A Rare Chest Wall Deformity; Poland Syndrome: Case Report (original) (raw)
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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 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
Poland syndrome: A proposed classification system and perspectives on diagnosis and treatment
Seminars in Pediatric Surgery, 2018
Poland Syndrome (PS) is a rare condition, with an estimated incidence of approximately 1 per 30,000 births and encompasses a wide range of severities of chest and upper arm anomalies. The etiology remains unknown, but genetic involvement is suspected. Few radiological investigations have proven useful in the study PS phenotypes and we propose a reference algorithm for guiding pediatricians. Our experience with 245 PS patients in the last 10 years stimulated a phenotypical classification of PS. The management of the different PS types and a therapeutic algorithm according to the phenotypical features of each PS patient are also proposed.
Poland’s Syndrome: A Case Report
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.
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.
Clinical and Radiographic Poland Syndrome Classification: A Proposal
Aesthetic Surgery Journal, 2009
P oland syndrome (PS) is a rare abnormality with a sporadic presentation characterized by congenital malformations of the chest wall, with or without alterations to the ipsilateral superior limbs and hands. 1,2 Classically, it consists of a combination of unilateral aplasia of the sternocostal portion of the pectoralis major muscle (PMM) and hypoplasia of the ipsilateral hand, with syndactyly 3,4 and synbrachydactyly. 5 Only 400 cases of PS were reported by 1990; in these cases, several degrees of chest wall malformations extending to the superior limbs were seen. 1 The incidence of PS has a male-to-female ratio of 3:1 and its frequency is estimated at one in 30,000 live births, 6-11 with the right side being affected twice as often as the left side. 12,13 The etiology of PS is still unknown. Several studies have suggested that a genetic factor or, even more likely, extrinsic factors between the sixth and eighth weeks of pregnancy may interfere with PMM migration and the separation of the digits (chirodactile) that occurs in this period. 6-12 The recent vascular hypothesis for the etiology of PS implies that hypoplasia of the ipsilateral subclavian artery is the origin of this birth anomaly. 13-16 The main deformities associated with PS are as follows: (1) Muscles-Absence of the pectoralis minor muscle (PMM), absence of the clavicular and sternal segments of the PMM, 17 and hypoplasia of the serratus anterior, 18,19 latissimus dorsi, deltoid, and infraspinatus and supraspinatus muscles. 20 (2) Mammary glands-Areolar abnormalities, mammary absences, or hypoplasia. 17,18-21 Background: Many chest wall deformities have a characteristic radiologic appearance that can be the basis for a definitive diagnosis. Consequently, imaging techniques have fundamental roles in the detection, location, and characterization of these disorders. Objective: The authors propose a clinical and radiographic Poland syndrome (CRPS) classification system and possible treatment algorithm for the thoracic manifestations of Poland syndrome (PS) in women, based on both clinical examinations and imaging studies. Methods: A retrospective study was conducted of 28 female patients evaluated over 17 years in the 28th Infirmary, Plastic and Reconstructive Surgery Division of the Hospital Santa Casa da Misericórdia do Rio de Janeiro, Rio de Janeiro, Brazil. After clinical examination, all patients underwent radiographic examination with chest radiographs, conventional computed tomography scans, magnetic resonance imaging and, in some cases, additional imaging studies. All clinical and radiologic variables were compiled in a database and used in the classification system, which included three levels of disease severity. Results: Based on the CRPS classification of the 28 female patients, 10 patients had first-degree PS, 14 patients had second-degree PS, and four patients had third-degree PS. Eighteen patients underwent surgical correction; a total of 39 surgical procedures were performed using the CRPS algorithm. Conclusions: Identification of the severity of PS using the proposed classification system provided an accurate study of each patient and enabled better planning for the surgical correction of functional and aesthetic deformities.
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.
Current Thoracic Surgery
Background: The main subtypes of chest wall deformities are pectus excavatum (PE) and pectus carinatum (PC). Although these are not life threatening diseases, some functional or physico-social disabilities due to the abnormal chest wall structure require treatment. The main stem of the treatment is surgical correction. Since the beginning of the 20 th century various surgical techniques were introduced. In the last few decades some minimally invasive surgical techniques were also described. In the English literature many reports suggest that the vast majority of these abnormalities are PE. But in most of the reports patient groups consist of hospital admissions. The real frequency of this group of disease is controversial. Materials and Methods: In this report, a wide field study was designed to find the real frequency of the chest wall diseases. Total of 25117 children between 6-11 years of age were visited in the elementary schools of Kahramanmaraş. The team members were a thoracic surgeon, a pulmonologist and a pediatrician. Results: A careful physical examination revealed that a total of 255 patients had different degrees of chest wall deformity. We found that PC (90.58%) was the most frequent type of deformity in contrast with the previous reports suggesting PE as the most frequent type. In our results only 5.49% of the patients had PE and 3.93% of the patients had mixed type PE+PC. The most frequent ECG abnormality seen in 49 cases (19% of cases with deformity) was a negative T wave on V1 derivation and a biphasic T wave configuration on V1-V2 derivations. 8 patients (3.13%) had concomitant scoliosis and 6 patients (%2.35) had different degrees of cardiomegaly. Conclusions: The real frequency of chest wall deformities is an enigma. When PE patients themselves or their family notice the abnormality, they may consider it as a big health problem and admit to a healthcare unit. But in contrast, PC patients mostly do not care about this disorder until adolescence, so admission rate is less then PE. We suggest that the real frequency of the chest wall deformities may be found only by widespread field studies. Moreover, we found more accompanying cardiac disorders in PC group, and this type of screening may allow early diagnosis of some cardiac diseases.
Poland Syndrome Presenting with Failure to Thrive: A Case Report
Poland Syndrome is a rare congenital anatomic anomaly disorder characterized by ipsilateral breast and nipple hypoplasia and/or aplasia, deficiency of subcutaneous fat and axillary hair, absence of the sternal head of the pectoralis major muscle, hypoplasia of the rib cage, and hypoplasia of the upper extremity. I present the case of a 3-month-old male infant who exhibited symptoms of Poland syndrome, had no family history of the condition, and presented with a right upper chest deformity, including bulging of the lung with breathing, and failure to thrive.