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At least 19 recordsLinked to original sources

Endoscopically assisted reconstruction of male and female poland syndrome.

Poland syndrome produces deformities of the breast and chest wall that can be highly disfiguring in both men and women. Incisions from traditional surgical approaches can be unsightly, especially if a muscle harvest is used as part of the reconstruction. Herein is described a case series in which minimally invasive techniques were used to reconstruct defects in male and female patients with Poland syndrome. When evaluating patients with Poland syndrome, one should consider using minimally invasive techniques as an alternative reconstructive option.

Adolescent↗

Temporary dysplastic hematological features due to iron deficiency in a case of Poland syndrome.

Poland syndrome has been sporadically associated with hematological conditions, primarily acute leukemias. The authors report a child with this syndrome coexisting with temporary hematological dysplasia due to iron deficiency. Further hematological evaluation failed to demonstrate malignancy. Because of its premalignant nature, in patients with Poland syndrome, hematological dysplastic features should be an indication for further investigation. However, in addition to the well-known hematological malignancies, treatable causes may be responsible.

Blood Cells↗

[Early surgery in Poland syndrome].

Poland's congenital malformation presents a variable grade of complexity, depending upon the extent of the muscular and chondro-costal defect. Surgical repair for cosmetic reasons only, may be performed during childhood or puberty due to the absence of symptoms. However, this does not occur when the costal defects has a considerable size, with pulmonary herniation and impairment of respiratory function. In this cases, early surgical correction is preferred in order to adequately stabilize the chest wall. In this paper we present our experience of early surgical treatment in 5 patients affected by a complex syndrome, that were treated with autologous costal transplants and the use pof polytetrafluoroethylene to cover the chest wall defect. We discuss the surgical procedure performed, as well as the advantages of this material with respect to others described up to date and the good results obtained in one of the cases followed-up for five years.

Child, Preschool↗

Prenatal ultrasound diagnosis of poland syndrome.

BACKGROUND: Poland syndrome is a congenital nongenetic anomaly characterized by unilateral chest wall hypoplasia, ipsilateral hand abnormalities, and hemivertebrae. It has not ben described so far in the fetus. CASE: The patient was referred for suspected left-arm hypomelia at 22 weeks of gestation. On ultrasonography, we confirmed the presence of severe left-sided hypomelia and detected an asymmetry of the rib cage and 3 thoracic hemivertebrae. The absence of heart defects led us to make the putative diagnosis of Poland syndrome. After termination of pregnancy, the diagnosis was confirmed by the pathologist. CONCLUSION: The possibility of diagnosing Poland syndrome in utero is important for proper management and counseling. If the syndrome is suspected in a fetus, counselors may refer to specific postnatal data to provide the couple with survival rates, treatment options, and results and morbidity figures. If the pregnancy is terminated, a detailed necropsy is warranted to confirm the diagnosis because familial transmission has been reported.

Adult↗

Parry-Romberg syndrome in association with contralateral Poland syndrome.

A 35-year-old woman had a 4-year history of skin depression on the right side of both the forehead and chin. A biopsy specimen showed a normal dermis and an absence of subcutaneous tissue. She also had Poland syndrome on the left side of the body, with loss of the sternocostal portion of the greater pectoral muscle. Significant cosmetic improvement was achieved with dermal fat grafts and silicone implants.

Adipose Tissue↗

[Poland syndrome (a case report)].

Poland syndrome is characterized with unilateral absence of pectoralis major muscle. Its incidence is one in 30000 live births. A 20 years old case with Poland syndrome is presented together with its clinical and laboratory features in this study. The case had anomaly of shortness of right hand fingers and syndactily between second and third fingers in addition to absence of right pectoralis muscle group. There was not another associated anomaly except aforementioned ones. Strength loss in abduction and adduction of right shoulder was detected with Cybex dynamometer. Furthermore decrease in predicted maximal inspiratory and expiratory pressures was detected.

Adult↗

MR and CT in the assessment of Poland syndrome.

Seven cases of Poland syndrome were investigated with CT and/or MR to assess the extent of the muscle abnormalities of the shoulder girdle. In all cases the absence of the sternocostal head of the pectoralis major was clearly shown, as were any associated abnormalities of the pectoralis minor, serratus anterior, and latissimus dorsi. The latissimus dorsi muscle has an important role in reconstructive surgery and can be difficult to assess clinically. Cross-sectional imaging with CT or MR will give useful information in patients with Poland syndrome requesting anterior axillary reconstruction and breast mound formation.

Adolescent↗

Poland syndrome associated with renal agenesis.

Poland syndrome is characterized by unilateral aplasia or hypoplasia of the sternocostal portion of the pectoralis major muscle and ipsilateral syndactyly. In some cases other associated anomalies, including renal malformations, dextrocardia, and vertebral abnormalities, have been reported. We report a 7-month-old girl with Poland syndrome who also presented with ipsilateral renal agenesis. This report suggests that renal structural anomaly may be an integral part of this syndrome. We recommend renal imaging studies be performed on all children with Poland syndrome.

Female↗

The middle phalanx in Poland syndrome.

The classic deformity of Poland syndrome consists of the combination of unilateral aplasia of the sternocostal head of the pectoralis major muscle and an ipsilateral hypoplastic hand with simple syndactyly and short fingers. The current study specifically investigates the abnormalities of the middle phalanx in a series of 15 patients with Poland syndrome. The degree of hand hypoplasia and finger brachydactyly was classified into 3 grades: mild (n = 2), moderate (n = 10), and severe (n = 3). The middle phalanges of all fingers in all patients showed abnormalities. The degree of deficiency in the middle phalanx (from mild hypoplasia to aplasia) correlated with the degree of brachydactyly, which in turn correlated with the degree of hand hypoplasia. Several other radiologic features of the hypoplastic middle phalanges were documented, such as the central nidus, the rounded or truncated appearance, and the cone-shaped epiphyses. The embryology of limb development was reviewed and it was proposed that abnormalities of the middle phalanx in Poland syndrome occur as a result of a mild ischemic insult during stage 19 of the embryonic life, leading to an arrest or lack of chondrification of the mesenchymal mass of the phalanx.

Adolescent↗

[Anesthetic management of patients with Poland syndrome: report of two cases].

Poland syndrome is a rare anomaly characterized by unilateral absence of the pectoral muscles and ipsilateral syndactyly and microdactyly. Associated anomalies include defect of ipsilateral ribs and costal cartilages. We report two cases of Poland syndrome. The first case, 24 year old female patient underwent mammoplasty. Although her pectoral muscles were absent, there was no rib or costal cartilage defect. General anesthesia was maintained with assisted spontaneous respiration through laryngeal mask airway. The second case, 18 year old male patient had rib cage defect. Although the patient was asymptomatic when he was awake, paradoxical respiratory movement could have caused inadequate ventilation during general anesthesia. Anesthesia was maintained with intermittent positive pressure ventilation through endotracheal tube during skin flap plasty of latissimus dorsi muscle. In both cases, there was no respiratory complication during perioperative period.

Adolescent↗

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.

Adult↗

Poland syndrome in a neonate: report of one case.

Poland syndrome is an infrequent disease that presents in newborn infants and features an incidence of about one in 7,000 to one in 100,000 live births. The clinical features include an absence of the costosternal portion of the pectoralis major muscle, hypoplasia and/or aplasia of the ipsilateral breast or nipple, hypoplasia of subcutaneous tissue, abnormalities of the rib cage and upper extremity anomalies. We present a male newborn suffering Poland syndrome who featured a depressed left chest wall with respiratory distress. Ultrasonography of the chest revealed the absence of a section of pectoralis major muscle over the left chest wall, although no abnormality was found over the ipsilateral upper extremity. The patient was regularly followed up and showed no limitation to the range of motion of the left upper extremity at the age of four months.

Humans↗

[Poland syndrome].

A case of Poland's syndrome in a newborn is described. The syndrome is relatively unknown, especially in its epidemiological and aetiopathogenetic aspects. It's characterized by aplasia of the sternal head of the pectoral major muscle, hypoplasia of the upper extremities and homolateral aplasia of II, III, IV, V finger. Attention is drawn to the importance of reporting diagnosed cases in order to further our knowledge of the syndrome. The Authors are looking with a big interest the correlation between hair treatment and Poland's anomaly.

Adult↗

Poland syndrome: description of an atypical variant.

Poland syndrome comprises a unilateral absence of the large pectoral muscle, ipsilateral symbrachydactyly, and occasionally other malformations of the anterior chest wall and breast. The condition is more frequent among males, and usually occurs on the right hemithorax in the unilateral form. The syndrome is believed to be caused by a genetic disorder that reduces the embryonal circulation in the interior chest artery: the stronger the interaction, the more severe the pathology. This article analyzes an unusual pathologic case in which the 17-year-old patient lacked the large pectoral muscle on the left side, but showed no arterial alteration. This case raises questions as to the true pathogenesis of this syndrome.

Adolescent↗

[Role of computerized tomography in Poland syndrome].

A morphological CT study in Poland syndrome is presented. The osteomuscular anomalies of the chest, which are not demonstrated by usual x-Ray, are well defined by CT scans. The usefulness of CT in postoperative controls of muscles transfers is stressed.

Adolescent↗

[A case of Poland syndrome].

A typical case of Poland syndrome is reported; brachiothoracic hemi-aplasia with absence of major pectoralis muscle, shortening of the arm and symbrachydactylia. Additionally, there was some evidence of debility. There were no malformations in the family history.

Abnormalities, Multiple↗