A Rare Case of Poland's Syndrome (original) (raw)

East and Central African Journal of Surgery, 2009

Poland’s Syndrome is a rare congenital condition. It is classically characterized by absence of unilateral chest wall muscles and sometimes ipsilateral symbrachydactyly (abnormally short and webbed fingers). The condition typically presents with unilateral absence of the sternal or breastbone portion of the pectoralis major muscle which may or may not be associated with the absence of nearby musculoskeletal structures. We report a 12-year-old boy patient with typical features of Poland syndrome. To the best of our knowledge, this is the first documented case of a patient with Poland syndrome reported from Rwanda.

Poland Syndrome- A Rare Congenital Condition

Journal of Bangladesh College of Physicians and Surgeons, 2018

Named after Sir Alfred Poland, Poland syndrome is a rare congenital anomaly classically characterized by the absence of unilateral chest wall muscles and sometimes ipsilateral symbrachydactyly (abnormally short and webbed fingers). The aetiology is probably a vascular disruption sequence of the subclavian arteries. In most cases, Poland Syndrome is sporadic. We report a 26 year old male patient with typical features of Poland Syndrome associated with bronchiectasis and mitral valve prolapse- a very rare association. To the best of our knowledge, this is the first documented case of a Poland Syndrome with rare association reported from Bangladesh.J Bangladesh Coll Phys Surg 2018; 36(4): 166-169

Case Report Poland Syndrome Case Study

Poland syndrome describes the congenital absence of the pectoralis major and minor muscles on one side of the body accompanied by chest and upper extremity deformities on the same side. However, the only constant finding of the syndrome is the absence of the sternocostal component of the pectoralis major muscle. This finding may be accompanied by additional findings on the same side such as absence of pectoralis minor muscle and of ribs 2 to 5, under-development of breast tissue, latissimus dorsi, serratus anterior and external oblique muscles. The incidence is approximately 1 in 30 000 live births and is more common in men than in women. It mostly holds the right hemithorax of the body. Here we present a 20-year-old male patient without complaints, diseases or other functional anomalies, who has hypoplasia of the right hemithorax, palpation indicating hypoplasia of the pectoral muscle group, and thoracic CT is showing a lack of pectoral muscles.

Poland syndrome case study

Poland syndrome: a case report

International journal of scientific reports, 2024

History-Alfred Poland, a student demonstrator in anatomy, described his case as similar to Poland syndrome in the year 1841; however, there were earlier reports of this anomaly by Lallemandin in 1826 and Froriep in 1839. The full anatomical spectrum of this syndrome was first summarised by Thompson in 1895. In 1900, Furst suggested a common aetiology. In 1962, Clarkson named it as Poland's syndactyly after more than 300 patients had been reported. Epidemiology The reported incidence of Poland's syndrome is 1 in 7,000 to 1 in 100,000. Males are more frequently affected by a ratio of 2:1 to 3:1. In 1 of every 19,000 mammograms Poland syndrome was diagnosed. The right side of the body was found to be involved in 60% to 75% of patients. Vascular factors Vascular disruption theory is the most widely used theory for Poland syndrome. This theory postulates that deficient blood flow in the proximal subclavian artery and its branches during the 6th week of gestation leads to regional tissue loss. Intrinsic factors, such as thrombi or emboli, and external mechanical factors, such as cervical ribs, aberrant muscles, and amniotic bands, can contribute to this disruption of blood flow to the subclavian artery and its branches. Maternal factors, such as smoking and ABSTRACT Poland syndrome is characterized by the absence or hypoplasia of other chest muscles: small pectoral muscle, anterior tightened muscle, grandorsal muscle, and deltoid muscle. Anomalies of the thoracic cage: agenesis or hypoplasia of one or more costal segments, pectus carinatum, excavatum, clavicular hypoplasia, pulmonary herniation. Abnormalities of the mammary region: agenesis or hypoplasia of the breast, areola and nipple. The reported incidence of Poland's syndrome ranges from 1 in 7,000 to 1 in 100,000. Males are affected more frequently by a 2:1 to 3:1 ratio Poland's syndrome has also been diagnosed in 1 of 19,000 mammograms. The right side of the body was found to be involved in 60% to 75% of patients-associated malformations: genitourinary malformations, cardiac malformation, hepatic and biliary tract malformations. Case report Our case involves a 7-month-old boy with no medical history of cardiac or respiratory complaints. This is a male child with around 65cm height, and 7kg weight. There is no family history of consanguineous marriage and no family history of congenital anomalies. All siblings are alive and healthy. The child's milestones are within normal limits. There is aplasia of the right sternocostal head and clavicular heads and abdominal head of the pectoralis major muscle, there is a hypoplasia of the right nipple and breast and absence of subcutaneous fat on the same side. Ribs are visible on the right chest wall. Physical examination shows no history of hand and digit anomaly and no rib anomalies. Chest wall asymmetry with right chest wall showing depression on axillary floor. The anterior axillary fold is absent. On attempting abduction of the shoulder, it shows the absence of the sternocostal head of the pectoralis major.

Poland’s syndrome revisited

The Annals of Thoracic Surgery, 2002

Poland's syndrome is a rare congenital anomaly characterized by unilateral chest wall hypoplasia and ipsilateral hand abnormalities. Literary data suggest its sporadic nature. The prevailing theory of its cause is hypoplasia of the subclavian artery or its branches, which may lead to a range of developmental changes. The incidence of Poland's syndrome varies between groups (male versus female patients, congenital versus familial cases, and so on) and ranges from 1 in 7,000 to 1 in 100,000 live births. Cases of Poland's syndrome associated with leukemia, carcinoma of the hypoplastic breast, and other conditions, confirm the relationship between developmental defects and tumors, and require oncologic awareness. Various manifestations, age, and gender require different surgical approaches. Our experience, which includes 27 patients (15 male, 12 female), 20 of whom (12 male, 8 female) underwent operation, suggests that the repair should be done in two stages in children and in a single stage in adults. Reconstruction and/or stabilization of the aplastic ribs may be achieved using bone grafts or prosthetic mesh. Muscle flaps and breast implants may be used to correct muscle deficiency and breast hypoplasia and to help achieve a complete cosmetic repair.

POLAND ANOMALY, A SPORADIC SYNDROME: A REVIEW

Poland Anomaly, one of the sporadic congenital disease involves the congenital unilateral absence or underdevelopment of Pectoralis major muscle with ipsilateral cutaneous syndactyly. It is thought to be due to Subclavian Artery Supply Disruption Sequence. There are evidences which suggest it due to genetic factors or teratogenic. The child has structural abnormalities involving absence or underdevelopment of muscles, or bones like ribs. This is also associated with malignancies, dextrocardia, atrial septal defects, renal impairment, ophthalmic abnormalities. These are diagnosed by CT, MRI and X-Ray along with thorough clinical examination. The chest reconstruction therapy is the treatment available for the Poland Anomaly. Keywords: Poland Anomaly, Congenital sporadic syndrome, Subclavian Artery Supply Disruption Sequence

A case of Poland Syndrome associated with dextroposition

Italian Journal of Pediatrics, 2010

Classical Poland Syndrome (PS) is characterized by unilateral, partial or complete absence of the sternocostal head of the major pectoral muscle and brachysyndactyly of fingers on the same side. We report the case of a newborn infant with dextrocardia and PS located on the left side. This association is very rare: to date only 19 cases have been described in scientific literature. In all reported cases, as in the present, the Poland defect involved the left side and was associated to rib defects, whereas most cases of PS are on the right side and few have rib defects. This case supports the view that dextrocardia follows the loss of volume of the left hemithorax caused by Poland sequence and that the combination of PS and dextrocardia is not coincidental.

Controversies in Poland Syndrome: Alternative Diagnoses in Patients With Congenital Pectoral Muscle Deficiency

The Journal of Hand Surgery

Purpose Poland syndrome was first described as a deficiency of the pectoral muscle with ipsilateral symbrachydactyly. Currently, numerous case reports describe variations of Poland syndrome in which pectoral muscle deficiency is often used as the only defining criterion. However, more syndromes can present with pectoral muscle deficiency. The aim of this review is to illustrate the diversity of the phenotypic spectrum of Poland syndrome and to create more awareness for alternative diagnoses in pectoral muscle deficiency. Methods A systematic literature search was performed. Articles containing phenotypical descriptions of Poland syndrome were included. Data extraction included number of patients, sex, familial occurrence, and the definition of Poland syndrome used. In addition, hand deformities, thoracic deformities, and other deformities in each patient were recorded. Alternative syndrome diagnoses were identified in patients with a combination of hand, thorax, and other deformities. Results One hundred-and-thirty-six articles were included, describing 627 patients. Ten different definitions of Poland syndrome were utilized. In 58% of the cases, an upper extremity deformity was found and 43% of the cases had an associated deformity. Classic Poland syndrome was seen in 29%. Fifty-seven percent of the patients with a pectoral malformation, a hand malformation, and another deformity had at least 1feature that matched an alternative syndrome. Conclusions Pectoral muscle hypoplasia is not distinctive for Poland syndrome alone but is also present in syndromes with other associated anomalies with a recognized genetic cause. Therefore, in patients with an atypical phenotype, we recommend considering other diagnoses and/or syndromes before diagnosing a patient with Poland syndrome. This can prevent diagnostic and prognostic errors. Clinical relevance Differentiating Poland syndrome from the alternative diagnoses has serious consequences for the patient and their family in terms of inheritance and possible related anomalies.

A Rare Case of Poland's Syndrome (original) (raw)

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