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BMC Anesthesiology May 2018Poland syndrome is a rare congenital disease, characterized by agenesis/hypoplasia of the pectoralis major muscle, usually associated with variable thoracic anomalies... (Review)
Review
BACKGROUND
Poland 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 PRESENTATION
Here, 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.
CONCLUSIONS
Examinations before surgery, intraoperative monitoring, choice of general anesthetics and pain management after surgery should all be contemplated.
Topics: Anesthesia; Anesthetics, Intravenous; Child, Preschool; Humans; Male; Midazolam; Neuromuscular Nondepolarizing Agents; Poland Syndrome; Propofol; Rocuronium; Sufentanil; Thoracic Wall
PubMed: 29793431
DOI: 10.1186/s12871-018-0518-4 -
Hand (New York, N.Y.) Jul 2022Poland syndrome (PS) is a congenital condition characterized by pectoralis hypoplasia and an ipsilateral hand anomaly that frequently necessitates surgery in childhood....
BACKGROUND
Poland syndrome (PS) is a congenital condition characterized by pectoralis hypoplasia and an ipsilateral hand anomaly that frequently necessitates surgery in childhood. This study aimed to determine long-term functional and psychosocial implications of PS.
METHODS
Patients underwent strength testing of their affected limb by comparing with their contralateral arm. They completed Disabilities of the Arm, Shoulder, and Hand (DASH), 36-item Short-Form Health Survey (SF-36), and several Patient-Reported Outcome Measurement Information System (PROMIS) surveys. Aggregate scores were compared with population norms.
RESULTS
Twenty-eight patients were enrolled, and 16 returned for strength testing. The average age was 42 years (range, 18-65 years), and the average follow-up was 25 years (range, 1-52 years). Dynamometer testing showed decreased strength in shoulder internal rotation and abduction/adduction, hand grip, and key pinch/tip pinch. Average DASH and SF-36 scores were comparable with population norms. The PROMIS Upper Extremity score (46.2) was significantly lower than the average 50 (SD, 10.0); the Social Roles score (57.3) indicated significantly less disability than the general population. Seventy-nine percent reported higher than average satisfaction with life, 82% had normal self-esteem, and 21% and 4% met screening criteria for depression and anxiety, respectively. The Derriford Appearance Scale revealed that 68% of patients identified PS as their most self-conscious feature, with 36% reporting subsequent life adjustments.
CONCLUSIONS
This cohort of PS patients self-reported more favorable functional, psychological, and emotional outcomes than expected based on significant shoulder and hand strength deficits. Despite these deficits, patients make adaptations, allowing fulfilling lives with psychosocial functioning similar to their peers.
Topics: Adult; Disability Evaluation; Hand Strength; Humans; Patient Reported Outcome Measures; Poland Syndrome; Upper Extremity
PubMed: 34002631
DOI: 10.1177/1558944720963864 -
Acta Chirurgiae Plasticae 2000The author summarizes hitherto assembled experience with the clinical and genetic characteristics of Poland's and Möbius syndrome. Five selected case-records with this... (Review)
Review
The author summarizes hitherto assembled experience with the clinical and genetic characteristics of Poland's and Möbius syndrome. Five selected case-records with this disease and the sequence of the Poland-Möbius 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 Möbius 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.
Topics: Adult; Child; Child, Preschool; Female; Humans; Male; Mobius Syndrome; Phenotype; Poland Syndrome
PubMed: 11059047
DOI: No ID Found -
Aesthetic Plastic Surgery 2005Poland syndrome comprises a unilateral absence of the large pectoral muscle, ipsilateral symbrachydactyly, and occasionally other malformations of the anterior chest... (Review)
Review
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.
Topics: Adolescent; Breast; Diagnosis, Differential; Female; Humans; Male; Pectoralis Muscles; Poland Syndrome
PubMed: 15759092
DOI: 10.1007/s00266-004-0047-z -
The Breast Journal 2011Poland's syndrome is a rare congenital development malformation characterized by unilateral chest wall hypoplasia and ipsilateral hand abnormalities. It is also known to... (Review)
Review
Poland's syndrome is a rare congenital development malformation characterized by unilateral chest wall hypoplasia and ipsilateral hand abnormalities. It is also known to be associated with some malignant diseases. We herein report a case of Poland's syndrome associated with invasive ductal carcinoma of breast, and review the literatures to investigate the clinical characteristics of breast cancer with Poland's syndrome.
Topics: Adult; Breast Neoplasms; Carcinoma, Ductal; Female; Humans; Lymphatic Metastasis; Mammography; Mastectomy, Modified Radical; Neoplasm Invasiveness; Poland Syndrome; Ultrasonography
PubMed: 21410586
DOI: 10.1111/j.1524-4741.2010.01042.x -
Hand (New York, N.Y.) Dec 2016Poland anomaly is a sporadic, phenotypically variable congenital condition usually characterized by unilateral pectoral muscle agenesis and ipsilateral hand deformity.... (Review)
Review
Poland anomaly is a sporadic, phenotypically variable congenital condition usually characterized by unilateral pectoral muscle agenesis and ipsilateral hand deformity. A comprehensive review of the medical literature on Poland anomaly was performed using a Medline search. Poland anomaly is a sporadic, phenotypically variable congenital condition usually characterized by unilateral, simple syndactyly with ipsilateral limb hypoplasia and pectoralis muscle agenesis. Operative management of syndactyly in Poland anomaly is determined by the severity of hand involvement and the resulting anatomical dysfunction. Syndactyly reconstruction is recommended in all but the mildest cases because most patients with Poland anomaly have notable brachydactyly, and digital separation can improve functional length. Improved understanding the etiology and presentation of Poland anomaly can improve clinician recognition and management of this rare congenital condition.
Topics: Brachydactyly; Hand Deformities, Congenital; Humans; Pectoralis Muscles; Phenotype; Poland Syndrome; Syndactyly
PubMed: 28149203
DOI: 10.1177/1558944716647355 -
The Israel Medical Association Journal... Jul 2023
Topics: Male; Humans; Adolescent; Gynecomastia; Poland Syndrome
PubMed: 37461178
DOI: No ID Found -
Annales de Chirurgie Plastique Et... Feb 2011Correction of chest and breast abnormalities in Poland's syndrome differs among authors. The aim of this study is to discuss surgical protocols according to the grade of... (Review)
Review
INTRODUCTION
Correction of chest and breast abnormalities in Poland's syndrome differs among authors. The aim of this study is to discuss surgical protocols according to the grade of Poland's syndrome in women.
PATIENTS AND METHODS
The authors present a series of 11 women with Poland's syndrome among which nine were operated. Several surgical techniques were used. Cosmetic results were analysed according to the treatment and grade. Median follow up is 9 years.
RESULTS
In grade I, a round breast implant seems to be the best choice. In grade II, a latissimus dorsi flap combined with a breast implant gives better results than a breast implant alone. In grade III, breast and chest reconstruction are linked. The authors present a case of costal reconstruction followed by an expansion without a latissimus flap.
CONCLUSION
Many authors recommend a latissimus flap combined with a breast implant in severe grades of Poland's syndrome. Using this flap is not always possible nor mandatory. According to soft tissue quality, expansion may be an alternative way or a temporary solution during growth.
Topics: Adolescent; Adult; Child; Female; Humans; Middle Aged; Poland Syndrome; Retrospective Studies; Severity of Illness Index; Young Adult
PubMed: 21292378
DOI: 10.1016/j.anplas.2010.10.016 -
Akusherstvo I Ginekologiia 2007Poland's Syndrome is a rare congenital abnormality involving functional and aesthetic impairments. Constitutive mark of the condition: partial or total absence of the...
UNLABELLED
Poland's Syndrome is a rare congenital abnormality involving functional and aesthetic impairments. Constitutive mark of the condition: partial or total absence of the pectoralis major and minor muscles, symmetric chest wall deformity, brachydactyly/syndactyly. This syndrome is occasionally associated with either unilateral or bilateral hypoplasia of breast.
AIM
We present a case of a patient with Poland's Syndrome. This association has not been previously reported in the medical literature. Difficulties by preoperative diagnosis. The purpose of this article--to propose a classification system for the treatment of this anomaly requiring defining the age of the patients for plastic surgery on the chest.
METHODS AND MATERIALS
A 16-year-old female with Poland's Syndrome complained of drastic mood changes consisting of depression and anxiety, regular menses, dysmenorrhea and hypomastia. Two reconstructive surgeries at the age of 12 years--surgical corrections of the sternal malformation.
RESULTS AND CONCLUSION
This work shall describe Poland's Syndrome from a clinical, diagnostic, genetic and therapeutic point of view. The Italian Association for Poland Syndrome founded in October 2003 and two nationwide scientific conferences dealt with the position of scientific research and future prospects with regards to Poland Syndrome. Patients are subject to multi-specialist (surgical, gynecological, genetic, psychological, orthopaedic and plastic surgical) checkups, aiming at an accurate genetic and clinical organisation of a therapeutic programme.
Topics: Adolescent; Breast; Female; Humans; Poland Syndrome; Treatment Outcome
PubMed: 18173004
DOI: No ID Found -
Thoracic Surgery Clinics Nov 2010Defects of the thoracic cage with bone and/or muscle deficit are relatively rare and can present a real risk depending on the severity of manifestations. Cleft sternum... (Review)
Review
Defects of the thoracic cage with bone and/or muscle deficit are relatively rare and can present a real risk depending on the severity of manifestations. Cleft sternum results from failed midline fusion of the sternal halves that leaves the heart and great vessels unprotected, and is commonly associated with craniofacial hemangiomas. Correction is recommended during the neonatal period when compliant thorax allows direct suturing of the divided sternum. Sternal foramen requires precaution during biopsy and acupuncture as well as forensic awareness. In addition to the thoracic defect, Poland syndrome can be associated with hand anomalies, dextrocardia, renal agenesia, and various tumors. Age and gender, together with the degree of the defect, define the method of surgical correction.
Topics: Humans; Musculoskeletal Abnormalities; Orthopedic Procedures; Poland Syndrome; Sternum; Tomography, X-Ray Computed
PubMed: 20974442
DOI: 10.1016/j.thorsurg.2010.06.001