome, there are reports of associated anomalies in many organ systems like club foot, hemivertebrae, scoliosis, hypospadias, inguinal hernia, renal malformations, cardiovascular defects and hematopoetic abnormalities.…”

Section: Introductionmentioning

confidence: 99%

Anaesthetic challenges and difficult airway in a child with Poland’s syndrome

Kalra¹,

Gupta

2021

IJCA

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: Poland’s syndrome is an uncommon clinical disorder with a typical musculoskeletal deformity of thorax and ipsilateral upper limb with a variable spectrum of associated anomalies. Although patients of this musculoskeletal disorder have several features with potential anaesthetic risks including malignant hyperthermia, previous reports about anaesthetic management of these patients are limited. : We report the successful anaesthetic management of a child of Poland’s syndrome posted for surgical correction of club foot who had an anticipated difficult airway alongwith unilateral chest wall defect with paradoxical breathing and ipsilateral syndactyly and hence, posing challenges in the insertion of endotracheal tube, ventilatory management, regional anaesthesia and prevention of any risk of development of malignant hyperthermia. Paramount in the anaesthetic care of such patients is a thorough preoperative evaluation and a cautious intraoperative anaesthetic management. Avoidance of halogenated inhalational agents and depolarising neuromuscular blocking drugs, and use of controlled ventilation and regional anaesthetic techniques are recommended. The possibility of a compromised airway should always be kept in mind while anaesthetising these patients.

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“…Poland syndrome is a rare congenital condition with an estimated incidence of 1 in 30000 to 1 in 80000 live births, which was initially described by Alfred Poland in 1841 as a partial or complete absence of the pectoralis major and minor muscles, hypoplasia of the breast as well as ipsilateral upper limb malformations (1,2). This condition has male and right side predominance (3).…”

Section: Introductionmentioning

confidence: 99%