Dorsal rotation flap for centralization in radial longitudinal deficiency

PURPOSE: The purpose of this report is to present the results of a dorsal rotation flap for centralization in the treatment of radial longitudinal deficiency. METHODS: All patients surgically treated for radial longitudinal deficiency with a centralization procedure and use of the dorsal rotation flap between 1996 and 2006 were retrospectively reviewed. RESULTS: Twenty-one limbs in 15 patients treated with centralization using the dorsal rotation flap were reviewed, with photographs of the scar available for review for 15 limbs in 10 patients. There were no primary wound-healing problems, no hypertrophic scarring, and no color mismatch. In the 15 limbs with available photographs, all scars were rated as good. Use of this incision allowed good surgical access to the necessary structures on the radial and ulnar side of the wrist to allow for centralization. CONCLUSIONS: The dorsal rotation flap allows rotation of the skin in a radial direction while the hand and carpus are rotated in an ulnar direction; the redundant skin on the ulnar side of the wrist is rotated to compensate for the shortage of skin on the radial side of the wrist. This report describes use of the dorsal rotation flap with no complications attributable to the surgical approach.     

Radius lengthening for the treatment of Bayne and Klug type II and type III radial longitudinal deficiency

PURPOSE: We performed radius lengthening to treat radial deviation of the wrist in patients with Bayne and Klug type II and type III radial longitudinal deficiencies. The purpose of this investigation was to review our results of radius lengthening for radial longitudinal deficiency. METHODS: Beginning in 1991 radius lengthening was performed to treat 4 patients with radial longitudinal deficiency whose mean age at the initial lengthening was 16 months. The corrections for radial deviation of the wrists were performed simultaneously by soft-tissue distraction of the wrist. According to Bayne and Klug's classification 2 patients had type II and 2 patients had type III deficiencies. All but 1 patient had lengthening several times to correct the recurring discrepancy between the radius and the ulna. RESULTS: The corrections were achieved just after the lengthening but deformities recurred because of growth discrepancies between the radius and the ulna as the children grew. The radius was lengthened by a mean of 28 mm, with a mean length gain of 79%. The mean period in the fixator was 136 days. Two patients had both functionally and cosmetically acceptable correction after several lengthening procedures. In the other patient we abandoned this treatment method because of severe bone absorption at the distal end of the lengthened radius. One patient died of cardiac disease after one lengthening. CONCLUSIONS: Radius lengthening for Bayne and Klug type II and type III deficiencies may be accomplished successfully with the primary benefit of maintaining wrist and forearm motion. It is likely that at least 3 lengthenings may be required and this may need to be accompanied by a soft-tissue distraction at the ulnar carpal joint as well. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic, Level IV.     

A classification system of radial polydactyly: inclusion of triphalangeal thumb and triplication

PURPOSE: Radial polydactyly is a congenital anomaly with a wide range of manifestations. Current classifications do not have the capacity to classify all different types of radial polydactyly when combined with triphalangeal components. The objective of this study was to test an adjusted classification and nomenclature that allows classification of triphalangeal components and triplication in radial polydactyly. METHODS: Patients from 1993 to 2006 with radial polydactyly (N = 104), a total of 121 affected hands, were identified from the hospital database. All x-rays were carefully examined and classified according to the existing classifications for radial polydactyly and a modified classification. In the modified nomenclature, Wassel's level of duplication is preserved. Type VII and VIII are assigned for partial or complete duplication of the carpal bones according to Buck-Gramcko. Triplication and triphalangeal components can be assigned to each type of radial polydactyly by suffixes. Symphalangism, deviation, and hypoplasia can also be classified. Triplication on different levels of the thumb is classified by determining and including the different types of the original Wassel classification. RESULTS: Eighteen thumbs could not be classified according to existing classifications for radial polydactyly with triphalangeal components or triplication. Using the proposed classification, all patients could be classified. CONCLUSIONS: We propose a modified classification that is a practical and utilitarian scheme for nomenclature of radial polydactyly and that may assist comparison of treatment outcomes and individual cases. TYPE OF STUDY/LEVEL OF EVIDENCE: Diagnostic II.     

Ulnar growth patterns in radial longitudinal deficiency

PURPOSE: To determine the association between centralization surgical procedures and the longitudinal growth of the ulna in radial longitudinal deficiency (RLD). METHODS: The charts of 90 patients with 124 affected limbs were reviewed. Thirty-four patients were affected bilaterally and 56 were affected unilaterally. Based on the Bayne and Klug classification there were 5 type I, 3 type II, 9 type III, and 107 type IV deformities. Seventy-two limbs had available radiographs, which were measured for ulnar length. We plotted 384 ulnar length measurements in 72 limbs and compared these with both normative ulnar length data and ulnar length data in RLD. The average ulnar length was compared for the group (n = 46) treated with surgical centralization versus the nonsurgically treated group (n = 22). RESULTS: The nonsurgically treated group attained 64% of normal ulnar length whereas the nonnotched centralization group attained 58% of normal ulnar length. The notched centralization group attained 48% of normal ulnar length. Ulnar growth for the surgically treated group averaged 0.54 cm/y and the for the nonsurgically treated group averaged 0.71 cm/y, which showed no statistical significance. CONCLUSIONS: Wrist centralization procedures effectively increase the overall length of the limb by centralizing the hand and carpus over the shortened ulna; this must be weighed against the high rate of recurrent radial deviation deformity and some loss of ulnar growth.     

The treatment of longitudinal radial deficiency

Absence of the radius is the commonest of the longitudinal deficiencies but is itself quite an unusual congenital anomaly. No one surgeon is likely to encounter the condition more than once or twice in a career and there is a strong case for congenital upper limb conditions to be collected into special clinics. The pathology and methods of surgical treatment are reviewed. It is a difficult condition to treat but there is no doubt that function and appearance can be improved (Pulvertaft, 1973). Pollicisation of the index finger should be considered and can often greatly improve the function.     

The cloacal anomalies: Anatomical insights through a complex spectrum

PurposeTo study the detailed anatomy of cloacal anomalies using the multiplanar capabilities of MRI in addition to other available modalities.Patients and methodsThe study was conducted on 27 cases of cloaca that were managed at our unit during the last 12 years. Preoperative assessment included conventional investigations and MRI studies. Endoscopic and operative findings were important for confirmation and completion of the whole picture.For better perception of the degree of deviation from the norm, we included another control group of girls who underwent pelvic MRI studies for causes other than anorectal anomalies.ResultsFor practical reasons, we dissociated the cloacal complex into its two basic components to be described separately namely the anorectal anomaly and persistent urogenital sinus.The anorectal anomaly may be best described regarding two parameters: the position of the rectum and type of rectal communication with the urogenital tract.Persistent urogenital sinus anomalies were stratified based on referring the level of the urogenital confluence to the back of pubic symphysis. Three types could be identified (low, intermediate, and high) in which the mean length of the common channel was 6.4, 14.6, and 24 mm respectively with a significant statistical difference (Kruskal–Wallis chi-squared test).ConclusionApplying MRI in the preoperative assessment of cases of cloaca has the advantage of demonstrating the real anatomy of the anomaly in multiple planes. This can improve our perception of the degree of deviation from the norm in these cases, which may be important during planning for surgical reconstruction.Level of evidenceThis is a case control study (level III evidence).     

The ulnar longitudinal deficiency: proposition of a descriptive classification

Ulnar longitudinal deficiency is an extended malformation sometimes involving the whole upper extremity, even including sometimes the opposite side. The clinical and radiological aspects are variable and none of the existing classifications takes into account all the possible deformities. Multiple decisive factors in the surgical indications are missing such as shoulder stability, elbow position (extension or flexion with or without pterygium), orientation of the hand (internal rotation), wrist inclination and number of digits. Based on a review of our 46 clinical cases as well as the published cases in the literature we have developed a simple way to describe each level.     

Soft-tissue distraction with a ring external fixator before centralization for radial longitudinal deficiency

PURPOSE: To review the techniques and evaluate the use of soft-tissue distraction with a ringed fixator for radial longitudinal deficiency (RLD) before centralization. METHODS: Eight extremities in 6 patients with severe RLD were treated with ring fixator distraction followed by centralization. A clinical examination was performed and radiographic data were obtained prospectively and at an average follow-up period of 17 months. The techniques and the early objective (range of motion, position) and radiographic outcome were evaluated. RESULTS: The preoperative ring fixator accomplished an average of 16 mm of distraction and allowed the centralization procedure to be performed effectively and without tension. Clinical alignment was improved markedly. The hand-forearm angle improved from 72 degrees radial to 8 degrees ulnar after surgery. The volar carpal subluxation improved by an average of 6 mm. CONCLUSIONS: Precentralization ring fixator distraction allows the performance of a tensionless centralization procedure with good early radiographic and clinical outcome in patients severely affected with RLD. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic, Level IV.     

Surgical treatment of type 0 radial longitudinal deficiency

PURPOSE: The purpose of this study was 2-fold: (1) to describe the surgical anatomy associated with type 0 radial longitudinal deficiency (radially deviated hand in the presence of a normal-length radius) and (2) to report the results of a surgical procedure designed to improve the alignment of the hand and forearm. METHODS: Since 1986 there have been 6 cases of type 0 radial longitudinal deficiency in 5 children seen at the St. Louis Shriner's Hospital. These children were treated with a surgical procedure to release the radial soft tissues and correct the alignment with tendon transfers. Age at surgery ranged from 12 to 40 months, with an average age of 21 months. The average follow-up period was 21 months. The tight radial wrist extensors were detached at their distal insertion and the tight radial wrist capsule was released dorsally and volarly, thus relieving the radial tether. The extensor carpi ulnaris (ECU) tendon also was detached just proximal to its insertion and sutured to the dorsal wrist capsule to augment wrist extension. The radial wrist extensor tendon was reattached to the ECU tendon at its insertion, converting it into an ulnar deviator. RESULTS: In each case the radial wrist extensor was noted to be hypoplastic and the radial wrist capsule was noted to be tight, tethering the hand in radial deviation. In 4 cases there was only a single radial wrist extensor tendon. Average radial deviation at rest improved from 58 degrees to 12 degrees . Improvement in radial deviation at rest averaged 47 degrees , ranging from 25 degrees to 75 degrees . Improvement in active wrist extension averaged 53 degrees and improvement in passive wrist extension averaged 28 degrees . CONCLUSIONS: For children with type 0 radial longitudinal deficiency a soft-tissue procedure that releases the radial tether and augments ulnar deviation and wrist extension by tendon transfer satisfactorily improves wrist alignment with minimal morbidity.     

桡骨远端骨折分型研究概述

桡骨远端骨折是临床常见骨折，目前，其分型方法多样，往往会造成诊断、治疗和预后评价上的混乱，不利于临床选择最佳治疗方法。本文就桡骨远端骨折分型状况作一综述，以期更好地指导临床治疗。     

主动脉夹层的细化分型及其应用

目的探讨在Stanford分型的基础上根据主动脉夹层的部位和病变程度再进行细化分型,对指导临床选择手术时机、确定治疗方案和手术方式,以及判断预后的价值。方法1994年1月至2004年12月我院治疗主动脉夹层708例。其中Stanford A型夹层477例：（1）根据主动脉根部病变程度分为3型。A1型（主动脉窦部正常型）212例,行保留主动脉窦部的主动脉替换;A2型（主动脉窦部轻度受累型）72例,行主动脉窦部成形63例、David手术9例;A3型（主动脉窦部重度受累型）193例,行主动脉根部替换术（Bentall手术）。（2）根据主动脉弓部病变分为2型。C型（复杂型）78例,行主动脉弓部替换＋象鼻术;S型（单纯型）399例,行部分主动脉弓部替换。Stanford B型夹层231例,（1）根据主动脉扩张的范围分为3型：B1型：降主动脉无扩张或仅有近端扩张,147例,行腔内带膜支架主动脉腔内修复术103例（B1S型）、部分胸降主动脉替换术32例、部分胸降主动脉替换术＋远端支架象鼻术12例;B2型：全部胸降主动脉扩张,53例,行部分胸降主动脉替换术＋主动脉成形32例、全部胸降主动脉替换术21例;B3型：全部胸降主动脉及腹主动脉扩张,31例行胸腹主动脉替换术。（2）根据左锁骨下动脉和远端主动脉弓部是否受夹层累及分为2型：C型（复杂型）：夹层累及左锁骨下动脉或远端的主动脉弓部,44例,在深低温停循环下手术治疗;S型（单纯型）：远端主动脉弓部和左锁骨下动脉未受夹层累及,187例,介入治疗103例、手术治疗84例（常温阻断下手术60例,股动脉-股静脉转流下手术24例）。结果Stanford A型夹层住院病死率为4．6％（22／477）,并发症发生率为14．5％（69／477）。Stanford B型夹层：介入治疗组病死率1．9％（2／103）,并发症发生率为2．9％（3／103）,轻度内漏发生率为9．7％（10／103）;手术治疗组住院病死率为3．1％（4／128）,并发症发生率为18．8％（24／128）。结论细化主动脉夹层的分型对于术前判断手术时机、制定手术方案和初步判断预后,具有重要的指导作用。     

Radial longitudinal deficiency: the incidence of associated medical and musculoskeletal conditions

PURPOSE: Radial longitudinal deficiency (RLD) is associated with certain syndromes and medical and musculoskeletal conditions. The purpose of this investigation was to evaluate the incidence of these conditions with RLD. METHODS: A comprehensive chart review identified patients with RLD and a complete medical record. These charts were evaluated for the presence of associated medical and musculoskeletal conditions and biographic information on gestation, delivery, and family history. RESULTS: A total of 164 patients with 245 affected extremities were identified; 138 patients had radius abnormalities and 26 patients had isolated thumb hypoplasia. Twenty-five patients had thrombocytopenia absent radius syndrome; 22 patients had vertebral, anal, cardiac, tracheoesophageal, renal, and limb abnormalities association; 7 patients had Holt-Oram syndrome; and 1 patient had Fanconi anemia. There were 32 patients with cardiac abnormalities and 60 patients with spinal or lower-extremity musculoskeletal abnormalities. The percentage of patients with associated abnormalities increased with an increasing severity of RLD. One hundred two of the 138 patients with types I through V RLD had associated medical or musculoskeletal abnormalities. In contrast, only 9 of 26 patients with an isolated thumb hypoplasia (type 0 RLD) had associated abnormalities. CONCLUSIONS: The high incidence of associated medical and musculoskeletal abnormalities in patients with RLD emphasizes the importance of a complete assessment including a complete musculoskeletal examination, cardiac auscultation, complete blood count, echocardiogram, renal ultrasound, and spinal radiographs. Although approximately one third of patients in this investigation had a syndrome commonly associated with RLD, most patients with RLD types I through V had an additional medical or musculoskeletal anomaly. Patients with type 0 RLD were less likely to have comorbidities.     

桡骨远端关节内骨折的分类与关节镜下复位固定

目前的桡骨远端骨折分类方法有一个共同的缺陷，即没有详细描述关节面的解剖改变。基于CT断层扫描和三维重建技术，根据关节面骨块的数目，日本的土井一辉(Doi)教授将桡骨远端关节内骨折分为3型，即2，3、4块型。此分类法简单但直观准确描述了关节面的结构改变，对关节镜操作非常实用。根据此分类，针对不同的骨折类型，采用腕关节镜辅助下的复位固定，以期达到关节面移位小于1mm的复位和固定效果。1992～2003年，91例桡骨远端关节内骨折患者接受关节镜手术，年龄21～79岁。其中42例为2块型，占46％；34例3块型，占37％；15例4块型，占17％。关节镜用于复位后检查14例，引导克氏针13例，辅助复位固定61例，其中4例改为开放复位内固定。固定方法包括外固定架联合克氏针或钢板、钢板联合拉出钢丝或螺钉，6例单用克氏针，1例单用螺钉。长期的功能随访结果表明，与其它传统方法相比，桡骨远端关节内骨折采用关节镜辅助的复位固定可获得更好的关节活动度和握力。     

Assessment of thumb metacarpophalangeal joint arthrodesis using a single longitudinal K-wire

PURPOSE: To evaluate retrospectively the functional and radiographic outcome of patients who had a thumb metacarpophalangeal joint arthrodesis with a single longitudinal K-wire. METHODS: We performed a retrospective review of 27 patients who had a thumb metacarpophalangeal joint arthrodesis with a single longitudinal K-wire. Outcomes were assessed using self-reported measures (Patient-Rated Wrist/Hand Evaluation; AUSCAN Osteoarthritis Hand Index; Disabilities of the Arm, Shoulder and Hand questionnaire; Short Form-36 General Health questionnaire), measured physical impairments (range of motion, strength, manual dexterity), and radiographic review. RESULTS: Patient-rated outcome scores indicated mild pain, difficulty with specific and usual tasks, and minimal concern with hand appearance. Most physical impairment measures were not significantly different from the side not surgically treated, and patient satisfaction was high. There was a slight decrease in tripod pinch strength, manual dexterity, interphalangeal joint flexion, handspan, and thumb opposition compared with the contralateral side. The overall complication rate was 11% (3 complications). Twenty-five patients (89%) were able to return to their previous occupations. There were no radiographic malunions or nonunions. Radiographic evidence of degenerative changes was present in 20% of patients at the carpometacarpal joint and 30% of patients at the interphalangeal joint. CONCLUSIONS: Our technique of performing a thumb metacarpophalangeal joint arthrodesis with a single longitudinal K-wire is effective, with high overall patient satisfaction and a relatively low complication rate, despite small losses of strength, manual dexterity, and motion. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic IV.     

Reverse osseofasciocutaneous radial forearm flap for thumb reconstruction: a flap design and case series

PURPOSE: To report the outcome of the modified design of the osseofasciocutaneous radial forearm flap. The flap was modified because of problems associated with the use in thumb reconstruction. METHODS: We performed retrospective review of all patients who sustained nonreplantable thumb amputations who had this procedure between August 2000 and March 2005. The patients' ages ranged between 24 and 39 years. Demographic data and details of the level of amputation and alternative surgical procedures were reviewed. Eight male patients were identified and constituted the study cohort. The size of flap, length of the radius harvested, time to union, sensibility (moving 2-point discrimination test), grip and pinch strengths, complications, and patient outcome were determined. RESULTS: All patients who had the butterfly design of the reverse-flow osseofasciocutaneous radial forearm flap had an adequate and painless soft-tissue padding on the tip of the reconstructed thumb that provided good protection for the distal stump of the vascularized bone graft. Patients did not experience strictures at the bottom of the reconstructed tubes. CONCLUSIONS: The butterfly design is a useful modification of the skin paddle of the reverse osseofasciocutaneous radial forearm flap for thumb reconstruction. The design offers an easy way to reconstruct a tube shape with adequate padding on the tip and no terminal scar. In addition it prevents the constricting effect of the circumferential scar on the button of the reconstructed tube.     

Developmental biology and classification of congenital anomalies of the hand and upper extremity

Recent investigations into the mechanism of limb development have clarified the roles of several molecules, their pathways, and interactions. Characterization of the molecular pathways that orchestrate limb development has provided insight into the etiology of many limb malformations. In this review, we describe how the insights from developmental biology are related to clinically relevant anomalies and the current classification schemes used to define, categorize, and communicate patterns of upper limb malformations. We advocate an updated classification scheme for upper limb anomalies that incorporates our current molecular perspective of limb development and the pathogenetic basis for malformations using dysmorphology terminology. We anticipate that this scheme will improve the utility of a classification as a basis for diagnosis, treatment, and research.