[Poland's syndrome and free autologous fat grafts]. / Síndrome de Poland e injertos libres de grasa autóloga.

OBJECTIVE: To evaluate the final reconstructive results in 6 cases of female Poland syndrome treated in a first stage with an anatomical high cohesive breast implants and in a second stage with free autologous fat grafts. METHODS: Six females were submitted to bilateral breast reconstruction. The reconstruction was done using anatomical breast implants as first step and after 11 to 18 months the free autologous fat grafting using the Coleman technique as well as treating the contralateral breast to achieve a better symmetry and volume. Photographies taken pre and postoperatively and the Foucras Classification were used to determine volume. Four months after the last surgery the patients answered a satisfaction questionnaire. RESULTS: In all 6 patients there was an improvement of the thoracic contour a year after the last surgery in terms of volume, projection and width of the breast. CONCLUSIONS: The use of free autologous fat grafts improves the results in cases of Poland's syndrome previously treated with breast implants making possible to correct the anterior axillary fold, projection and symmetry with a versatile method, with a low morbidity rate, allowing for a personalized treatment.

Hand and upper limb anomalies in Poland syndrome: a new proposal of classification.

BACKGROUND: The Poland anomaly (PA) comprises unilateral absence or hypoplasia of the pectoralis major muscle and a variable degree of ipsilateral hand and upper limb anomalies. Various hand and upper limb anomalies classifications in PA have been previously published. In this work, a new classification of hand and upper limb anomalies in PA is proposed, on the basis of the clinical and instrumental evaluation of 175 patients. METHODS: The patients have been followed by a multidisciplinary approach, consisting in orthopaedic, surgical, and genetic evaluation and chest, upper limb, and ultrasound examination of major and minor pectoralis muscles, heart, and kidney. RESULTS: Hand and upper limb anomalies were classified in 8 groups on the basis of the clinical degree of severity and on the basis of the presence of coexisting associated anomalies. Data regarding the sex and laterality, previously reported in the medical literature, were confirmed by our analysis. Etiopathogenetic mechanisms leading to the anomaly are discussed. CONCLUSIONS: The proposed classification is derived from the observation of the widest group of patients described in the medical literature. Our proposal could help in the management of patients affected by Poland syndrome and in understanding etiological and pathologic aspects of the disease. LEVEL OF EVIDENCE: IV.

Breast cancer and Poland's syndrome: a case report and literature 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.

Anatomical, histologic, and genetic characteristics of congenital chest wall deformities.

There is a large and diverse group of congenital abnormalities of the thorax that manifest as deformities and/or defects of the anterior chest wall and, depending on the severity and concomitant anomalies, may have cardiopulmonary implications. Pectus excavatum, the most common anterior chest deformity, is characterized by sternal depression with corresponding leftward displacement and rotation of the heart. Pectus carinatum, the second most common, exhibits a variety of chest wall protrusions and very diverse clinical manifestations. The cause of these conditions is thought to be abnormal elongation of the costal cartilages. Collagen, as a major structural component of rib cartilage, is implicated by genetic and histologic analysis. Poland syndrome is a unique unilateral chest/hand deficiency that may include rib defects, pectoral muscle deficit, and syndactyly. Cleft sternum is a rare congenital defect resulting from nonfusion of the sternal halves, which leaves the heart unprotected and requires early surgical intervention.

Poland syndrome.

Poland syndrome is characterized by hypoplasia or absence of the breast or nipple, hypoplasia of subcutaneous tissue, absence of the costosternal portion of the pectoralis major muscle, absence of the pectoralis minor muscle, and absence of costal cartilages or ribs 2, 3, and 4 or 3, 4, and 5. The chest wall defect is often associated with a lung hernia. Clinical manifestations are extremely variable and rarely are all the features recognized in 1 individual. Fortunately it is invariably unilateral, allowing for an easier reconstruction. Single-stage reconstruction of the chest wall combined with simultaneous augmentation mammoplasty and transfer of an island pedicle myocutaneous flap of latissimus dorsi muscle are major improvements over previous multiple-stage procedures that provide less satisfactory cosmetic results in management of patients with Poland syndrome.

Malformations attributed to the process of vascular disruption.

BACKGROUND: Several malformations have been attributed to the process of vascular disruption. The central hypothesis for this etiology is that blood flow to a structure has been altered after that structure had formed normally. The decreased blood flow leads to hypoxia, endothelial cell damage, hemorrhage, tissue loss, and repair. After recovery, some structures are normal and others show either tissue loss or structural abnormalities, such as syndactyly and constriction rings. METHODS: The phenotypic features of the 7,020 infants with one or more malformations, who were born to women who had always planned to deliver at Brigham and Women's Hospital (BWH) between, 1972 and 2012, that is, maternal nontransfers, were reviewed. The phenotypes associated with vascular disruption, such as the amniotic band syndrome and terminal transverse limb defects (TTLD), were identified. RESULTS: One hundred and five fetuses and infants had malformations attributed to the process of vascular disruption. Some specific causes of the amniotic band limb deformity were identified. TTLD with associated small digit-like nubbins occurred at three levels: proximal forearm, wrist, and metacarpal-phalangeal joint. Other causes included severe hemoglobinopathies and exposures to misoprostol and to prenatal procedures. CONCLUSIONS: Malformations attributed to the process of vascular disruption were a distinctive entity, among the recognized etiologies. The timing of the causative event in the first trimester was established for infants with exposures to either the prostaglandin misoprostol or the prenatal diagnosis procedure chorionic villus sampling. One challenge is to identify the developmental steps in vascular disruption when no causative exposure can be identified.

Breast cancer, Poland's syndrome, and sentinel lymph node involvement.

Poland's syndrome includes a wide range of abnormalities that affect the thorax and the upper extremities. We present a case of a woman of 39 years of age who attended our clinic because of a lump in the right breast. Our experience with the present case suggests that the sentinel lymph node biopsy is feasible in patients with Poland's syndrome and breast cancer.

Ipsilateral foot and contralateral hand anomalies in a patient with Poland-Moebius syndrome.

This report describes a patient who had bilateral facial nerve paralysis, external ophthalmoplegia, absence of pectoralis major muscle at right side, ipsilateral hand and foot, and contralateral hand anomalies. To our knowledge, this is the first patient with Poland syndrome reported in combination with Moebius syndrome, presenting with contralateral hand and ipsilateral foot anomalies.

Poland-Möbius syndrome and cocaine abuse: a relook at vascular etiology.

This report presents a severe case of Poland-Möbius syndrome with central apnea at birth, ventilator-dependent, and with brainstem calcifications. The newborn had unilateral defect of the right pectoralis muscle, breast, and limb. He manifested bilateral paralysis of the cranial nerves resulting in shallow respiration, apnea, and dysphagia. He finally required tracheostomy and gastrostomy. Maternal history revealed multiple uses of cocaine during the first 3 months of pregnancy. The patient supports the Poland-Möbius combination and the possibility of vascular disruption sequence.

[Poland syndrome (a case report)]. / Poland sendromu (olgu sunumu).

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.

Bilateral Poland anomaly.

Bilateral absence of the pectoralis major muscle with accompanying abnormalities of shoulder muscles has been reported in patients without Poland anomaly (PA). However, symmetric absence of pectoralis major muscles, hypoplasia of breasts and nipples with symmetric chest wall deformity and bilateral hand anomaly has not previously been reported. A 6-year-old girl with bilateral absence of pectoralis major muscles and hand involvement and symmetric chest wall deformity is, to our knowledge, the first known case of bilateral Poland anomaly.

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.

Management of the chest-wall deformity in male patients with Poland's syndrome.

The chest-wall deformity associated with Poland's syndrome was reconstructed in eight male patients 16 to 38 years old (average age 20 years). Follow-up ranged from 1 to 10 years. Two patients had custom silicone implants placed subcutaneously. In one of these patients, the edge of the implant could be seen. Three patients had transfer of an ipsilateral pedicled latissimus dorsi muscle flap with intact thoracodorsal nerve. All these patients had noticeable atrophy of the flap, and one underwent subsequent implantation of a custom silicone implant beneath the flap. Three other patients had a custom silicone implant covered immediately by a latissimus dorsi muscle flap. All four patients who had a combination of silicone implant and latissimus dorsi muscle flap had satisfactory correction of their deformity.

Anterior thoracic hypoplasia: a separate entity from Poland syndrome.

SUMMARY: Women presenting with anterior thoracic depression, breast hypoplasia, and subsequent asymmetry are often diagnosed with Poland syndrome regardless of pectoralis involvement, or are placed in the generic category of breast asymmetry or skeletal dysplasias. Recently, though, the term "sunken chest" has been used to describe forms of chest wall depression that previously may have fallen under generic skeletal dysplasias. The authors believe that, combined with hypoplasia of the ipsilateral breast, superior location of the nipple-areola complex compared with the contralateral side, and normal pectoralis muscles, this represents a previously undefined and real condition called anterior thoracic hypoplasia. During the past 4 years, the authors have treated eight women who have presented with a diagnosis of Poland syndrome or pectus excavatum, all of whom share the same characteristics-unilateral sunken anterior chest wall, hypoplasia of the breast, superiorly placed nipple-areola complex, normal pectoralis muscle, and normal sternal position. All of the patients underwent correction of breast asymmetry and unilateral anterior thoracic hypoplasia with augmentation mammaplasty, a method that when tailored for each side yields good aesthetic results. The average age of the patients was 31 years and the average chest size was 34. Cup size, as measured by the patient's standard bra, was a B on the nonaffected side in all patients and an A on the affected side in all patients except one. Of the eight patients, seven had the right anterior chest and breast involved, whereas one patient had involvement on the left. For all of the patients, the nipple and areola of the hypoplastic side were smaller and in a more superior position compared with the contralateral side on visual inspection. In the eight patients, a total of 19 augmentations (15 primary augmentations and four revisions) and one mastopexy were performed. Ten inframammary-fold approaches and nine periareolar approaches were used, and all of the implants were placed in a partial submuscular position, except for two implants placed in a subglandular position that were converted to partial submuscular positions in a secondary setting. In all the women, the sternal head of the pectoralis muscle was present and the pectoralis muscle appeared to be equal in size compared to the contralateral side. Nine different types of implants were used. Average implant fill volume measured 412 cc on the hypoplastic side and 257 cc on the contralateral side. In follow-up, all of the patients were satisfied with their operation and rated their aesthetic outcome as very good to excellent. The authors believe that anterior thoracic hypoplasia is a real, previously misdiagnosed and undescribed condition, and that both chest wall and breast deformities can be corrected safely and with excellent results using proper augmentation planning and implant selection.

Maxillomandibular deformity in association with Poland anomaly.

A case of jaw deformity in association with right-sided Poland anomaly in an 18-year-old male patient is presented. As there have been no cases previously reported of pectoralis major muscle hypoplasia combined with dentofacial deformity, it is hoped that other reports would emerge to describe a new syndrome.

Unilateral absence of the trapezius and pectoralis major muscle: a variant of Poland syndrome.

We report a female patient with unilateral absence of the trapezius and pectoralis major muscles without other associated limb abnormalities. We believe that this anomaly belongs to the spectrum of anomalies resulting from disruption of the blood supply in the embryonic subclavian and vertebral arteries, as suggested in Poland syndrome.