1、Congenital Breast Malformations,1,1,Summary,The expression of the abnormality may be complete,and anatomic components are often missing,deformed, or weakened Most breasts arenaturally asymmetrical. These asymmetriesmay include discrepancies in breast size andshape, location of the nippleareola compl
2、ex,the inframammary fold, or skeletal abnormalities Preoperative evaluation begins with athorough medical and surgical history, particularlyrelated to breast disease.,1,2,Abbreviations,DIEP Deep inferior epigastric perforator flap 腹壁下动脉穿支横行下腹部皮瓣 LDF Latissimus dorsi muscle myocutaneous flap 背阔肌肌皮瓣 S
3、GAP Superior gluteal artery perforator flap 臀上动脉穿支皮瓣 TRAM Transverse rectus abdominis myocutaneous flap 横形腹直肌肌皮瓣,3,1,Polands Syndrome,Polands syndrome was first described by AlfredPoland as a medical student in London in 1841 临床症状集中于躯体及上肢，男性多见，一般为单侧，极少双侧发病。最轻度者仅为胸大肌的胸骨头部缺损和第34指并指畸形。严重的病例除整块胸大肌外，还涉及其
4、下的胸小肌、前锯肌、肋间肌，甚至其邻近的部分背阔肌、腹外斜肌，乃至前胸部的部分肋骨、肋软骨。有的还表现为胸部反常呼吸、肺疝出、肩胛骨高位、患部皮肤和皮下脂肪发育不良，以及乳头高位，或女性乳房发育小或无乳房。手部畸形表现为不同类型的并指、短指、缺指、24指中节指骨缺损、手指深浅屈腱融合、腕骨融合、尺桡骨融合等。个别病例还可伴有耳廓畸形、半椎体、脊柱侧凸、肾畸形、隐睾等。,4,Polands Syndrome,右侧乳房缺如,poland综合征中的手.,5,Polands Syndrome,表现：胸壁畸形和肋骨凹陷，有时可以偶然的伴有背阔肌及前锯肌、肋间外斜肌的缺如 遗传：胸大肌的缺如的发生率是在7
5、000分之一到十万分之一。同时男性的发生率是女性的2到3倍。 遗传导致的右侧发生率为6075% 病因：循环缺陷学说-锁骨下动脉在妊娠期的发育异常， 锁骨下动脉的直径过小及血流减小均导致其发生。 它会导致一系列的外形及功能缺陷。,6,The surgical correction of Polands syndrome deformity,It requires addressing four separate issues:Addressing the chest wall deformity;Addressing the breast deformity in females ;Surgic
6、al correction of any upperextremity deformities, lastly;Nippleareola reconstruction.,7,Poland综合症治疗的目的是达到乳房对称,矫正胸壁畸形，回缩前腋窝皱襞,使得女性锁骨下区域在美观上更加丰满。 大块的肌瓣的转移可以达到使得女性乳房外形缺陷的掩盖。如背阔肌肌瓣转移，因此背阔肌及皮瓣被用来做乳房重建。 背阔肌在男性很适合用来重建胸大肌，在女性可以用来提供覆盖假体或者扩张器的组织。,The surgical correction of Polands syndrome deformity,8,治疗时间的选择,
7、在女性青春期时可以置入一个扩张器临时来使得外形改善，如贝克假体。以后可以等完全发育后放入永久性的假体置入物. 重建的时间选择是，肌瓣转移的时间可以放在5-6岁学龄前期，以防止影响患儿的心理成长。 女性在背阔肌转移后，如果没有放置假体或者扩张器，会使得乳房外形看起来不是很丰满。如果患者的组织缺陷很大，年龄过大，并且腹壁组织明显松弛，可以应用横形腹直肌肌皮瓣。深腹壁下穿支皮瓣，腹壁下浅动脉皮瓣转移来修复。,9,名 称 供血动脉 穿过的肌肉腹直肌肌皮瓣 TRAM 腹壁上动脉 腹直肌（上段）背阔肌肌皮瓣 LDF 胸背动脉 背阔肌腹壁下动脉穿支皮瓣 DIEP 腹壁下动脉 腹直肌（下段）臀上动脉穿支皮瓣
8、SGAP 臀上动脉 臀大肌,常用穿支皮瓣,10,背阔肌肌皮瓣 LDF,背阔肌皮瓣手术常因皮瓣容量不足，需要在皮瓣下再放置人工假体,11,背阔肌肌皮瓣 LDF,实际上poland综合征也包括背阔肌的发育不全。游离皮瓣移植术可以提供更好的效果。,12,腹壁下动脉穿支皮瓣 DIEP,13,腹壁下动脉穿支皮瓣 DIEP,腹壁下动脉穿支皮瓣 修复乳癌术后,14,臀上动脉穿支皮瓣 SGAP,15,臀上动脉穿支皮瓣 SGAP,臀上动脉穿支皮瓣修复骶尾部创面,16,TRAM,TRAM Transverse rectus abdominis myocutaneous flap 横形腹直肌肌皮瓣,17,Two-s
9、tage minimally invasive technique,Borschel described a that requires only asingle small incision in the axilla for the muscle dissection and implant placement. In the first stage of this technique, a tissue expander is placed endoscopically to increase the size of the skin envelope. After a period o
10、f expansion. At a second stage, the tissue expander is removed endoscopically and a permanent saline implant is placed. The latissimus dorsi muscle can then be transferred.,18,A separate deformity from Polands syndrome was described by Spear et al. in 2004, and it was coined the anterior thoracic hy
11、poplasia syndrome.This deformity and Polands syndrome share similar traits,19,Axillary Breast 副乳,20,Axillary Breast 副乳,It can be found in unusual locations such as thescapula, thigh,posterior leg, or the labia majora. It includes fibrocystic disease,mastitis, fibroadenomas,atypical hyperplasiaand ca
12、rcinoma Marshall in 1994 reported anincreased incidence of cancer in aberrant breast tissue.,21,Axillary Breast 副乳,Axillary breast tissue presenting as a palpable axillary mass can undergo monthly menstrual changes as well as tenderness, swelling,difficulty with shoulder motion and irritation.All sy
13、mptoms may be aggravated, becoming more apparent during puberty and pregnancy.,22,Axillary Breast 副乳,It suggests that aberrant tissue may be at increased risk for malignant change. The treatment of axillary breast tissue is elliptical excision. It has also been reportedly removed with ultrasound-ass
14、isted liposuction to avoid a large scar.,23,Polythelia,24,Polythelia,25,Polythelia,Supranumerary nipples or polythelia can be found along the line from the axilla to the groin. A remnant of milk lines after improper regression of the mammary ridges at approximately 3 months of gestation causes acces
15、sory nipples.,26,Polythelia,In approximately 50% of the patients, it can be bilateral.sporadic polytheliamay be associated with other congenital deformities including kidney urologic abnormalities.,27,Congenital Absence of the Breast -amastia,Isolated absence of the breast was first described by Fro
16、riep in 1839.Most cases of amastia are a component of other developmental syndromes such as UllrichTurner (乌尔里希特纳综合征)syndrome-又名性腺发育障碍症 Or the AREDYLD syndrome,28,Amastia,Breast reconstruction techniques undertaken are similar to those used in breast cancer surgery. It uses the transverserectus abdominus myocutaneous flap or latissimus dorsi flap plus an implant. Tissue expansionwith subsequent placement of a siliconeimplant is also a viable option.,29,
