Diagnostic value of chest computed tomography images in adult Poland syndrome: a report of two cases

Poland syndrome is a rare congenital developmental deformity characterized by unilateral agenesis or hypoplasia of thoracic wall soft tissue. We report two adult cases of Poland syndrome detected by computed tomography (CT) images. CT images of the two cases depicted an asymmetric chest wall with the absence of a breast and agenesis of the pectoralis muscles. A physical examination of case 1 showed a thin right chest wall with depression of the right nipple region. Hand deformities were also observed, including brachydactyly and syndactyly. In case 2, hand deformities were not found in a physical examination. Using multi-planar reconstruction, the size, position, origin, and termination of bilateral pectoral muscles could be compared symmetrically. For patients with Poland syndrome, a timely diagnosis and treatment are important. The use of chest CT in clinical practice could play an important role in the early diagnosis and treatment of Poland syndrome.

An unusual case of a neonate with left sided type 3 poland syndrome with dextrocardia

Poland syndrome (PS) is right sided in 75 percent of cases. Only 10 percent are associated with dextrocardia. It is usually detected late for the milder nature of the condition. Only in severe cases it comes to attention during neonatal period. Left sided Poland syndrome with dextrocardia is extremely rare. To the best of our knowledge this is the first case of left sided type 3 Poland syndrome with dextrocardia diagnosed at birth with chest wall getting sucked in and bulging out during inspiration and expiration respectively

Features of surgical correction of various forms of hand syndactyly in children. Retrospective study of own treatment experience

Syndactyly is a congenital malformation which is characterized by impaired differentiation of upper extremity tissues. Surgical correction of syndactyly is aimed to achieve satisfactory cosmetic and functional result. Most often, elimination of the total syndactyly form of the fingers implies is achieved by techniques according to Flatt (1962), Cronin (1943), Gilbert (1986), Wood (1998), bone form requires usage of Buck-Gramko technique. Objective. To conduct a retrospective study of surgical treatment results in patients with various forms of hand syndactyly. Methods. The study included 84 patients (109 hands) with hand syndactyly who were operated during the period from 2012 to 2020 in the pediatric orthopedics clinic of the Sytenko Institute of Spine and Joint Pathology National Academy of Medical Sciences of Ukraine. The mean age of patients was 6.5 years (1 to 16), 39 (46.4 %) boys and 45 (53.6 %) girls. Most often syndactyly of III–IV fingers (105 (96.3 %) hands) was managed by the Wood method, namely in 63 (60.0 %) hands and 8 (7.6 %) cases with severe bone forms were corrected by Buck-Gramko method. Rotational skin pieces Ghani and Buck-Gramko were used for surgical correction of I–II fingers syndactyly. Treatment results were evaluated by the Vancouver Scar Scale (VSS). Results. According to VSS, the treatment result was classified as satisfactory in 73 (67.0 %) hands. Complications were noted in 11 (10.1 %) cases: 2 patients (18.2 % of 11) with congenital amniotic membranes were found to have lysis of a free skin piece; 1 (9.1 %) after removal of the bony syndactyly form had deviation of the nail phalanx; 3 (27.3 %) with Poland-syndrome were shown to have scarring of the interdigital space; 5 (45.4 %) with a complex bony form of syndactyly further on developed pulling scars, which caused deformity of the fingers and resulted in a correction in the form of multistage Z-plastics. Conclusions. All the patients showed improvement in the function and cosmetic results of the hand at the end of treatment. The best results were obtained in the case of simple and total forms of syndactyly treated with Wood technique.

Сoronary Artery Bypass Grafting in a Patient with Congenital Chest Abnormality with Poland Syndrome

Introduction: A rare clinical case of coronary artery bypass grafting in congenital chest abnormality with Poland syndrome is described. This syndrome is a congenital anomaly, which is characterized by a combination of unilateral aplasia or hypoplasia of the sternocostal head of the pectoralis major muscle with ipsilateral brachysyndactyly in the classic version. The cause of Poland syndrome is unknown, but it is believed that it can form with a short-term decrease in blood flow in the subclavian and vertebral arteries. Case Presentation: The presented case shows the possibility of successful completion of coronary artery bypass grafting in Poland syndrome. Revascularization by transluminal balloon angioplasty with stenting reduces the risk of postoperative complications. However, given the degree of damage to the coronary bed and myocardium, in some cases, the patient needs to undergo only coronary artery bypass grafting. The presented case shows the possibility of successful completion of coronary artery bypass grafting in Poland syndrome. Conclusions: We believe that, despite the high risks of postoperative complications, patients with Poland syndrome can be recommended to perform a planned operation of coronary artery bypass grafting, including access through a median sternotomy.

Development of A Surgical Treatment Algorithm for Breast Reconstruction in Poland Syndrome Patients Considering Severity, Sex, and BMI

Introduction: Poland syndrome is a rare, challenging combination of chest wall and breast deformities for reconstructive surgeons and selecting the treatment can prove difficult. This study aims to help surgeons in choosing the best viable option for treatment by sharing our institutional experience and proposing a guiding algorithm. Methods: A retrospective analysis of all patients with Poland syndrome undergoing treatment for breast and chest wall deformities at a single institution between December 2011 and May 2020 was performed. Medical charts were reviewed to allow for a description of patient demographics, treatment modalities and complications. A treatment algorithm to aid in selecting the adequate reconstructive option based on our institutional experience was formulated. Results: A total of 22 patients (six male, 16 female) were identified who received treatment for Poland Syndrome related deformities. Nine received microsurgical free flap reconstruction (three Deep Inferior Epigastric Perforator flaps, six Transverse Myocutaneous Gracilis flaps), two received reconstruction with a local flap (two Latissimus dorsi flaps), nine received implant based reconstruction, and two were treated with autologous free fat transfer only (17 in combination with other surgical methods). Conclusion: Free flap reconstruction with the TMG flap is a valid option for patients with low Body Mass Index (BMI), while Deep Inferior Epigastric Perforator flaps should be considered for patients with a higher BMI. Autologous free fat transfer proves to be a safe and efficient treatment option in mild cases of Poland syndrome for male and female patients, in combination with or without implant based reconstructive surgery. Multicentre studies should be conducted to achieve higher case numbers of this rare disease and support clinical decisions with more data.

Poland Syndrome Associated with Lung Cancer: A Case Report and Literature Review

Background: Poland syndrome is a rare congenital disease consisting a spectrum of defects occupied by unilateral chest wall hypoplasia and ipsilateral hand abnormalities. It is also closely associated with some malignant diseases and some benign diseases, such as breast cancer, lymphoma, recurrent spontaneous pneumothorax, perforating dermatosis, hemangioma, gastric cancer and lung cancer. The low incidence of Poland syndrome has hindered investigators’ attempts to develop randomized prospective trials, so the disease is poorly understood. Now We find out a case of Poland’s syndrome associated with adenocarcinoma of right lung, in which before this article there was only article depicting a case of lung squamous cancer with poland syndrome, this is an interesting topic. To the best of our knowledge, this is the second case of poland syndrome closely related to lung cancer.Case presentation: A 61-year-old man came to the hospital accompanied by intermittent dry cough and hemoptysis that lasted for 2 months. Chest CT revealed showed a large lobulated mass about 5.5cm × 4.0 cm × 3.0 cm in the right lower lung field and the normal right pectoralis major muscle and pectoralis minor were both absent. we diagnosed the patient with stage IIIa lung adenocarcinoma. After two cycles of preoperative platinum-doublet chemotherapy, we have performed a regular right lower lobectomy and lymph node dissection. Until now, after 2.5 years of follow up, he did not relapse.Conclusions: In addition to some other reported types of malignancies, there is increasing evidence that an association between these two clinical entities. Although we do not understand the underlying mechanisms, we assume there exists some common gene pathways that regulate these two clinical entities. Because these patients who got Poland syndrome are susceptibility to different kinds of malignancies, longitudinal epidemiological studies may be needed.