Poland Syndrome

Kennedy, Kelsey R.; Wang, Apphia L.

doi:10.1056/nejmicm1709713

Cited by 11 publications

(9 citation statements)

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“…Disruption of blood flow through the subclavian artery during the sixth embryonal week and abnormalities of the apical ectodermal ridge have been postulated as possible causes. 1 The hallmark of Poland syndrome is the unilateral absence or underdevelopment of the sternal portion of the pectoralis major muscle with or without pectoralis minor involvement. It occurs sporadically though there are few instances where individuals of the same family are affected.…”

Section: Discussionmentioning

confidence: 99%

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Poland Syndrome: A curious anomaly

Adjei¹

2020

Afri Jour Cur Med Res

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Background: Poland syndrome also referred to as Poland Anomaly or sequence is a rare congenital anomaly characterized by absence or under development of the pectoralis major and sometimes minor muscle with or without limb asymmetry and a combination of other physical anomalies. The global incidence is estimated to be 1:10,000 to 1:100,000 but is thought to be underestimated due to the different degrees of the physical anomalies and a general lack of awareness about the condition. No cases have been reported from Ghana to the best of our knowledge. History: A sixteen year old girl presented with gross asymmetry of the breasts. Further examination revealed asymmetry of the upper limbs with shortened digits on the left. Anomaly only became noticeable during puberty. Conclusion: Though defects in Poland syndrome are present at birth, some individuals with Poland syndrome only present during puberty. Poland syndrome can be left-sided and also occur in females though majority of reported cases tend to be right-sided involving males.

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Section: Discussionmentioning

confidence: 99%

“…Martin Adjei(MBChB) 1* ; 1,2 1 School of Medical Sciences-Kwame Nkrumah University of Science and Technology (SMS-KNUST), Kumasi-Ghana 2 Komfo Anokye Teaching Hospital (KATH) Kumasi-Ghana…”

Section: Poland Syndrome: a Curious Anomalymentioning

confidence: 99%

Poland Syndrome: A curious anomaly

Adjei¹

2020

Afri Jour Cur Med Res

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“…The exact etiology of Poland syndrome is unknown but is thought to be related to disruption of the blood supply to the embryonic tissues that give rise to the chest wall and hand. 35 Clinically, patients often present with breast hypoplasia and thoracic abnormalities, including subclavicular hollowing and absence of the sternal head of the pectoralis major muscle and an anterior axillary fold. Long bones, rib cage, cartilage, skin, breast, and subcutaneous tissue can all be malformed on the affected side.…”

Section: Poland Syndromementioning

confidence: 99%

“…Long bones, rib cage, cartilage, skin, breast, and subcutaneous tissue can all be malformed on the affected side. 35,36 In males, chest wall asymmetry is a major issue. 36 In females, breasts are also abnormal in both size and position, making breast asymmetry a major issue, 36,37 Autologous fat grafting has been used alone, and more often in conjunction with traditional reconstructive techniques such as flaps and implants, to fill the volume and contour deficits seen in patients with all grades of severity of Poland syndrome.…”

Section: Poland Syndromementioning

confidence: 99%

Applications of Fat Grafting in Pediatric Patients

Shih

Abu‐Ghname

Davis

et al. 2020

Seminars in Plastic Surgery

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Autologous fat grafting has become a widely utilized technique for a variety of cosmetic and reconstructive procedures. Its potential for volume restoration and tissue regeneration has made it a popular method for treating soft tissue defects in both adult and pediatric populations. While autologous fat grafting in the pediatric setting is not as well characterized as it is in the adult setting, various reports have demonstrated the safety and utility of its applications in nonadult patient populations. In this article, we present the first comprehensive review of the current applications of autologous fat grafting in pediatric patients. Specific challenges to fat grafting in the pediatric setting and future applications will also be discussed.

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“…Poland syndrome (PS) is characterized by hypoplasia / agenesis of the pectoralis major muscle, variably associated with upper limb anomalies (ULA)and/or thoracic anomalies (TA) [ 1 , 2 ]. A wide range of TA is involved in PS, which includes thoracic skelecton anomalies, such as chest wall depression, rib hypoplasia to aplasia, sternal anomalies, as well as thoracic soft tissue anomalies, such as subtle breast, limited subcutaneous fat and axillary hair absence.…”

Section: Introductionmentioning

confidence: 99%

Anaesthesia for chest wall reconstruction in a patient with Poland syndrome: CARE-compliant case report and literature review

2018

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BackgroundPoland syndrome is a rare congenital disease, characterized by agenesis/hypoplasia of the pectoralis major muscle, usually associated with variable thoracic anomalies that needed chest wall reconstruction under general anesthesia. Anaesthetic management in Poland syndrome has scarcely been described.Case presentationHere, we present our anaesthetic management of Nuss procedure for chest wall correction in a 5 years old patient with Poland syndrome. We also reviewed the reports of anaesthetic management of Poland syndrome by searching Pubmed, and summarize the perioperative procedures that may warrant a safe surgery.ConclusionsExaminations before surgery, intraoperative monitoring, choice of general anesthetics and pain management after surgery should all be contemplated.

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Poland Syndrome

Cited by 11 publications

References 0 publications

Poland Syndrome: A curious anomaly

Poland Syndrome: A curious anomaly

Applications of Fat Grafting in Pediatric Patients

Anaesthesia for chest wall reconstruction in a patient with Poland syndrome: CARE-compliant case report and literature review

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