Surgical treatment of congenital syndactyly of the hand

Syndactyly is a congenital anomaly of the hand that is more common in males, is present bilaterally in 50% of affected patients, and often is associated with other musculoskeletal malformations or systemic syndromes. The goal of syndactyly release is to create a functional hand with the fewest surgical procedures while minimizing complications. For simple syndactyly, surgical reconstruction can begin at approximately 6 months, although many surgeons prefer to wait until the infant is 18 months old. Special situations, such as complex syndactyly and involvement of border digits, may warrant surgical intervention earlier than 6 months. Reconstruction of the web commissure is the most technically challenging part of the operation, followed by separation of the remaining digits. Full-thickness skin grafting is almost always required for soft-tissue coverage. Complex syndactyly and syndactyly associated with other hand anomalies warrant special consideration. After reconstruction, patients should be examined periodically until they have achieved skeletal maturity because late complications such as web creep can occur.     

Annular constricting bands.

We reviewed forty-five patients with congenital constriction bands with an average follow-up of 15.8 years. The family history and associated anomalies were recorded and particular attention was paid to temperature gradients and neurocirculatory deficits with respect to band location. No family history of band constrictions was elicited. Most of our patients were products of first pregnancies and of young mothers. Deformity of the nails was a constant finding in patients with distally located bands. All patients demonstrated at least one other anomaly, most commonly a malformation of the hand. Neural deficits were noted in 23 per cent of the patients, while circulatory findings appeared in 16 per cent. A significant temperature gradient averaging 2.38 degrees Celsius was noted in twenty patients in whom the bands were located proximally. All patients with neural deficits demonstrated a significant temperature gradient. Staged z-plasty was the procedure of choice in surgical release of constriction bands.     

上肢先天性绞扼轮综合征的临床治疗

目的 对上肢先天性绞扼轮综合征进行临床分型,探讨一期完全切除环形绞扼轮术式的可行性,总结先天性绞扼轮综合征合并短小指、并指畸形的治疗经验.方法 先天性绞扼轮综合征的部位进行分区,对绞窄的程度进行临床分度.一次完全切除环形绞扼轮,并同时依据临床分型对软组织进行松解、修复.结果 本组11例15侧上肢绞扼轮均予以一期完全切除,术后肢体均未出现血运障碍.随访7个月至3.5年,平均2.1年.功能评价:优9侧占60.0%,良5侧占33.3%,差1侧占6.7%.结论 一期完全切除绞扼轮,对肢体远端的血运不会产生影响,在切除绞扼轮的同时,应根据绞扼轮的临床分型对深部组织予以修复.     

Congenital ring-constriction syndrome of the limbs; a report of 19 cases

The authors report 19 cases of congenital ring-constriction syndrome of the limbs. Complications arose in eight of the pregnancies. The limb anomalies were always congenital ring-constrictions together with distal amputation. Moreover, 13 patients also had syndactyly and 15 associated anomalies were registered in 9 patients--8 of the limbs, 3 craniofacial and 4 visceral. One patient had a family history of hypospadias and cardiac malformation. Treatment usually required several operations, staged Z-plasty being the procedure of choice in surgical release of constriction bands. The acrosyndactylies were released early in life. More extensive fusions required division and skin grafting even if the webs did exist. The aetiology of the condition is still under discussion. The number of cases in the literature that cannot be explained by the exogenous theory of the congenital ring-constriction syndrome is increasing. Among them are four cases in our series.     

96例先天性并趾畸形的临床特点

目的 探讨先天性并趾畸形的临床特点.方法 对2015年1月至2019年6月华中科技大学同济医学院附属协和医院手外科收治的先天性并趾畸形患者的临床资料进行回顾性分析.记录患者性别以及并趾的侧别、临床类型、是否合并其他肢体畸形、累及的趾蹼和累及趾蹼数量等数据,采用SPSS 20.0软件对数据进行统计学分析,P<0.05为差...     

先天性缩窄带综合征并指畸形的临床分型及治疗策略

目的 研究和分析先天性缩窄带综合征并指畸形的临床特点,建立新的临床分型系统并确立相关的治疗策略.方法 回顾性分析2002年2月至2009年8月经治疗的先天性缩窄带综合征并指畸形40例,以临床形态特点为依据,结合既往已有的Patterson分型、Blauth分型,将具有相同或相似解剖形态学改变的归为同一类型,归纳出新的分型特点.新的分型系统包括:Ⅰ型(IA、m),Ⅱ型,Ⅲ型(ⅢA、ⅢB、ⅢC、ⅢD、ⅢE),Ⅳ型,Ⅴ型,以及Ⅵ型.方果 在本分型系统中,包括了既往Patterson分型、Blauth分型系统中的所有内容,同时增加了新的内容,如ⅢC、ⅢD、ⅢE、Ⅳ型、Ⅴ型,并将Blauth Ⅲ型(Ⅰ型)分为两个亚型.方论 新分型系统详细、全面地涵盖了先天性缩窄带综合征并指畸形的临床形态学特征,为合理选择手术治疗策略提供了基础.     

Classification of the pattern of intrauterine amputations of the upper limb in constriction ring syndrome

Twenty patients with congenital upper limb amputations caused by constriction rings were reviewed to classify the pattern of these amputations. In the 20 patients studied, 31 upper limbs had congenital amputations. The pattern of amputation was classified into three types. Proximal upper limb amputation was considered type I and was only seen in one limb. The most common pattern of amputation was digital amputation associated with "coning" or "superimposition" of the digits (type II) and was seen in 20 hands. Type II amputations were subclassified according to the involvement of all, ulnar, radial, or central digits by the constriction ring. In type III amputations (N = 10 limbs), there was no associated coning or superimposition of the digits. This type of amputation was subclassified into type IIIA (multiple-digit amputations within the same hand) and type III B (single-digit amputation). Associated anomalies are reviewed and the pathogenesis of constriction rings is discussed.     

A new technique for correction of congenital constriction rings

BACKGROUND: Congenital constriction ring (CCR) is among the most common congenital anomaly of the extremities. However, few techniques have been described for correction of this anomaly, and none of these techniques could provide a complete elimination of the deformity. Therefore, new surgical techniques are needed for correction of this challenging deformity. PURPOSE: Here, a new technique, namely, Mutaf procedure, for the correction of congenital constriction rings is described. MATERIAL AND METHOD: In this technique, following excision of the fibrotic constriction ring, the groove is filled with the turnover dermofat flaps elevated from both sides of the ring in an alternating pattern. Then skin closure is done with rectangular-plasty technique. Over 8 years, this new technique was used for correction of severe CCRs of the lower extremities in 7 patients aged 1-7 years. RESULTS: In all patients, a normal extremity contour was obtained, with complete elimination of the sandglass deformity caused by the constriction ring. A mean follow-up of 3 years revealed a normal growth of the operated extremity in all patients. The scars were found to be esthetically acceptable. No further surgery was required. CONCLUSIONS: Filling the circular groove caused by the constriction ring with dermofat flaps, this new technique eliminates the soft tissue deficiency and provides a normal extremity contour. Moreover, since rectangular-plasty allows replacing the major limbs of the incisional scars within the relaxed skin tension lines, it provides a better scar in comparison with old Z-plasty techniques.     

Evaluation of the operative treatment of syndactyly.

A review of the records of 61 patients with 176 surgically treated webs was completed to evaluate the results of the different operative techniques used to separate the fingers. The postoperative follow-up period averaged 14 years, with a range from 2 to 38 years. The patients were assessed in two groups: those with major associated anomalies and those in whom syndactyly was the principal anomaly. Recurrence of the webs and flexion and extension contractures occurred more often when split-thickness grafts were used. To obtain a satisfactory result, a second procedure was necessary in 59% of patients with major associated anomalies, and in 30% of the patients who had syndactyly as the principal abnormality. Two types of patients were found to require subsequent operations: children treated prior to 18 months of age and those with complex syndactyly.     

Clinical and roentgenological study of the hands in synbrachydactyly, constriction band syndrome and cleft hand complex

One hundred and six cases of synbrachydactyly, 63 cases of constriction band syndrome and 69 cases of cleft hand complex were clinically and roentgenologically studied and the following results were obtained. Synbrachydactyly is a group of hand anomalies in which the bone reduction appears transversely, and its severest form is a hand missing all the hand bones and the distal portion of the forearm, and with rudimentary digits on the stump. The association of dermal syndactyly or pectoralis muscle defect frequently seen in the milder cases of synbrachydactyly would be a secondary change to mesodermal hypoplasia or a related, but isolated malformation. Cleft hand complex which includes ordinary cleft hand, central ray polydactyly and central ray osseous syndactyly is a group of hand anomalies basically due to an ectodermal abnormality of separation of the digits. However, it is frequently associated with mesodermal hypoplasia or defect of various severity. The hand malformation in constriction band syndrome has a definite characteristic that the area of bone reduction is limited in phalanges.     

Congenital constriction band syndrome

Fifty-five patients, 28 male and 27 female, with congenital constriction band syndrome were reviewed. There were no remarkable abnormalities in the family or neonatal history except 10 out of 35 mothers had colds in early pregnancy and nine had threatened maternal bleeding. Metacarpal length is usually normal in the constricting ring syndrome, but short in the failure of formation defect. There are, however, a small number of patients who have the same features of both congenital hand anomalies. Surgical procedures for constriction ring, fenestrated syndactyly, webbing of the digits, and amputated digits are discussed.     

Non-occlusive ischaemic enteritis

Non-occlusive infarction of the intestine without any associated medical disorder responsible for prolonged hypotension, is a rare clinical entity. A retrospective analysis of 46 cases revealed that the disease occurred predominantly in young, healthy, male patients. The classical presentation was of abdominal pain and persistent tachycardia out of proportion to the abdominal signs of distension, mild tenderness and reduced bowel sounds. The presenting features were of peritonitis due to perforation (63%) and intestinal obstruction (37%). A preoperative diagnosis was suspected only in six cases. Operative findings revealed that the bowel was infarcted without involvement of the mesenteric vessels, which were pulsatile up to the bowel wall. The characteristic findings of the disease, which mainly affected the jejunum (69.6%), were: (i) infarcted area of the bowel surrounded by skip lesions of purple, constriction bands; (ii) multiple small perforations in a paper-thin bowel: or (iii) a long linear perforation. Histologically, ischaemic changes were found to affect the mucosa and submucosa beyond the macroscopic limits. Mortality was high (19.6%).     

Clinical forensic evidence in burns: rescuer burns

In the literature no systematic study is available on rescuer burn for victims of burn injury. This is a retrospective study of nine patients (five admitted and four outpatients) were treated in this hospital as rescuer burns in 3.5 years. All nine patients were males. Average age of the patient treated on outpatient basis was 47 years (ranging between 44 and 52) and total burn area ranged for 1–4%. Average age of the five patients treated on inpatient basis was 32.6 years (ranging between 30 and 34). The total burn area ranged from 14.5 to 38%. During the period of study, in addition to nine rescuer burns, one patient sustained burn before the rescue attempt due to the victim hugging the rescuer.

Based on the study of patterns of burn, these patients were found to have three grades of burn injury: Grade 1—upper extremity involvement only. (A) only one upper extremity involvement, (B) both upper extremities involvement, Grade 2—upper extremity/extremities and face involvement, Grade 3—upper extremity/extremities, face–neck, adjacent chest and lower extremity involvement.     

Vascular disease in the antiphospholipid syndrome: a comparison with the patient population with atherosclerosis.

The antiphospholipid syndrome was diagnosed in 19 of 1078 patients treated between 1987 and 1991. All patients with antiphospholipid syndrome had either anticardiolipin antibody (16/19) or lupus anticoagulant (10/19); three patients had thrombocytopenia, eight patients had a prolonged partial thromboplastin time, and 10 patients had an elevated erythrocyte sedimentation rate. The most common site of involvement was the cerebral circulation (nine patients), manifested by transient ischemic attacks or stroke. Eight patients had upper extremity disease, characterized by symptoms of Raynaud's phenomenon, with angiographic lesions involving the brachial, radial, ulnar, and/or digital arteries. Lower extremity disease occurred in seven patients, with clinical presentations similar to those of atherosclerosis and varying angiographic patterns. In comparison with the population having atherosclerosis, patients with arterial manifestations of antiphospholipid syndrome were more likely to be women (13 of 19 versus 411 of 1078, p less than 0.02), were significantly younger (46.2 years versus 63.6 years, p less than 0.0001), did not smoke (1 of 19 patients versus 700 of 1078, p less than 0.0001), had a higher percentage of upper extremity involvement (8 of 18 versus 13 of 1078, p less than 0.0001), and had a higher incidence of early graft failure (9 of 12 grafts versus 13 of 371 grafts, p less than 0.0001). The syndrome is associated with the repetitive failure of vascular reconstructions and occlusion of native vessels. Antiphospholipid syndrome should therefore be suspected in young, female, nonsmokers with vascular disease, especially those with involvement of the upper extremity, cerebrovascular disease with normal findings on extracranial carotid angiography, and premature graft failure.     

High-grade extremity soft tissue sarcomas: factors predictive of local recurrence and its effect on morbidity and mortality

OBJECTIVE: To identify patient characteristics associated with the development of local recurrence and the effect of local recurrence on subsequent morbidity and mortality in patients with intermediate- to high-grade extremity soft tissue sarcomas. SUMMARY BACKGROUND DATA: Numerous studies on extremity soft tissue sarcomas have consistently shown that presentation with locally recurrent disease is associated with the development of subsequent local recurrences and that large tumor size and high histologic grade are significant factors associated with decreased survival. However, the effect of local recurrence on patient survival remains unclear. METHODS: From 1975 to 1997, 753 patients with intermediate- to high-grade extremity soft tissue sarcomas were treated at UCLA. Treatment outcomes and patient characteristics were analyzed to identify factors associated with both local recurrence and survival. RESULTS: Patients with locally recurrent disease were at a significantly increased risk of developing a subsequent local recurrence. Local recurrence was a morbid event requiring amputation in 38% of the cases. The development of a local recurrence was the most significant factor associated with decreased survival. Once a patient developed a local recurrence, he or she was about three times more likely to die of disease compared to similar patients who had not developed a local recurrence. CONCLUSIONS: Local recurrence in patients with intermediate- to high-grade extremity soft tissue sarcomas is associated with the development of subsequent local recurrences, a morbid event decreasing functional outcomes and the most significant factor associated with decreased survival. Although 85% to 90% of patients with high-grade extremity soft tissue sarcomas are treatable with a limb salvage approach, patients who develop a local recurrence need aggressive treatment and should be considered for trials of adjuvant systemic therapy.     

Symphalangism associated with constriction rings: syndactyly and brachytelophalangy in a black patient

A 7-year-old girl presented with symptoms of constriction ring syndrome, syndactyly, and brachytelophalangy in the presence of proximal unilateral symphalangism. Physical findings revealed previous surgical correction of constriction rings of the right index and long fingers, a short right thumb with a hypoplastic distal phalanx, a shortened long finger with a stiff proximal interphalangeal joint and characteristic absence of skin creases, and incomplete syndactyly of the right first, second, and third web spaces. A brief review of symphalangism and a surgical treatment plan are presented. This is believed to be the first case report of symphalangism in a black patient with multiple congenital anomalies.     

Dorsal sympathectomy for hyperhidrosis--the posterior paravertebral approach

Definitive therapy for hyperhidrosis is sympathectomy. The authors have used a posterior approach to perform 36 dorsal sympathectomies for upper extremity hyperhidrosis in 18 patients (12 female, 6 male). All 18 patients suffered from excessive sweating of the upper extremity (17 palmar, 1 axillary) that caused significant psychological and occupational problems. Eleven patients (61.1%) had lower extremity involvement as well. For all 18 patients conservative medical treatment had failed. Bilateral operations were performed, via a posterior extrapleural approach, through the bed of the third rib. All 36 limbs were relieved of excess sweating. There were no deaths and only two minor wound complications. In no patient did Horner's syndrome develop. Long-term follow-up did not reveal any recurrence of hyperhidrosis. Two patients did complain of compensatory hyperhidrosis of the lower extremities. Dorsal sympathectomy was effective in all of the patients with upper extremity hyperhidrosis in this series. The posterior approach is technically simple, allows simultaneous bilateral operations, and is associated with only infrequent minor complications.     

In utero limb salvage: fetoscopic release of amniotic bands for threatened limb amputation

The natural history of extremity amniotic band syndrome (ABS) is a progression to deformity or amputation. Fetoscopic laser release of amniotic bands in extremity ABS offers the potential to prevent limb amputation. The authors report on 2 patients with isolated extremity ABS who underwent fetoscopic release. Both patients had isolated extremity ABS with compromised extremity perfusion and no other significant structural abnormality diagnosed sonographically. The first patient underwent fetoscopic release of bands of the left wrist, and the second patient underwent lysis of bands around the right wrist and an unsuspected band around the right lower extremity. Both patients had restoration of blood flow by color Doppler to the affected extremity. There were no perioperative complications. In select patients with isolated extremity ABS, early fetoscopic release may prevent amputation and allow improved limb development.     

Lymph node metastasis in soft tissue sarcoma in an extremity

For patients with soft tissue sarcoma in an extremity, the outcome is thought to be poor if lymph node metastasis develops. The purpose of this study was to examine the impact of lymphatic involvement from soft tissue sarcoma on patient survival. Thirty-nine (3.7%) of 1066 patients who had surgery for soft tissue sarcoma in an extremity had lymph node metastases develop. Three (20%) of 15 patients with epithelioid sarcoma, four (19%) of 21 patients with rhabdomyosarcoma, two (11.1%) of 18 patients with clear cell sarcoma, and two (11.1%) of 18 patients with angiosarcoma had lymphatic involvement. Thirty patients who had resection of involved lymph nodes had an estimated 5-year survival of 57%, whereas nine patients treated without surgery all died within 30 months. An estimated 4-year survival of 71% for patients with isolated lymph node metastases was significantly better than 21% for patients with synchronous systemic and lymph node involvement. There was no difference in outcome for patients with isolated lymphatic involvement compared with patients with American Joint Committee on Cancer Stage III extremity sarcomas. These results suggest that long-term survival is possible after surgical resection of lymphatic metastases from soft tissue sarcoma. The American Joint Committee on Cancer should consider separating isolated nodal metastases from systemic involvement in patients with Stage IV sarcoma.