Repair of chest wall defect in Poland syndrome

In 1841, Poland first described congenital pectoralis major and pectoralis minor muscles with and without deformity. It was not until 1962 that such malformations were called Poland syndrome. The malformation of this syndrome also includes sternal head dysplasia of the pectoralis major and pectoralis minor muscles, and pulmonary sputum in the anterior portion of the second to fifth ribs and the costal cartilage defect. When inhaling, the defect of the right thoracic cartilage is inwardly depressed; when exhaling, the right thoracic cartilage defect is convex outward. Mild dysplasia of the breast, to the complete absence of the breast, lack of nipples. There is less subcutaneous fat in the chest and no hair. Hand deformities include palm deformity, short fingers, and finger deformities; small scapula, elevation, and wing deformity. The syndrome is relatively rare and the estimated incidence is 1 in 3 million. Poland syndrome can also be associated with a second rare syndrome, bilateral or unilateral hernia and ocular abductor paralysis, called Mobius syndrome. Shamberger et al reported 75 cases of Poland syndrome, 40 males, accounting for 53.3%, 35 females, accounting for 46.7%; 44 patients on the right, accounting for 58.7%, 30 patients on the left, accounting for 40%; 1 case on both sides, accounting for 1.3%; combined hand deformity accounted for 66.7%; combined breast deformity accounted for 49.3%. The chest of the child was inwardly depressed, the sternum was rotated to the affected side, and the opposite side was convex and deformed, accounting for 32.0%. Determining the extent of chest wall muscle and bone defects is important for the design of the chest wall reconstruction and the choice of repair materials. Treatment of diseases: chest wall soft tissue malformations Indication Poland syndrome chest wall defect repair is applicable to: 1. The rib cartilage supporting the thoracic cage has a large invagination or defect, and should be surgically repaired in order to reduce the thoracic invagination and eliminate the abnormal breathing of the chest wall. 2. The costal cartilage defect, the sternum is rotated to the affected side, with or without contralateral costal cartilage. 3. Female patients, lack of breast on the affected side, can perform breast angioplasty at the same time. 4. The chest and small muscles are limited to the sternum end. Those without functional influence do not need to do chest wall repair. Preoperative preparation Comprehensive examination of the extent and extent of chest and hand deformities, collaboration with the Department of Plastic Surgery, staging the correction of chest and hand deformities. Surgical procedure Intratracheal intubation combined with anesthesia. In the supine position, if the latissimus dorsi muscle flap is to be used, the lateral position is used. Incision The median longitudinal incision of the sternum or the fourth rib level between the two nipples and the transverse incision in the lower breast fold. Cut the skin, subcutaneous tissue, muscle, free flap and free chest muscle flap from the lateral side of the sternum, retaining the intact thoracic and small muscles. 2. Exposing costal cartilage Lack of costal cartilage, chest muscle dysplasia, due to the chest fascia, deep chest fascia to the skin is very thin, should be very careful when separating, do not separate this layer into the chest. 3. Correct the sternal rotation inward deformity The sternum is generally rotated into the affected side, and the wedge-shaped sternum osteotomy is transversely offset under the second costal cartilage, and the sternum plate is cut 5 to 8 mm, and then sutured with a thick thread. 4. Subperiosteal cartilage resection Bone grafts should be given to patients with large areas of costal cartilage defects. The contralateral rib cartilage protruded from the chest, and the subperiosteal cartilage was removed. The method was the same as the funnel chest surgery. If the costal cartilage defect is 2 to 4, the 5th and 6th ribs are taken from the opposite side, and the rib is split along the longitudinal axis to maintain the maximum mechanical support force, and the suture is fixed between the broken costal cartilage and the sternum. 5. latissimus dorsi muscle flap transfer Women with breast deficiency, can do breast angioplasty at the same time. Large chest and small muscle absent, can use the latissimus dorsi muscle transfer muscle flap, directly with the subclavian, sternal fixation, covering the ribs, correcting the absence of the chest muscle and subclavian depression. In the absence of female breasts, artificial materials are implanted under the latissimus dorsi to complete the mammoplasty. complication 1. The skin and muscle flap are necrotic due to insufficient blood supply. 2. Breast implants are implanted with artificial prosthesis and need to be removed.

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