Secuencia de Poland, de las bases embriológicas a la práctica clínica / Poland sequence, going from the embryological bases to the clinical practice

La secuencia de Poland es un defecto congénito típicamente descrito como la ausencia congénita unilateral del músculo pectoral, que puede encontrarse asociado a la aparición de otras anomalías torácicas ipsilaterales y/o de extremidades superiores, las cuales abarcan diferentes grados de severidad funcional y de alteraciones estéticas. En la literatura se describen distintas asociaciones con diferentes síndromes, sin embargo su etiología es aún desconocida, aunque existen varias hipótesis sobre su posible causa, y es la más aceptada aquella que hace referencia a procesos de disrupción vascular. En la práctica clínica, las formas de expresión leve pueden ser ignoradas, pero en los casos más severos es requerida la atención de grupos multidisciplinarios de especialistas para su tratamiento. Se hace una revisión sistemática de la literatura sobre la secuencia de Poland.

Poland sequence is a birth defect typically described as unilateral congenital absence of the pectoralis muscle, which may be associated with the occurrence of other ipsilateral thoracic and/or upper extremities anomalies that cover different degrees of functional severity and esthetic alterations. The literature describes various types of association with different syndromes; however, its etiology remains unknown and several hypotheses about the possible cause of this disease exist, being the most accepted the one that deals with vascular disruption processes. In the clinical practice, mild forms of expression can be ignored, but in the most severe cases, the attention of a multidisciplinary group of specialists is required for disease management. A systematic literature review on Poland sequence was made.

Secuencia de Poland, de las bases embriológicas a la práctica clínica / Poland sequence, going from the embryological bases to the clinical practice

La secuencia de Poland es un defecto congénito típicamente descrito como la ausencia congénita unilateral del músculo pectoral, que puede encontrarse asociado a la aparición de otras anomalías torácicas ipsilaterales y/o de extremidades superiores, las cuales abarcan diferentes grados de severidad funcional y de alteraciones estéticas. En la literatura se describen distintas asociaciones con diferentes síndromes, sin embargo su etiología es aún desconocida, aunque existen varias hipótesis sobre su posible causa, y es la más aceptada aquella que hace referencia a procesos de disrupción vascular. En la práctica clínica, las formas de expresión leve pueden ser ignoradas, pero en los casos más severos es requerida la atención de grupos multidisciplinarios de especialistas para su tratamiento. Se hace una revisión sistemática de la literatura sobre la secuencia de Poland(AU)

Poland sequence is a birth defect typically described as unilateral congenital absence of the pectoralis muscle, which may be associated with the occurrence of other ipsilateral thoracic and/or upper extremities anomalies that cover different degrees of functional severity and esthetic alterations. The literature describes various types of association with different syndromes; however, its etiology remains unknown and several hypotheses about the possible cause of this disease exist, being the most accepted the one that deals with vascular disruption processes. In the clinical practice, mild forms of expression can be ignored, but in the most severe cases, the attention of a multidisciplinary group of specialists is required for disease management. A systematic literature review on Poland sequence was made(AU)

Poland's syndrome: a concise review of the clinical features highlighting associated dermatologic manifestations.

Poland's syndrome is a rare congenital condition characterized by absence of the pectoralis major muscle and a wide spectrum of associated ipsilateral chest wall and upper extremity anomalies. Associated dermatologic anomalies classically include pectoral and axillary alopecia, anhidrosis due to the absence of sweat glands, and deficiency in subcutaneous fat. Functional limitations are minimal, and thus surgical correction is primarily indicated for aesthetic purposes. Chest wall reconstruction typically involves transposition of a latissimus dorsi myocutaneous flap with or without a temporary subcutaneous tissue expander prior to surgical reconstruction. Using the PubMed database, a literature review was done on Poland's syndrome. We summarize the key features of Poland's syndrome, including the epidemiology, clinical presentation, pathogenesis, and management, and highlight the dermatologic associations reported in the literature.

[Chiari type I malformation in a patient with Poland's syndrome]. / Malformación de Chiari tipo I en un paciente con síndrome de Poland

Chiari malformation type I and Poland's syndrome are two rare diseases and their simultaneous presentation had not been previously described in the literature. We report the case of a 27 year old male with history of Poland's syndrome, who referred headache and motor impairment of the intrinsic muscles of the left hand. In a cervical spine MR a Chiari I malformation with syringomyelia from C1 to T2 was found, which was treated by foramen magnum decompression, dural plasty and removal of the posterior arch of the atlas. A discussion of the embryological mechanisms that might be involved in the coexistence of these two entities is presented, emphasizing the role of para-axial mesoderm.

[Poland-Möbius syndrome associated with lacrimal punctal and canalicular agenesis]. / Syndrome de Poland-Möbius associé à une agénésie des points et des canalicules lacrymaux.

We report the case of a 21-year-old patient who presented with epiphora in the left eye and a bilateral ocular motility problem. The clinical examination revealed bilateral moderate abduction limitation (impairment of the VIth cranial nerve), facial diplegia with amimic face (impairment of the VIIth cranial nerve), punctal agenesis of the left eye, absence of the left pectoralis major muscle, left breast aplasia, and hypoplasia of the left upper limb and hand. Neither the lacrimal puncta nor the canaliculi could be found during surgical exploration of left lacrimal system using operating microscope. Based on these findings, the patient was diagnosed with Poland-Möbius syndrome associated with punctal and canalicular agenesis. Poland-Möbius syndrome is a rare entity characterized by the association of two different syndromes: Poland syndrome and Möbius syndrome. Several abnormalities in association with this syndrome have been published. However, punctal and canalicular agenesis is not among these reported abnormalities.

Congenital and acquired pediatric breast anomalies: a review of 20 years' experience.

BACKGROUND: The purpose of this article was to review the senior author's 20 years of experience in the treatment of pediatric breast abnormalities, to propose a classification system for their treatment, and to provide a synopsis of treatment options. METHODS: Congenital and acquired breast anomalies were identified in a retrospective chart review (n = 66). Breast abnormalities were classified as hyperplastic (n = 44), deformational (n = 11), or hypoplastic (n = 11). Hyperplastic abnormalities included gynecomastia, hyperplasia, polythelia, polymastia, and giant fibroadenoma. Deformational abnormalities were categorized as either iatrogenic (previous thoracostomy, thoracotomy, or tumor excision) or traumatic (thermal or penetrating injuries). Hypoplastic abnormalities included athelia, unilateral and bilateral hypoplasia, tuberous breast, and Poland syndrome. Type of surgery, age at initial operation, and number of operations were recorded for all patients. RESULTS: Hyperplastic abnormalities were treated with breast reduction techniques and required the fewest operations per patient (1.14), followed by iatrogenic breast injury (2.1 per patient). The average number of procedures required for hypoplastic abnormalities was 2.45 per patient. The highest reoperation rates were seen in patients with burn injuries to the breast and patients with Poland syndrome. Mean age at initial operation was highest in the deformational group (18.5 years) and lowest in the hyperplastic group (17.4 years). CONCLUSION: Classification of pediatric breast abnormalities and considerations about timing for surgery and the likely need for staged operations aid in anticipating and optimizing clinical outcomes.

[Poland's syndrome]. / Zespól Polanda.

Poland's syndrome consists of the variable clinical features, but always includes unilateral aplasia of the chest wall muscles and ipsilateral anomalies of upper extremity. The incidence of Poland's syndrome, reported by different authors ranges from 1:10,000 to 1:100,000 and is observed more frequently in males than in females with the right side of the body affected more often than the left. The etiology of this syndrome is still discussed. However most of described cases were sporadic, rare familial incidence of Poland's syndrome were also presented. Therefore different etiologic factors of the Poland's syndrome are taken into account: genetic, vascular compromise during early stages of embriogenesis but also teratogenic effect of environmental xenobiotics (e.g. cigarette smoking by pregnant women). The authors present also the case of 20-years old man with inherited bilateral syndactyly with the right side aplasia of major pectoralis muscle and face asymmetry. The familial history was negative in respect to the features, associated with Poland's syndrome.

Poland's syndrome associated with chronic granulocytic leukemia.

Poland's syndrome has been sporadically associated with haematological neoplasms, namely acute lymphoblastic and myeloblastic leukaemias and non-Hodgkin's lymphomas. The authors present the case of a child in whom this syndrome coexists with a Philadelphia negative, chronic granulocytic leukaemia, which has only required one course of treatment with busulphan in two and a half years of follow-up.

A case report of Poland's syndrome from Indonesia.

Poland's syndrome consists of ipsilateral aplasia of the sternal head of the pectoralis major muscle and symbrachydactyly. The first case of Poland's syndrome to be reported from Indonesia is presented here.