Cases reported "Poland Syndrome"

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1/102. lower extremity counterpart of the poland syndrome.

    Below-the-knee right leg hypoplasia and ipsilateral toe brachysyndactyly were observed in a 4-year-old female with an otherwise normal phenotype. Electromyographic and nerve conduction studies were normal. The Doppler evaluation was consistent with a 50% reduction in the blood supply from the femoral artery, suggesting vascular disruption as the pathogenic mechanism. Our observations support the hypothesis that a lower extremity counterpart of the poland syndrome does exist and that the extent of limb involvement is dependent on the level of vascular disruption. ( info)

2/102. The occurrence of Poland and Poland-Moebius syndromes in the same family: further evidence of their genetic component.

    The combination of Moebius and Poland anomalies is rarely described in the literature. Some authors believe that this association is an independent syndrome, while others propose that Poland, Moebius and Poland-Moebius are a spectrum of the same condition. Here we report a family where a child has Poland-Moebius syndrome and an aunt is affected with Poland anomaly. This probably represents the second report of possible autosomal dominant transmission of Poland-Moebius syndrome. The presence of contralateral triphalangeal thumb in the proband and in other members of the family can be explained by chance alone or, alternatively, by a pleiotropic expression of this gene. ( info)

3/102. poland syndrome: a case with a combination of syndromes.

    The case of poland syndrome reported incorporates a number of previously unrecorded features: hypoplasia of the left lung and agenesia of the epiglottis in addition to the standard symptoms. The case also presents certain features typical of the Moebius Syndrome. ( info)

4/102. breast cancer in poland syndrome.

    A 33-year-old African-American woman with a severe manifestation of poland syndrome developed breast cancer in the ipsilateral breast. She had a severely hypoplastic upper extremity, including symbrachydactyly, and a hypoplastic forearm and upper arm. In addition, she lacked the sternal origin of the pectoralis muscle. She had a very small nipple-areola complex and no axillary hair. This is the first case report of breast cancer developing in the ipsilateral breast of a patient with poland syndrome. ( info)

5/102. Poland sequence: two unusual cases and brief review of the literature.

    Poland Sequence consists of a congenital absence of the pectoralis major muscle. Classically, the sequence also includes ipsilateral hand anomalies. Identified patients may be screened for renal and other anomalies. The condition results in physical and cosmetic disabilities which can be treated if properly triaged. The chest wall anatomy may be delineated with computed tomography and MRI if surgery is planned. Two cases with unusual findings including lumbosacral spine malformation and contralateral hand syndactyly are presented. ( info)

6/102. Microneurovascular transfer of contralateral latissimus dorsi in Poland's syndrome.

    A 2-year-old girl with Poland's syndrome presented with syndactyly of the first, second and third webs of the left hand and ipsilateral absence of the sternocostal portion of pectoralis major. Latissimus dorsi on the same side was also absent. A microvascular free contralateral latissimus dorsi muscle transfer was undertaken to reconstruct the chest wall and anterior axillary fold. The transfer was innervated by intercostal nerve transfer and produced a neuromuscular unit of good aesthetic appearance and function. ( info)

7/102. Poland's syndrome in one identical twin.

    female twins were evaluated at the Shriners Hospital in Lexington, kentucky. One twin was normal, and the other twin had the classic findings of Poland's syndrome, manifested by absence of the pectoralis major and symbrachydactyly of the right upper extremity. Buccal smears from each child were submitted for dna testing. The test confirmed monozygosity with 99.9% probability. Some previous reports have stated that Poland's syndrome is an autosomal dominant, genetically determined trait, whereas others have maintained that there is no genetic association. The original case described by Poland in 1841 was his cadaver, and no family history was reported. This twin study provides strong evidence that the condition is not determined by gene transmission. ( info)

8/102. Poland's syndrome and recessive X-linked ichthyosis in two brothers.

    Poland's syndrome consists of unilateral congenital absence of the pectoralis major muscle with a variable degree of ipsilateral upper limb deformity. The aetiology of Poland's syndrome is unknown, although an inherited tendency to develop a compromised embryonic vascular supply in the affected areas has been suggested. The majority of reported cases are sporadic, and in only a few instances is there a familial incidence. We describe the occurrence of Poland's syndrome in two brothers who also had recessive X-linked ichthyosis. To the best of our knowledge this is the first report of such an association. ( info)

9/102. Poland's syndrome with lung cancer. A case report.

    Poland's syndrome, a rare congenital anomaly characterized by pectoralis muscle defect, has been reported in association with lymphoreticular malignancies and some solid tumors. lung cancer associated with Poland's syndrome has not been previously described. We present the first report of a case of Poland's syndrome associated with lung cancer and demonstrate the CT findings. ( info)

10/102. Poland-mobius syndrome and disruption spectrum affecting the face and extremities: a review paper and presentation of five cases.

    The author summarizes hitherto assembled experience with the clinical and genetic characteristics of Poland's and mobius syndrome. Five selected case-records with this disease and the sequence of the Poland-mobius syndrome are presented. Another case-record is devoted to an allied syndrome, hypoglossia-hypodactyly, found in a spontaneously aborted fetus. For establishment of a more accurate symptomatology, an irreplaceable place is held by anthropometric examination; for objectifying the asymmetry of the chest the so-called cyrtogram, the chest circumference recorded by means of a wire, is valuable. From the aspect of genetic counseling, preconception care is always provided to mothers from families with reproductive intentions, as well as ultrasonographic examination of the fetus in areas of assumed acral symptomatology (signaling phenotype). In two families ultrasonography was used for prenatal diagnosis. Invasive prenatal diagnosis by amniocentesis was employed in a family with mobius syndrome. In these families dermatoglyphs have certain common characteristics, such a tendency towards simple patterns. In the wider family of one of our patients we detected in a cousin Parkes-Weber-Klippel-Trenaunay's syndrome, which may indicate common vascular predisposing factors. ( info)
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