Diagnostic value of ultrasonography in Poland′s syndrome / 中华超声影像学杂志

Objective:To investigate the diagnostic value of ultrasonography in Poland′s syndrome.Methods:From February 2016 to December 2020, the ultrasonographic images of 15 patients with Poland′s syndrome diagnosed by Provincial Hospital Affiliated to Shandong First Medical University were retrospectively analyzed, and the ultrasonographic features were summarized.Results:High-frequency ultrasound could clearly show the anatomical structures of each layer of the chest wall of the patients with Poland′s syndrome. The sonogram of Poland′s syndrome mainly showed the absence of all or part of the pectoralis major on the affected side, some of which were combined with the absence of pectoralis minor. The difference between the thickness of the affected chest wall and the healthy side was statistically significant ( P<0.01). Among the 15 cases of Poland′s syndrome, 11 cases had brachydactyly or syndactyly. Ultrasound showed that the bifurcation position of the common palmar digital artery of the middle finger was lower than that of the healthy side. Conclusions:The ultrasonography is an effective imaging method for diagnosis of Poland′s syndrome.

Colgajo dorsal ancho por abordaje único vertical en el síndrome de Poland: caso clínico / Retalho latíssimo do dorso por abordagem único vertical na síndrome de Poland: caso clínico / Latissimus dorsi vertical single-approach flap in Poland syndrome: clinical case

El síndrome de Poland constituye una malformacion que asocia diversos grados de anomalia toracica y a nivel del miembro superior, con una incidencia de 1 cada 30000 nacidos vivos. Esta patologia geenra ausencia de la glandula mamaria y del musculo pectroral mayor como malformacion mas frecuente. Lo cual produce una asimetria a nivel de torax con perdida de la armonia la cual lleva a una alteracion tanto fisica como psicologica a nivel de la paciente. Por esto es necesario resolver esta patologia con la menor morbilidad posible. El planteo de generar la menor morbilidad nos llevo a realizar una tecnica por abordaje unico sub axilar, a traves del cual se diseca el musculo dorsal ancho y se transpone de forma anterior, obteniendo una sola incision, la cual se mantiene oculta debajo del miembro superior adducido. Produciendo menor tiempo operatorio, menor tiempo de internacion, disminuyendo el número de incsiones y logrando una adecuada fijacion anterior del musculo dorsal ancho.

Poland's syndrome is a malformation associated with varying degrees of thoracic and upper limb abnormality, with an incidence of 1 in 30,000 live births. This pathology generates the absence of the mammary gland and the major pectoral muscle as the most frequent malformation. Which produces an asymmetry at the chest level with loss of harmony which leads to both physical and psychological alteration at the level of the patient. For this reason, it is necessary to resolve this pathology with the least possible morbidity. The proposal to generate the least morbidity led us to perform a single sub axillary approach technique, through which the latissimus dorsi muscle is dissected and transposed anteriorly, obtaining a single incision, which is kept hidden under the upper limb adduced. Producing less operative time, shorter hospitalization time, reducing the number of incisions and achieving an adequate anterior fixation of the latissimus dorsi muscle.

A síndrome de Poland é uma malformação associada a vários graus de anomalia torácica e de membros superiores, com uma incidência de 1 em 30.000 nascidos vivos. Essa patologia gera a ausência da glândula mamária e do músculo pectoral principal como a malformação mais frequente. O que produz uma assimetria ao nível do peito com perda de harmonia que conduz a alterações físicas e psicológicas ao nível do paciente. Por isso, é necessário resolver essa patologia com a menor morbidade possível. A proposta de gerar o mínimo de morbidade nos levou a realizar uma única técnica de abordagem subaxilar, por meio da qual o músculo grande dorsal é dissecado e transposto anteriormente, obtendo-se uma única incisão, que é mantida escondida sob o membro superior. aduzido. Produzindo menos tempo operatório, menor tempo de internação, reduzindo o número de incisões e conseguindo uma fixação anterior adequada do músculo grande dorsal.

Síndrome de Poland. Reporte de un caso / Poland syndrome. Report of a case / Síndrome da Polônia. Relato de um caso

RESUMEN Introducción: el síndrome de Poland es un trastorno congénito raro que se caracteriza por la ausencia o hipoplasia unilateral del musculo pectoral mayor asociado a malformaciones en la extremidad superior y tejido mamario ipsilateral. Las manifestaciones clínicas en los recién nacidos solamente se describen en la literatura, por lo tanto, muchos aspectos de su presentación en las guarderías son ignoradas. Presentación del caso: presentamos el caso de un recién nacido que manifiesta una respiración paradójica desde el momento del nacimiento. Este tipo de hallazgo clínico esta vagamente descrito en el Síndrome de Poland y menos aún en el periodo neonatal. Discusión: este síndrome puede verse relacionado con enfermedades, como parálisis de los pares VI y VII, lo que conlleva a un mayor número de asociaciones complejas.9 Se plantea asociado a dextrocardia, que según algunos autores es secundaria a la deformidad torácica producida por malformaciones costales y su efecto sobre la formación cardíaca en el periodo embrionario. No se han observado deficiencias mentales ni trasmisión genética de la enfermedad. Se ha descrito de forma excepcional, asociación familiar. Esto podía corresponder a una herencia autosómica dominante con penetración reducida. Conclusiones: en este paciente el síndrome de Poland se caracterizó por una hipoplasia del pectoral mayor izquierdo, asociado a malformaciones esqueléticas de la mano del mismo lado.

ABSTRACT Introduction: Poland syndrome is a rare congenital disorder characterized by the unilateral absence or hypoplasia of the pectoralis major muscle associated with malformations of the upper limb and ipsilateral breast tissue. Clinical manifestations in newborns are only described in the literature, therefore, many aspects of their presentation in nurseries are ignored. Presentation of the case: we present the case of a newborn who manifests paradoxical respiration from the moment of birth. This type of clinical finding is loosely described in Poland Syndrome and even less in the neonatal period. Discussion: this syndrome can be related to diseases, such as paralysis of pairs VI and VII, which leads to a greater number of complex associations.9 It arises in association with dextrocardia, which according to some authors is secondary to the thoracic deformity caused by malformations ribs and their effect on cardiac formation in the embryonic period. No mental deficiencies or genetic transmission of the disease have been observed. An exceptional family association has been described. This could correspond to an autosomal dominant inheritance with reduced penetration. Conclusions: in this patient, Poland syndrome was characterized by hypoplasia of the left pectoralis major, associated with skeletal malformations of the hand on the same side.

RESUMO Introdução: A síndrome da Polônia é um distúrbio congênito raro caracterizado pela ausência ou hipoplasia unilateral do músculo peitoral maior associado a malformações do membro superior e tecido mamário ipsilateral. As manifestações clínicas em recém-nascidos são descritas apenas na literatura, portanto, muitos aspectos de sua apresentação nos berçários são ignorados. Apresentação do caso: apresentamos o caso de um recém-nascido que manifesta respiração paradoxal desde o momento do nascimento. Esse tipo de achado clínico é descrito livremente na Síndrome da Polônia e menos ainda no período neonatal. Discussão: essa síndrome pode estar relacionada a doenças, como a paralisia dos pares VI e VII, que leva a um maior número de associações complexas.9 Ela surge associada à dextrocardia, que segundo alguns autores é secundária à deformidade torácica causada por malformações costelas e seu efeito na formação cardíaca no período embrionário. Não foram observadas deficiências mentais ou transmissão genética da doença. Uma associação familiar excepcional foi descrita. Isso pode corresponder a uma herança autossômica dominante com penetração reduzida. Conclusões: nesse paciente, a síndrome da Polônia foi caracterizada por hipoplasia do peitoral maior esquerdo, associada a malformações esqueléticas da mão do mesmo lado.

A rare case of Poland: Mobeius syndrome in an infant

Mobius syndrome is a rare condition of unclear origin, characterized by a unilateral or bilateral congenital facial weakness with impairment of ocular abduction, which is frequently associated with limb anomalies. Poland Syndrome is a rare condition that is evident at birth (congenital). Associated features may be extremely variable from case to case. However, it is classically characterized by absence (aplasia) of chest wall muscles on one side of the body (unilateral) and abnormally short, webbed fingers (symbrachydactyly) of the hand on the same side (ipsilateral). In those with the condition, there is typically unilateral absence of the pectoralis minor and the sternal or breastbone portion of the pectoralis major. In females, there may be underdevelopment or absence (aplasia) of one breast and underlying (subcutaneous) tissues. In some cases, associated skeletal abnormalities may also be present, such as underdevelopment or absence of upper ribs; elevation of the shoulder blade (Sprengel deformity); and/or shortening of the arm, with underdevelopment of the forearm bones (i.e., ulna and radius). Other associated abnormalities may include dextrocardia, diaphragmatic hernia and renal anomalies etc. Poland Syndrome affects males more commonly than females and most frequently involves the right side of the body. The exact cause of the condition is unknown. The combination of Poland-Mobius syndrome is rare, with an estimated prevalence 1:500 000.

Quality of life and effects of breast reconstruction in patients with breast deficiency: a cross-sectional study / 中华整形外科杂志

Objective@#To investigate the satisfaction and well-being of Chinese women with breast deficiency, and to analyze the relevant influencing factors.@*Methods@#A cross-sectional study was conducted using a self-designed questionnaire to investigate the women with breast deficiency, visiting the Plastic Surgery Hospital of Chinese Academy of Medical Sciences from November 2013 to August 2018. The BREAST-Q BR (breast reconstruction) pre-operation and post-operation modules were used to assess the quality of life.@*Results@#A total of 139 effective questionnaires were collected, including 83 patients before BR surgery and 56 post. There was no difference in quality of life in different ages, marital status, education levels, working status, and causes of breast deficiency. Mean scores of satisfaction with breasts, psychosocial well-being and sexual well-being of patients after breast reconstruction were significantly higher than those of patients without reconstruction (P=0.000), but there was no statistical difference in physical well-being-chest score. The physical well-being abdomen scores of patients with abdominal flap reconstruction was lower than that of the patients without reconstruction (P=0.007). With regard to analysis of specific items, compared with preoperative patients, patients with abdominal flap reconstruction reported lower scores in the items related to abdominal muscle weakness (P<0.05).@*Conclusions@#The breast deficiency resulting from Poland syndrome, breast cancer and other causes, can lead to a significant decrease in quality of life. Breast reconstruction can improve satisfaction with breast, psychosocial and sexual well-being in women with breast deficiency. The abdomen well-being of patients with abdominal flap reconstruction is decreased. Therefore, more attention should be paid to retain abdominal muscles to maintain abdominal shape and motor function during operation.

Deformidad de la caja torácica / Chest wall deformity

Resumen Las deformidades de la caja torácica se pueden dividir en dos tipos, las que son productos del desarrollo anormal del pecho en el crecimiento y las congénitas que son las secundarias a una malformación estructural del pecho evidente en el nacimiento. Las malformaciones del desarrollo son las más comunes, como por ejemplo pectus excavatum o pectun carinatum. Las menos comunes son las de tipo congénito: síndrome de Poland, displasia espondilotorácica, displasia espondilocostal, síndrome de Jeune y los defectos de la costilla o el esternón. Las deformidades del pecho de tipo congénita se caracterizan por afectar la relación entre la columna vertebral, la caja torácica y los pulmones. La mayoría de estos pacientes desarrollan un disturbio respiratorio progresivo de tipo restrictivo conocido como Síndrome de Insuficiencia Torácica. Este síndrome se define como la deficiencia de la caja torácica para mantener una respiración normal y sostener el crecimiento fisiológico del pulmón. En este artículo discutiremos varias condiciones que afectan el desarrollo y función de la caja torácica.

Chest wall deformities are divided as an abnormal development during the growth or those secondary to a congenital malformation. The developmental type is the most common: pectus excavatum or pectus carinatum. The less common are the congenital types of chest wall abnormalities: Poland's syndrome, Jeune's syndrome, espondylothoracic dysplasia, espondylocostal dysplasia and defects of the ribs or sternum. The congenital type usually affects the relationship between the spine, rib cage and the lungs. Therefore, many of these patients will develop a progressive respiratory disturbance of restrictive type known as Thoracic Insufficiency Syndrome. Thoracic insufficiency syndrome is defining as a deficiency of the rib cage to maintain a normal respiration and to sustain the physiological growth of the lungs. In this article will discuss several conditions that will affect the development and function of the chest wall.

Síndrome de Poland: descripción de un caso en la sala de partos / Poland syndrome: Description of a case in the delivery room

El Síndrome de Poland es un trastorno congénito raro que se caracteriza por la ausencia o hipoplasia unilateral del musculo pectoral mayor asociado a malformaciones en la extremidad superior y tejido mamario ipsilateral. Las manifestaciones clínicas en los recién nacidos solamente se describen en la literatura, por lo tanto muchos aspectos de su presentación en las guarderías son ignoradas. Presentamos el caso de un recién nacido que manifiesta una respiración paradójica desde el momento del nacimiento. Este tipo de hallazgo clínico esta vagamente descrito en el Síndrome de Poland y menos aún en el periodo neonatal.

Poland Syndrome is a rare congenital condition characterized by the absence unilateral pectoralis major muscle. it can be associated with other multiple malformations usually in the same affected side. The clinical manifestation in newborns are merely described in the literature, therefore many aspect in its presentation in the nursery are ignored. We present the case of a newborn manifesting paradoxical breathing since birth. This clinical finding is barely describe in poland's syndrome and even less during newborn period.

Serie de Casos: Síndrome de Poland Completo vs. Incompleto. Resolución Quirúrgica / Case Series: Poland Syndrome Complete vs Incomplete. Surgical Resolution

INTRODUCCIÓN: El síndrome de Poland es una enfermedad congénita polimalformativa extremadamente rara, su etiología es aún desconocida, afectando principalmente el desarrollo del músculo pectoral mayor; se acompañan frecuentemente de malformaciones de la mano, mama, complejo areola-pezón, pectoral menor ipsilateral y otras malformaciones asociadas. Su diagnóstico se basa en un correcto examen clínico junto con una tomografía axial computarizada con reconstrucción en 3D. El tratamiento quirúrgico final es individualizado y dependerá de la existencia de una o más malformaciones. CASOS CLÍNICOS: Se trató de cuatro pacientes con cuadro clínico compatible con síndrome de Poland, siendo común el subdesarrollo o ausencia del músculo pectoral mayor. EVOLUCIÓN: Se realizó a cada paciente una tomografía axial computarizada con reconstrucción en 3D para confirmar su diagnóstico y realizar un tratamiento quirúrgico según la necesidad de cada paciente, después del procedimiento se dio seguimiento a los pacientes con una respuesta favorable y sin complicaciones. CONCLUSIONES: El síndrome de Poland se caracteriza por un cuadro clínico variable, con la ausencia o subdesarrollo del músculo pectoral mayor, el tratamiento quirurgico fue personalizado para cada paciente. Las técnicas quirúrgicas incluyeron reconstrucción de la pared torácica mediante osteosíntesis en los casos graves independientemente del sexo del paciente; rotación de colgajos del músculo dorsal ancho, colocación de expansores tisulares y prótesis mamarias en el caso de las pacientes femeninas y colocación de prótesis preformadas mediante estereolitografía en el caso de los pacientes masculinos.

BACKGROUND: The Poland syndrome is an extremely rare polymalformative congenital disease, the etiology is still unknown, mainly affecting the development of the pectoralis major muscle; they are frequently accompanied by malformations of the hand, breast, nipple-areola complex, ipsilateral minor pectoral and other associated malformations. The diagnosis is based on a correct clinical examination together with a computerized axial tomography with 3D reconstruction. The final surgical treatment is individualized and will depend on the existence of one or more malformations. CASE REPORTS: Four patients with a clinical picture compatible with the Poland syndrome were treated, with underdevelopment or absence of the pectoralis major muscle being common. EVOLUTION: Each patient underwent a computerized axial tomography with 3D reconstruction to confirm their diagnosis and perform a surgical treatment according to the needs of each patient; after the procedure, patients were followed up with a favorable response and without complications. CONCLUSIONS: The Poland syndrome is characterized by a variable clinical picture, with the absence or underdevelopment of the pectoralis major muscle, the surgical treatment was customized for each patient. Surgical techniques included reconstruction of the chest wall by osteosynthesis in severe cases regardless of the sex of the patient; rotation of flaps of the latissimus dorsi muscle, placement of tissue expanders and mammary prosthesis in the case of female patients and placement of preformed prostheses by stereolithography in the case male patients.

Video Caso Clínico: Disección Aórtica Rota Tipo Stanford A. Resolución Quirúrgica: Cirugía de Bentall De Bono / Video Case Report: Rupture Aortic Dissection Stanford Type A. Surgical Resolution: Surgery Bentall and De Bono

La disección aórtica rota que compromete la raíz y la aorta ascendente es una emergencia que requiere cirugía inmediata, debido al mal pronóstico y sus complicaciones agudas: ruptura, insuficiencia aórtica aguda, hematoma intramural y endocarditis aguda; con elevadas tasas de morbilidad y mortalidad posoperatoria temprana, estimada en las 24 horas el 1 % por cada hora, a las 48 horas 29 %, en la primera semana 44 % y a las 2 semanas 50 %; con una mortalidad global de 15 % a 35 % y una sobrevida del 65 % al 75 % en un rango de 5 años [1 - 3]. En Ecuador se llevó a cabo un estudio tipo prospectivo que incluyo 120 pacientes, llevado a cabo desde 1999 a 2000, en Guayaquil; en el que reportaron 34 casos, con edad promedio de 64 años, sexo masculino, cuyo factor de riesgo más importante fue hipertensión arterial. Se presentó en el 100 % de los casos, dolor torácico transfictivo, soplo aórtico diastólico en el 70 % y solo el 2 % presentaron infarto de miocardio posteroinferior transmural sin fibrinólisis, por ser contraindicado en pacientes con disección aórtica [4]. El tratamiento quirúrgico consiste en la resección y reemplazo de la porción rota por una prótesis vascular asociada o no al reemplazo valvular aórtico dependiendo del grado de insuficiencia. El tratamiento estará orientado en función del grado de afectación de la disección y la rotura, pudiendo requerir un reemplazo convencional de la aorta ascendente (RCAA) con o sin reemplazo total del arco aórtico (RTAA); el tratamiento de elección de la disección rota tipo A es el reemplazo de la raíz y la aorta ascendente con injerto tubular valvulado, denominado procedimiento de Bentall De Bono [1 - 3, 5, 6]. Dentro de las complicaciones postquirúrgicas se encuentran: pseudoaneurisma, disección radical recurrente o residual, cambios importantes en el diámetro de la raíz o insuficiencia aórtica significativa [7]

The rupture aortic dissection that compromises the root and the ascending aorta is an emergency that requires immediate surgery; for it is poor prognosis, acute complications: rupture, acute aortic insufficiency, intramural hematoma and acute endocarditis, with high morbidity rates and early postoperative mortality, estimated at first 24 hours in 1 % per each hour, 48 hours 29 %, in the first week 44 % and at 2 weeks 50 %; with a global mortality of 15 % to 35 % and a survival of 65 % to 75 % in a range of 5 years [1-3]. In Ecuador, a prospective study realized that included 120 patients, since 1999 to 2000, in Guayaquil; in that they reported 34 cases with an average age of 64 years, male, whose most important risk factor was hypertension. The patients presented, 100 % transfictive chest pain, diastolic aortic murmur in 70 %, and only 2 % had transmural posteroinferior myocardial infarction without fibrinolysis, as it was contraindicated in patients with aortic dissection [4]. Surgical treatment consists of the resection and replacement of the broken portion by a vascular prosthesis associated or not with aortic valve replacement depending on the degree of insufficiency. The treatment will be oriented according to the degree of involvement of the dissection and rupture, and may require a conventional replacement of the ascending aorta (CRAA) with or without total aortic arch replacement (TRAA); the treatment of choice for the rupture aortic dissection type A is the replacement of the root and the ascending aorta with valvular tubular graft, called the Bentall De Bono surgery [1 - 3, 5, 6]. Of the postsurgical complications we found: pseudoaneurysm, radical or recurrent radical dissection, important changes in the diameter of the root or significant aortic insufficiency

Síndrome de Poland. Presentación de un caso y revisión de la literatura

Se han descrito diversas alteraciones en la morfología y estructura de la glándula mamaria, como resultado de variaciones en los mecanismos reguladores de su desarrollo. La amastia es una de las anomalías congénitas de la mama menos frecuente, y consiste en la ausencia total de una o de ambas glándulas. Cuando es unilateral generalmente se acompaña también de la carencia del músculo pectoral mayor y del menor además de otro grupo de malformaciones, constituyendo así lo que se conoce como síndrome de Poland. Se presenta el caso de una adolescente de 15 años, que exhibe una ausencia congénita de la mama derecha, así como de los músculos pectorales, sin otra malformación acompañante. Es este el primer caso de síndrome de Poland diagnosticado en Cienfuegos, por lo cual se considera de interés para la comunidad científica la publicación de este reporte.

Diverse changes in the morphology and structure of the mammary gland have been described as a result of the variation of the regulatory mechanisms of its development. Amastia is one of the less frequent congenital anomalies of the breast and it consists on the total absence of one or both glands. When it is unilateral it is generally also accompanied by the lack of the major and minor pectoral muscle besides other malformations, constituting what is known as Poland Syndrome. A case of a fifteen year old female patient is presented who shows the congenital absence of the right breast. This is the first case of Poland Syndrome diagnosed in Cienfuegos, this is why the publication of this case is considered of interest for the scientific community.

Síndrome de Poland: dois relatos de caso com suas respectivas condutas cirúrgicas / Poland's syndrome: two case reports with their respective surgical procedures

A Síndrome de Poland é uma anomalia congênita de baixa incidência, que afeta em média 1:32000 nascidos vivos e tem causa idiopática. Essa condição é caracterizada pela atrofia ou ausência do músculo peitoral maior, mas pode acometer também outros músculos, como o peitoral menor e serrátil. Nessa síndrome pode ocorrer associação de outros fenômenos, como a sindactilia ipsilateral, hipoplasia da mão, alterações ósseas e deformidade de Sprengel, na qual a escápula passa a ser menor e alada. No presente estudo, relatamos dois casos de Síndrome de Poland com base na revisão de prontuários. No primeiro caso, percebemos uma atrofia muscular na mama direita em que foi necessária a utilização de dois expansores para fazer a reconstrução, um na mama direita e outro na região toracoabdominal, em virtude de atrofia tecidual entre a pele e o osso. Em sequência, foi colocada a prótese mamária definitiva de silicone e foi feita a correção da atrofia tecidual. Já no segundo caso, não houve a necessidade de colocação de expansores. Em cada caso foi usada uma maneira de correção, uma vez que, nesta síndrome, não há um método único de tratar o paciente, devendo cada caso ser estudado individualmente. Com o estudo, é possível notar que a Síndrome de Poland não causa uma patologia de fato, já que na ausência do músculo peitoral maior não há comprometimentos motores, o que ocorre é um comprometimento estético(AU)

Poland 's syndrome is a congenital abnormality of low incidence, which affects roughly 1: 32,000 live births and is idiopathic. This condition is characterized by atrophy or absence of the pectoralis major muscle, but can also affect other muscles such as the pectoralis minor and serratus. This syndrome can occur in association with such other phenomena as ipsilateral syndactyly, hand hypoplasia, bone changes, and Sprengel's deformity, in which the scapula becomes smaller and winged. In the present study we report two cases of Poland's syndrome based on chart review. In the first case there was muscular atrophy in the right breast, where the use of two expanders was required for the reconstruction, one on the right breast and the other in the thoracoabdominal region, due to tissue atrophy between the skin and the bone. Subsequently, the final silicone breast implant was placed and correction of tissue atrophy was performed. In the second case there was no need for using expanders. In each case a different way of correction was used, since in this syndrome there is no single method to treat the patient, and each case should be studied individually. From this study one can see that Poland's Syndrome does not cause an actual pathology, as the absence of the pectoralis major does not bring any motor compromising; what happens is aesthetic compromising(AU)

Unilateral and Complete Agenesis of Right Pectoralis Major Muscle.

Introduction: Pectoralis major Myocutaneous flaps (PMMC) are extensively used for Head, Neck, Face and post mastectomy mammary gland reconstructive surgeries. Pectoralis major is responsible for flexion, adduction and medial rotation of shoulder joint. Its absence may obvious because of compromised movement of shoulder joint or as a part Poland’s syndrome or may be sporadic as seen in our case. Case study: Pectoralis major muscle was completely absent on right side of an adult female cadaver. Result: Complete unilateral agenesis of right Pectoralis major muscle was found in an adult female cadaver during routine undergraduate dissection in the department of Anatomy. Right Mammary gland was lying directly on hypertrophied Pectoralis minor muscle. Discussion: Awareness about absence of Pectoralis major muscle is a must for planning and success of various reconstructive surgeries, flawless interpretation of Radiographs, CT and MRI scans. Unilateral and complete agenesis of Pectoralis major muscle not associated with any other anomaly visible to naked eye as seen in our case is a rare finding and hence needs to be documented.

Poland Syndrome in One Dizygotic Twin: A Case Report / 대한주산의학회잡지

Poland syndrome, also known as pectoral aplasia-dysdactylia syndrome, is a rare anomaly, characterized by aplasia of the pectoralis major muscle with ipsilateral upper extremity anomalies. The cause of Poland syndrome is still unknown. Several twin patients were reported to propose a genetic or nongenetic hypothesis. We experienced a female baby showing absence of the pectoralis major muscle in a pair of mixed twin. The patient was referred to our hospital for evaluation of an asymmetric chest wall. She showed depression of the left chest wall, ipsilateral nipple hypoplasia, and axillary webbing. A chest CT scan showed aplasia of the left large pectoral muscle. Our patient had no abnormalities such as symbrachydactyly in an ipsilateral upper extremity. In comparison, a physical examination showed that the fraternal twin boy was completely normal.

Poland's syndrome with unusual hand and chest anomalies: A rare case report.

Poland’s syndrome is a rare congenital anomaly consisting of a unilateral absence of the pectoralis major, ipsilateral muscle, hand anomaly and occasionally associated other malformations of the chest wall and breast. Many structural and functional abnormalities have been described in association with this syndrome. We report an incidentally diagnosed case in a 27-year-old male patient who presented to us with symbrachydactyly. In addition to this, anterior depression of 2nd, 3rd and 4th ribs and bifid (forked) 5th rib was present on radiological investigations. The body of sternum was short and deformed on the right side with absence of xiphoid process. All middle phalanges were absent on right hand. It is a rare variant of Poland’s syndrome.

Poland syndrome.

Poland’s syndrome is a rare congenital condition, characterized by the absence of the sternal or breastbone portion of the pectoralis major muscle, which may be associated with the absence of nearby musculoskeletal structures. We hereby report an 8‑year‑old boy with typical features of Poland syndrome, the first documented case from Uttar Pradesh, India.

Tratamiento de la anisomastia en una paciente con síndrome de Poland: a propósito de un caso / Treatment of the anisomastia in a patient with Poland's Syndrome: a case report

Fundamento: el síndrome de Poland es una enfermedad congénita que tiene como denominador común la hipoplasia o agenesia del músculo pectoral mayor aparejado a trastornos del desarrollo mamario. Objetivo: describir el cuadro clínico de una paciente con este síndrome. Caso clínico: se trata de una paciente con Síndrome de Poland que presenta una deformidad del tórax, con un desarrollo mamario desigual y fue sometida a tratamiento quirúrgico reparador. Conclusiones: el síndrome de Poland tiene una presentación clínica variable, con distintos grados de extensión y severidad. En caso anisomastia resulta útil en el tratamiento quirúrgico.

Background: Poland's Syndrome is a congenital disease that has like common denominator the hipoplasia or infertility of the bigger pectoral muscle matched up to upsets of the mammary development. Objective: describing the clinical picture of a patient with this syndrome. Clinical case: It has to do with a patient with Poland's Syndrome that presents a deformity of the thorax, with a mammary unequal development and was submitted to surgical reparative treatment. Conclusions: Poland's syndrome has a clinical variable presentation, with several degrees of extension and severity. In case anisomastia proves to be useful in the surgical treatment.

Secuencia Poland: presentación de casos con recurrencia familiar / Poland sequence: case reports of familial recurrence

Introducción: la Secuencia Poland es un conjunto raro de defectos congénitos caracterizado por la agenesia total o parcial del músculo pectoral mayor con asociaciones variables de otros defectos a nivel de tórax y extremidades superiores. Se presenta de forma esporádica y mayoritariamente afecta un solo lado. Objetivos: caracterizar desde el punto de vista clínico la Secuencia Poland presente en dos individuos afectados dentro de una misma familia. Material y Métodos: se realizó un estudio descriptivo de tipo reporte de casos. Conclusiones: el hallazgo orienta hacia la transmisión familiar de genes de susceptibilidad que participan en el origen de esta entidad bajo la probable acción de factores ambientales que justifican la marcada heterogeneidad clínica del defecto.

Introductión: Poland sequence is a rare set of congenital defects characterized by total or partial agenesis of the chest muscle (pectoralis major) with varied associations of other defects at the level of thorax and superior limbs. Its presentation is sporadic and most often affects one side of the body. Objective: to characterize Poland sequence in two individuals of the same family from the clinical point of view. Material and Methods: a descriptive study of case reports. Conclusions: the findings lead to a familial transmission of gene susceptibility participating in the origin of this syndrome under the potential actions of environmental factors that justify the marked clinical heterogeneity of this birth defect.

Achados radiológicos na síndrome de Poland / Poland 's syndrome: radiologic findings

A síndrome de Poland é uma anomalia congênita rara não hereditária. Os autores descrevem os achados radiológicos clássicos da síndrome de Poland através de um relato de caso de um paciente masculino de quatro anos de idade com assimetria torácica e das mãos, ilustrando os critérios imaginológicos fundamentais para a conclusão diagnóstica.

Poland's syndrome is a rare non-inherited congenital anomaly. The authors describe the classic radiologic findings of Poland's syndrome by reporting the case of a male four-year old patient with asymmetry of hands and chest, illustrating the fundamental imaging criteria for a conclusive diagnosis.

Moebius-Poland syndrome: A case report / Síndrome Moebius-Poland: Reporte de un caso

The Moebius syndrome is an infrequent symptomology in which the sixth and seventh cranial nerves are involved. Such involvement is translated in facial paralysis. There have been described around 500 cases in the world medical literature and some of them have received surgical treatment. Moebius syndrome has also received other names such as congenital nuclear aplasia, childlike nuclear aplasia, Oculofacial congenital paralysis and facial diplegia. Poland syndrome is another rare congenital abnormality of the chest wall, characterized by unilateral partial or total absence of the great pectoral muscle and ipsilateral symbrachydactyly. However, the Moebius-Poland syndrome is rarer. Its first case was recently reported in 2007 by Diego López de Lara et al. In this article we will report this infrequent case; the combination between both syndromes Moebuis and Poland in a three -month- old male patient.

El síndrome de Moebius es una sintomatología poco frecuente en la que los pares craneales sexto y séptimo están involucrados. Esta implicación resulta en parálisis facial. Se han descrito unos 500 casos en la literatura médica mundial y algunos de ellos han recibido tratamiento quirúrgico. Además el síndrome ha recibido otros nombres, tales como aplasia congénita nuclear, aplasia nuclear infantil, parálisis congénita oculofacial y diplejía facial. El síndrome de Poland es otra anomalía congénita muy poco frecuente de la pared torácica, caracterizado por ausencia unilateral parcial o total del músculo pectoral mayor y braquisindactilia ipsilateral. Sin embargo, el síndrome de Moebius-Poland es más raro, ya que el primer caso fue reportado recientemente en el año 2007 por Diego López de Lara et al. En este artículo se presentará este caso poco frecuente, que es una combinación entre ambos síndromes Moebius y Poland en un paciente masculino de tres meses de edad.

Reconstrução de mama usando o músculo grande dorsal: descrição de uma nova técnica com cicatriz reduzida / Breast reconstruction using latissimus dorsi muscle flap: a novel surgical technique with reduced scarring

INTRODUÇÃO: O retalho de músculo grande dorsal (MGD) é uma das opções cirúrgicas mais usadas nas reconstruções mamárias. A cicatriz da área doadora no dorso, embora por vezes situada sob a alça do sutiã, frequentemente é extensa e pode ser de qualidade ruim (alargada ou hipertrófica). O objetivo deste trabalho é descrever a dissecção e rotação do MGD através de duas incisões reduzidas. MÉTODO: No total, 12 pacientes do sexo feminino foram submetidas a reconstrução mamária unilateral com retalho de MGD isolado (sem pele). Dessas pacientes, 10 eram portadoras de síndrome de Poland e tiveram suas mamas reconstruídas com expansor tecidual num primeiro tempo, com posterior rotação do retalho de MGD para cobrir a prótese de silicone definitiva; e outras 2 pacientes tinham evoluído com diminuição do volume, irregularidades e retrações da mama após adenomastectomia, e tiveram sua prótese coberta pelo MGD. O procedimento cirúrgico consistiu de incisão longitudinal de 5 cm sobre a borda lateral do MGD e no sulco inframamário. Realizou-se dissecção, secção e rotação do músculo somente com a retração da pele. RESULTADOS: Em seguimento ambulatorial, a extensão da cicatriz apresentou média de 6,2 cm. O tempo cirúrgico médio para rotação do retalho e síntese da área doadora foi de 80 minutos. Não foram observadas complicações pós-operatórias nesta série. CONCLUSÕES: A incisão reduzida para dissecção do MGD nas reconstruções mamárias mostrou-se bastante factível e segura, com vantagem estética e sem aumento dos custos e do tempo cirúrgico.

BACKGROUND: The latissimus dorsi muscle flap is one of the most commonly used surgical options for breast reconstruction. The donor area scar on the back, although sometimes hidden beneath the bra strap, is often extensive and enlarged or hypertrophic. The aim of this study is to describe the dissection and rotation of the latissimus dorsi muscle using 2 reduced vertical incisions. METHODS: Twelve female patients underwent unilateral breast reconstruction with latissimus dorsi muscle flap. Ten patients had Poland's syndrome, and their breast reconstruction was performed with a tissue expander at the first instance, with subsequent latissimus dorsi muscle flap rotation to cover the silicone prosthesis. Two patients experienced volume decrease, irregularities, and breast retractions after adenomastectomy; their prosthesis were also covered by a latissimus dorsi muscle flap. The surgical procedure consisted of a 5-cm longitudinal incision on the lateral border of the latissimus dorsi muscle and another on the inframammary fold. Dissection, section, and rotation of the muscle were performed through skin retraction. RESULTS: The final extension of the scar at follow-up averaged 6.2 cm. The mean surgical time for flap rotation and synthesis of the donor area was 80 minutes. No postoperative complications were observed in any of the cases. CONCLUSIONS: The reduced incision for dissection of the latissimus dorsi flap for breast reconstruction proved to be feasible and safe; this method provides aesthetic advantages without increasing cost or surgical time.