小儿同足趾顺行趾动脉神经蒂皮瓣的临床应用

目的报道小儿同趾顺行趾动脉神经蒂皮瓣的临床应用。方法2008年1月至2013年6月我们对8例拇趾端（腹）缺损的患者应用同足趾顺行趾动脉神经蒂皮瓣进行修复。其中趾端缺损3例,趾腹缺损5例,皮肤缺损面积在1．0cm×1．5cm～1．2cm×1．8cm。年龄为3～12岁,平均年龄5岁。结果皮瓣全部成活,无动、静脉危象发生。经1个月至半年随访,皮瓣外形饱满,色泽、供受区仅有线样瘢痕,趾端感觉正常,无不耐寒及趾端疼痛病例,患儿步态正常。结论同趾顺行趾动脉神经蒂皮瓣是修复小儿拇趾趾端（腹）缺损的良好方法。     

手术治疗婴幼儿先天性并指畸形35例

目的 探讨婴幼儿先天性并指畸形的治疗时机、手术方法,以提高疗效.方法 对35例婴幼儿各类先天性并指畸形于9个月～3岁行手术治疗,并指间皮肤采用多Z形锯齿状切口,根据并指相连皮肤软组织量采用掌背侧M-V形皮瓣或背侧矩形皮瓣重建指蹼.指侧创面应用掌背侧Z形切口形成的皮瓣交叉缝合,皮瓣覆盖不全创面行全厚皮瓣移植.结果 全部病例术后手指血运良好,皮瓣全部成活,4例指侧皮瓣部分坏死,经换药后愈合.手指及指蹼外形满意,手指功能明显改善.31例患儿随访2个月～4 a,2例指蹼变浅,2例因指侧瘢痕挛缩出现手指侧屈畸形,余病例患指外形、功能良好,指蹼外形接近正常.结论 先天性并指畸形的手术治疗在低龄儿童进行是可行的,早期合理治疗可恢复手部的外观及重建功能.     

扩大的足背动脉内侧皮瓣修复儿童足跟及其周围软组织缺损

目的 探讨扩大的足背动脉内侧皮瓣修复儿童足跟部皮肤缺损的效果.方法 2004年8月至2008年12月,共10例患儿足跟及其周围软组织缺损,男8例,女2例,年龄4～10岁.均为创伤所致.创面大小约6.5 cm×4.0 cm～9.5 cm×4.0 cm.10例小儿足跟及其周围软组织缺损采用对侧扩大的足背动脉内侧皮瓣修复.7例皮瓣行交腿皮瓣转移修复,3例皮瓣行游离移植,供区创面采用游离植皮修复.术中把皮瓣内隐神经与受区隐神经吻合.结果 扩大的足背动脉内侧皮瓣范用约7.0 cm×4.5 cm～12.0 cm×4.5 cm.所有皮瓣均完全存活.8例获随访9～18个月,再造足跟两点辨别觉5～9mm,外形良好、无溃疡发生.结论 扩大的足背动脉内侧皮瓣可修复对侧足跟及其周围软组织缺损.该皮瓣是隐神经的绝对支配区,移植后可重建精细感觉.     

穿支皮瓣修复儿童指趾末端缺损

目的探讨穿支皮瓣在儿童指（趾）末端缺损治疗中的应用价值。方法根据手足的血运特点,结合伤口形状,使用指（趾）动脉的穿支皮瓣,修复儿童手指、足趾缺损7例,观察术后恢复情况,评价动脉穿支皮瓣的临床应用效果。结果7例患儿皮瓣均完全存活,术后手指、足趾外形良好,触觉正常。结论合理使用穿支皮瓣,可以较好修复儿童指（趾）末端缺损,具有一定的临床推广意义。     

游离踇趾腓侧皮瓣移植修复儿童手部创伤性软组织缺损

目的 探讨游离踇趾腓侧皮瓣移植治疗儿童手部创伤性软组织缺损的疗效及其应用价值。方法 2013年7月至2018年4月对11例因机械外伤导致的手部软组织缺损患儿,采用游离踇趾腓侧皮瓣移植的方法修复创面。其中,男10例,女1例;年龄5～17岁,平均11.5岁;移植皮瓣面积最小3.0 cm×1.5 cm,最大5.5 cm×2.5 cm。将第一跖背动脉(或足背动脉)与桡动脉吻合,皮瓣供区创面用腹股沟全厚皮片游离植皮。结果 本组平均手术时间3.5 h,均吻合"一动两静",术后发生血管危象1例,及时手术探查,最终11例皮瓣均完全成活。3例出现皮瓣供区植皮坏死,经换药后痂下愈合。经平均10个月随访,皮瓣外形满意,色泽正常,弹性、质地良好,与受区周围组织基本一致。根据Michigan手功能评价,本组11例对外观均"非常满意";7例对伤指功能评价"非常满意",2例"满意",另有"不满意"和"非常不满意"各1例。皮瓣供区足趾运动功能无明显障碍。结论 在熟练掌握显微小血管吻合技术及重视儿童围手术期综合管理的基础上,游离踇趾腓侧皮瓣移植修复儿童手部软组织缺损取得满意疗效,受区美观,供区隐蔽,且手指及足趾功能良好...     

Repair of soft tissue defect in the heels of children with the perforator flaps of the posterior tibial artery with nerve anastomosis

目的 评价胫后动脉穿支皮瓣带神经修复小儿足跟软组织缺损的临床效果.方法 应用胫后动脉穿支皮瓣带神经修复9例足跟负重区软组织缺损,年龄:4～12岁(平均7.5岁).切取皮瓣面积最大12 cm×6 cm,最小6 cm×4.5 cm.供区创面行Ⅰ期全厚网状游离植皮.皮瓣携带的隐神经在足跟部与腓肠神经吻合.结果 所有的皮瓣完全存活,取得了满意的临床效果.随访1.5～4年,平均随访2.5年,皮瓣恢复了感觉,两点分辨觉为30～33 mm,受区未发生溃疡.结论 胫后动脉穿支带蒂皮瓣具有血管解剖恒定、血运丰富以及操作简单等优点,适宜修复小儿足跟负重区软组织缺损.     

Application and efficacy of dorsal metacarpal island flap on reconstruction of the web-space in congenital syndactyly

目的 探讨掌背岛状皮瓣重建先天并指指蹼的应用和临床疗效.方法 2009年2月至2011年1月,采用掌背岛状皮瓣重建小儿先天并指指蹼9例.所有患儿采用并指掌背侧皮肤锯齿状皮瓣分指,交叉缝合重建手指,掌背岛状皮瓣重建指蹼.结果 本组9例患儿创口Ⅰ期愈合,重建指蹼的掌背皮瓣全部存活,指体侧方的锯齿形瓣全部存活.手指无瘢痕挛缩,手指活动范围较健侧无明显减小,成形指蹼外形良好,有近似正常的指蹼斜坡及指蹼沟,指蹼最大外展度达40°,患儿家长表示满意.结论 采用掌背岛状皮瓣重建先天并指畸形指蹼的矫形手术方法,能够得到的理想的指蹼外观及功能,避免植皮,疗效满意.     

Pennig微型支架治疗儿童单纯性并指症的临床疗效观察

目的 探讨Pennig微型支架治疗儿童单纯性并指的临床效果.方法 我科自2014年3月至7月共收治5例单纯性并指患儿,女2例,男3例,左手3例,右手2例,均采用Pennig微型支架行并指间皮肤牵伸,Ⅱ期行并指皮肤分离成形术.结果 5例患儿均获得随访,平均随访时间为13.5个月(12～16个月),5例患儿通过Pennig微型支架行并指间皮肤牵伸均获得充足皮源,2期手术不需游离植皮,治疗过程中未出现感染、皮肤坏死及延迟愈合或不愈合等症状,术后功能及外观较为满意,评分如下:根据Vancouver瘢痕量表分析,1例总分为2,余4例为0,总分平均为0.8;5例患儿指蹼爬移程度均为0级;手指TAM值5例均为优;根据视觉模拟量表,5例患儿术后疼痛均为0分,外观平均得分为1分,功能平均得分为1.2分.结论 基于张力-应力法则,我们使用Pennig微型支架缓慢牵张并指间皮肤,由此获得充足皮源,Ⅱ期手术分离并指可避免损伤并指血供及游离植皮,降低皮瓣坏死概率,改善手指功能,缩小瘢痕形成,为治疗并指的一种有效方法,且其简易、轻巧易于使用,值得临床借鉴.除此之外Pennig微型支架在治疗并趾、复杂性并指(趾)等畸形亦有潜力,需要进一步探索.     

儿童面部黑色素痣诊治379例

目的回顾性分析2010年1月至2014年12月在本院经手术治疗的379例面部黑色素痣患儿临床资料,总结儿童面部黑色素痣的手术治疗经验。方法379例中,男175例,女204例;年龄44 d至16岁。采用3种术式:单次(235例)或分次(71例)切除缝合,切除加全厚植皮(38例),切除加皮瓣修复,邻近皮瓣转移(34例),1例左面部巨痣行痣大部分切除加股前外游离皮瓣转移修复。采用皮肤科医师和患儿家属双盲主观评价疗效,评价内容为切口瘢痕、面部畸形、色差。结果除1例股前外游离皮瓣转移患儿游离皮瓣吻合口栓塞改为全厚植皮外,所有患儿均完整切除面部黑色素痣,切口Ⅰ级愈合。所有单次或分次切除缝合、临近皮瓣转移者未出现明显瘢痕增生或挛缩,皮肤色泽和弹性与正常皮肤接近,除部分邻近皮瓣转移患儿家长对附加切口不满意外,均获得满意评价。全厚植皮术者植皮区创缘发生轻度瘢痕增生,植皮区色素沉着。所有患儿未继发面部畸形。结论儿童面部黑色素痣的手术设计需兼顾其所处面部美容单位、面积大小,遵循面部美容单位自然的分界线及面部皱纹线走向,选择适宜的切除缝合、皮瓣转移、全厚植皮等术式进行修复,术后可获得较好的美容效果。     

趾短屈肌肌皮瓣修复儿童足跟部软组织缺损伴跟骨外露

儿童足跟部创伤较少见,由于特殊的解剖特点和功能,其皮肤和软组织缺损的修复成为临床一个重要而困难的问题.处理不好,常遗留足跟部软组织缺损、慢性溃疡、跟骨外露继之发展成慢性骨髓炎,严重者可致患肢负重功能丧失.既往常采用"桥式"交腿皮瓣,小腿后侧逆行岛状皮瓣、小腿前外侧逆行岛状皮瓣、胫后动脉岛状皮瓣转移或转位、足底内侧岛状皮瓣等方法修复."桥式"交腿皮瓣法的病程较长、患儿痛苦大,后几种方法的皮肤耐磨性不好、皮肤感觉恢复欠佳.2001年5月至2007年10月间,作者应用趾短屈肌肌皮瓣修复3例儿童足跟部软组织缺损伴跟骨外露,取得满意疗效,报告如下.     

Dose-related Induction of Digital Malformations in Rats by 5-Azacytidine: Description of the Defects

Abstract Dose-related changes of 5-azacytidine (5-AC) induced digital malformations are reported. Dosages of 0.4, 0.5, 0.6, 0.7, and 1.0 mg/kg of 5-AC were injected intraperitoneally into pregnant rats on day 13 of gestation. The susceptibility order of each digit was the second > third > first > fourth > fifth in the forepaw and the second > first > third ė fourth > fifth in the hindpaw. In the forepaw, the percentages of cutaneous syndactyly with closely apposed metacarpals or osseous syndactyly between the first and second digits (type FA defect) and brachydactyly of the third digit (type FB defect) were increased in a clear dose-related trend. In the hindlimb, the percentages of osseous or cutaneous syndactyly between the first and second digits (type HA defect), brachydactyly or ametatarsia of the second digit (type HB defect), and osseous or cutaneous syndactyly, closely apposed metacarpals, lateral metacarpal fusion, or osseous or cutaneous syndactyly with lateral metacarpal fusion or closely apposed metacarpals between the third and fourth digits (type HC defect) reached a maximum at 0.7 mg/kg of 5-AC and then decreased. However, the percentages of ectrodactyly, 1 missing digit (type M1 defect) or 2 missing digits (type M2 defect), were increased from 0.6 mg/kg or 0.7 mg/kg, respectively. Ectrodactyly, missing 3 digits (type M3 defect), was rarely observed in the dose range used.     

Case of Complex Craniofacial Anomalies, Bilateral Nasal Proboscides, Palatal Pituitary, Upper Limbs Reduction, and Amnion Rupture Sequence: Disorganization Phenotype?

We report a case of a dizygotic twin with complex abnormalities of head, body, and limbs. The anomalies include the following: lateral and midline cleft upper lip, ectopic palatal pituitary, natal teeth, bilateral nasal proboscides with an absent nose, left microphthalmia with conjunctival-lined cyst, right ocular dysgenesis, bilateral retinal dysplasia, platybasia with skull asymmetry, hydrocephalus secondary to aqueductal atresia, brain hemispheric asymmetry with a parietal–occipital cortical flap, agenesis of posterior corpus callosum, absence of the olfactory nerves and left anterior cerebral artery, leptomeningeal and intraventricular heterotopias, right radial longitudinal terminal meromelia with constriction rings of fingers, partial syndactyly of the third and fourth left fingers, dorsiflexed great toes and pes equinovarus bilaterally, and multiple skin tags with a sacral appendage. Additionally, this twin's placental disc and extraplacental membranes were devoid of amnion. We regard these anomalies as a possible expression of the human homologue of the disorganization phenotype or another gene mutation. Nevertheless, an abnormality of blastogenesis with early damage to organizing tissues of the frontonasal region and limbs, or a vascular disruption, cannot be excluded. Early amnion rupture sequence (possible extraamniotic pregnancy with amniotic bands, limb reduction defects with Streeter bands, and multiple skin tags tapering into amniotic bands) was also present in this case, and may have acted as a contributing factor. Received August 12, 1999; accepted January 26, 2000.     

小儿隆突性皮肤纤维肉瘤的临床特征与外科治疗方案

目的 研究小儿隆突性皮肤纤维肉瘤的临床特征与外科治疗方案.方法 以我院2010年1月至2015年12月间治疗的21例隆突性皮肤纤维肉瘤患儿为研究对象,分别调取所选病例的临床资料,并对其进行回顾性分析.本组病例都接受外科手术治疗,若病理切缘呈阳性需要继续补切至切缘呈阴性,分析21例患儿隆突性皮肤纤维肉瘤的临床特征与外科治疗方案.结果 21例患儿均选择手术治疗,其中有12例患儿选择扩大切除术,9例患儿选择扩大切除术联合植皮、转移皮瓣修补术,术后有3例患儿联合放疗治疗.有4例患儿复发,有1例患儿死亡,死亡原因为肿瘤转移.结论 隆突性皮肤纤维肉瘤属于皮肤恶性肿瘤的一种,具有极高的复发率,选择外科治疗方案彻底切除具有重要作用,是临床治疗隆突性皮肤纤维肉瘤的首选方法,同时配合放疗也能提高治疗效果,而肿瘤复发和转移都会影响患儿手术预后效果,外科治疗方案值得推广应用.     

Kasabach-Merritt综合征治疗中自体原位植皮术的应用

目的 对Kasabach-Merritt综合征手术治疗过程中使用自体原位植皮术,评价其治疗效果,提出一种新的手术治疗方式.方法 回顾性分析2010年至2015年间自体原位植皮手术治疗的Kasabach-Merritt综合征患儿23例,病例纳入标准:均为瘤体分布弥散,皮肤大面积(＞5 cm×5 cm)异常,手术切除瘤体后创面缝合困难;非手术治疗方法无效;血小板值在2×109/L～31×109/L.23例Kasabach-Merritt综合征患儿瘤体均位于四肢,上肢6例,下肢17例;男9例,女14例;年龄7 d～9个月.总结该手术治疗方法的手术要点及术中、术后处理,分析治愈率及术后并发症.术后均经病理学检查证实为卡波西样血管内皮瘤(kaposiform hemangioendothelioma,KHE)20例,丛状血管瘤(tufted angioma,TA)3例.结果 23例患儿均在术后1周内血小板值恢复正常,随访1～5年,治愈率为100％;18例患儿自体皮片原位移植全部成活,有3例成活面积约90％,经后续换药处理后愈合,2例原位皮片移植成活约60％,行二期皮肤移植修补手术后治愈,术后并发症少.结论 手术治疗Kasabach-Merritt综合征可作为有效的治疗方法,本组资料显示,自体原位植皮术作为一种新的手术方式,治愈率100％,术后并发症少,其对符合适应证者是一种可选择治疗手段.     

分期Duckett术式治疗重度尿道下裂的疗效评价

目的：通过与一期 Duckett 术式手术效果进行比较,探讨分期 Duckett 术式治疗重度尿道下裂的应用及价值。方法2009年1月至2014年10月,我们收治重度尿道下裂72例,均为阴茎阴囊交界型或会阴型尿道下裂,患儿第1次手术时年龄2～3岁,平均2.4岁。其中38例采用管形包皮岛状皮瓣法一期尿道成形术,即一期 Duckett 术式,为 A 组,成形尿道长度4～6 cm。34例采用分期手术,一期手术中矫正阴茎下弯后,采用管形包皮岛状皮瓣法成形部分尿道,即分期 Duckett 术式,为 B 组;第一期成形尿道长度为3～4.5 cm;术后1年行二期尿道成形术,成形尿道长度1.5～2.5 cm,两期手术成形尿道总长度4～6 cm。结果两组均获随访,术后随访6个月至5年,平均3.5年,无一例阴茎下弯复发。A 组有7例（7／38,18.5％）术后发生尿瘘。B 组有3例（3／34,8.8％）术后发生尿瘘,均出现在二期尿道成形术后。A 组有4例出现尿道狭窄（4／38,10.5％）,B 组1例出现尿道狭窄（1／34,2.9％）。A组有4例出现尿道憩室（4／38,10.5％）,B 组无一例尿道憩室发生。A 组手术成功率为60.5％,B 组手术成功率为88.3％,差异有统计学意义（P ＝0．008）。结论与一期 Duckett 术式相比,分期 Duckett术式治疗重度尿道下裂,术后并发尿瘘、尿道狭窄和尿道憩室少。分期 Duckett 术式有效降低了重度尿道下裂的手术难度和并发症的发生率,提高了复杂尿道下裂的疗效,具有较好的临床应用价值。     

胫前动脉皮瓣在小儿小腿及足部软组织损伤中的应用

报道８例以胫前动脉为蒂的足背或小腿外侧皮瓣翻转移位修复小儿小腿及足部软组织损伤，所有皮瓣全部成活。该皮瓣的优点是：其血管行走方向恒定，血管蒂有足够长度，皮瓣大小、形态可自由设计选择，血供丰富，血管分支多，适合于各种小腿和足部大面积深部损伤。     

Congenital Hypoplastic Anemia in Six Patients: Unusual Association of Short Proximal Phalanges with Mild Anemia

Six congenital hypoplastic anemia (CHA) patients from five families who have been followed from 2 months to 28 years are presented. Mild hypoplastic anemia in a 13-year-old girl was associated with clinodactyly of the fifth finger on both hands, shortness in the proximal phalanges on all fingers, and syndactyly between the second and third toes and short fourth toe on the right foot. These abnormalities, except for clinodactyly, have not been reported previously in CHA. In one of the five families genetic transmission was thought to be autosomal-dominant since both the father and the son had the disease. Therapy with corticosteroids was initiated in allpatients at the ages of 3.5 months to 13 years. Complete or near-complete recovery of anemia was obtained.     

An alternative way to cover ventral penile skin defect in Mathieu technique.

Mathieu technique is used satisfactorily in distal penile hypospadias without chordee or with minimal chordee. After using this technique, a large defect may sometimes appear on the ventral surface of the penis. To cover the defect, a few techniques, including preputial island flap, Byar's flap and Ombrédanne-Nesbit's flap, are used. We describe a new flap to cover the defect more cosmetically. Twenty-four patients ranging in age from one year to 14 years (median age 6.5 years) were operated on. In the patients for whom the defect could not be covered primarily, a longitudinal incision was made along the midline through the penile shaft skin from penile radix up to the border of preputial skin. The relaxed penile skin, which was incised on the dorsal surface, could be approached and sutured easily on the ventral surface without stretching. The new defect that developed on the dorsal surface was closed with the prepuce matching the defect. Nine patients, two with chordee and seven without chordee, underwent this technique. The cosmetic and functional results were excellent in all patients and none of the patient's parents complained about the cosmetic aspect. Only one fistula complication, which healed spontaneously, developed on the 20th postoperative day. Considering these results, we may conclude that excellent cosmetic results can be accomplished by the use of this flap technique.     

尿道下裂术后尿瘘原因及修补体会

目的 探讨各型尿道下裂术后尿瘘发生原因及修补方法。方法 小尿瘘采用结扎法，切开缝合法，皮瓣覆盖法修复，大尿瘘采用Thiersch法或重新行尿道成形术。结果 123例尿道下裂术后29例出现尿瘘(23．58％)。尿瘘发生后立即修复的成功率(3／9，33．33％)远低于尿瘘发生半年后修复的成功率(14／17，82．35％)。3例半年内自行愈合。结论 尿瘘的发生与尿道成形材料，局部血运及创面感染有关，与术式本身关系不大。瘘口大小、术式选择、修补时间是尿瘘修补成功的关键。