Ulnocarpal arthrodesis as a new treatment for ectrodactyly in a dog and a cat.

CASE HISTORY: A 4-month-old male Shih Tzu dog (Case 1) and an 11-month-old female Devon Rex cat (Case 2) were referred to specialist veterinary hospitals for evaluation of right thoracic lameness and growth abnormality in the distal aspect of the forelimb. CLINICAL FINDINGS: Non-weight-bearing lameness and decreased range of motion were noted in the affected limbs of both cases. Case 1 had a plantigrade stance, and a cleft separation between the first and second digits extending upwards to the distal third of the antebrachium. There was no pain on palpation, and the affected limb was shorter than the contralateral. Radiographic examination revealed cleft separation between metacarpal bones I and II, and carpal bone fusion (I, II, III), and the distal radius ended freely and was attached to the first metacarpal bone.Case 2 had a small cleft medial to metacarpal III. The limb was consistently held in abduction and had marked carpal varus. The limb had never been used for weight bearing. Radiographic examination showed agenesis of metacarpal bone II and separation of metacarpals I and III. The radius and ulna were separated and the radial head did not articulate normally at the elbow, leading to marked elbow incongruity. DIAGNOSIS: Ectrodactyly in both cases. TREATMENT AND OUTCOME: Amputation of the radius followed by ulnocarpal arthrodesis were performed in both cases. Follow-up evaluations up to 1 year (Case 1) and 10 weeks (Case 2) after surgery indicated satisfactory arthrodesis fusion, owner satisfaction, and a good clinical outcome. CLINICAL RELEVANCE: Ectrodactyly is a rare congenital deformity of the forelimb with a heterogeneous character, requiring an individualised treatment plan. These are the first cases reported in the literature of ectrodactyly in small animals that were treated successfully with ulnocarpal arthrodesis. This case series therefore provides evidence in support of this treatment option for this heterogeneous congenital deformity.

Pedicled Osteo-Onchyocutaneous Island Flap for Finger Macrodactyly: A Review of Literature.

We present a case of middle finger macrodactyly reconstructed in a single stage using multiple techniques. We elevated a pedicled osteo-onychocutaneous island flap, excised the remnant distal phalanx with a segment of 1 digital nerve and skin over the dorsum of the middle phalanx, performed epiphysiodesis and reduction of the middle phalanx as well as soft-tissue debulking, and inset the flap over the dorsum of the middle phalanx. Follow-up at 12 months revealed a satisfactory aesthetic and functional outcome.

The impact of limb loading and the measurement modality (2D versus 3D) on the measurement of the limb loading dependent lower extremity parameters.

BACKGROUND: Deformity assessment and preoperative planning of realignment surgery are conventionally based on weight-bearing (WB) radiographs. However, newer technologies such as three-dimensional (3D) preoperative planning and surgical navigation with patient-specific instruments (PSI) rely on non-weight bearing (NWB) computed tomography (CT) data. Additionally, differences between conventional two-dimensional (2D) and 3D measurements are known. The goal of the present study was to systematically analyse the influence of WB and the measurement modality (2D versus 3D) on common WB-dependent measurements used for deformity assessment. METHODS: 85 lower limbs could be included. Two readers measured the hip-knee-ankle angle (HKA) and the joint line convergence angle (JLCA) in 2D WB and 2D NWB radiographs, as well as in CT-reconstructed 3D models using an already established 3D measurement method for HKA, and a newly developed 3D measurement method for JLCA, respectively. Interrater and intermodality reliability was assessed. RESULTS: Significant differences between WB and NWB measurements were found for HKA (p < 0.001) and JLCA (p < 0.001). No significant difference could be observed between 2D HKA NWB and 3D HKA (p = 0.09). The difference between 2D JLCA NWB and 3D JLCA was significant (p < 0.001). The intraclass correlation coefficient (ICC) for the interrater agreement was almost perfect for all HKA and 3D JLCA measurements and substantial for 2D JLCA WB and 2D JLCA NWB. ICC for the intermodality agreement was almost perfect between 2D HKA WB and 2D HKA NWB as well as between 2D HKA NWB and 3D HKA, whereas it was moderate between 2D JLCA WB and 2D JLCA NWB and between 2D JLCA NWB and 3D JLCA. CONCLUSION: Limb loading results in significant differences for both HKA and JLCA measurements. Furthermore, 2D projections were found to be insufficient to represent 3D joint anatomy in complex cases. With an increasing number of surgical approaches based on NWB CT-reconstructed models, research should focus on the development of 3D planning methods that consider the effects of WB on leg alignment.

Precise Resection of Macrodactyly Under Assistance of Three-Dimensional Reconstruction Technology: A Case Report.

Macrodactyly of the foot is an extremely rare congenital malformation characterized by an increase in the size of all the elements or structures of a digit or digits. Most sources indicate that macrodactyly affects the hand more often than the foot. This rare medical condition usually requires surgical intervention with a precise preoperative plan and postoperative rehabilitation. We present a case of macrodactyly of the right foot in which surgical reduction of the foot under assistance of 3-dimensional image technology was performed with satisfying cosmetic and functional outcomes.

[Surgical Treatment of Congenital Cleft Foot - Case Study and Literature Review]. / Operacní lécení vrozeného rozstepu nohy ­ kazuistika a prehled literatury.

Congenital cleft foot is a deformity characterised by genetic heterogeneity and a high degree of phenotypic variability. This together with its very low incidence is the reason for rather controversial opinions on the treatment. The authors present a case study of a boy with unilateral cleft foot classified as type III by Blauth-Borisch and type II by Abraham et al., who underwent a surgery at the age of 12 months. The defect was closed by rectangular soft tissue flaps, the intermetatarsal ligament connecting the first metatarsal head and the third metatarsal head was reconstructed with local fibrous tissue flap. The present hypermobility with extension position of the first ray were stabilised by the Kirschner wire inserted along the axis of the first ray from the dorsum of the talus through the middle of its head to the medial chondrogenic tarsal bones and further through the first metatarsal bone and the corrected metatarsophalangeal joint of the hallux. At the age of 6 years and 7 months, due to dynamic abductovalgus foot deformity, lengthening calcaneal osteotomy was also performed. Favourable clinical and radiographic outcomes of the used surgical technique were observed by the authors. The cleft foot as such shall be treated after a thorough evaluation of the patient and his/her necessary monitoring during the childhood. Key words: ectrodactyly, cleft foot, split hand/foot malformation.

Outcomes of kidney transplants in pediatric patients with the vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula, renal anomalies, limb abnormalities association.

In this single-center retrospective study, we analyzed kidney transplant outcomes in nine pediatric patients with VACTERL [vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula, renal anomalies, limb abnormalities] association-making this the largest study of its kind. Of 743 pediatric kidney transplant recipients at our center (1980-2017), nine had documented diagnoses of VACTERL association. All nine had congenital anorectal malformations and renal anomalies, five had vertebral defects, and one had a bifid thumb and tracheoesophageal fistula. Renal anomalies included dysplasia (n = 6), aplasia (n = 3), and horseshoe kidney (n = 2). Congenital lower urinary tract anomalies included neurogenic bladder (n = 6), obstructive uropathy (n = 4), anovesicular fistula (n = 1), rectourethral fistula (n = 1), and posterior urethral valves (n = 1). Age at transplant ranged from 1.2 to 15 years (mean, 7.3; standard deviation [SD], 5.5); 6 (67%) were male, and 3 (33%) were female; 6 (67%) had a living related donor, and 3 (33%) had a deceased donor. The overall graft survival rate was 78% (range, 1.5 to 25.2 years; mean, 10.5; SD, 8.9). One month post-transplant, one recipient died with a functioning graft. At 3.7 years post-transplant, one graft failed because of recurrent pyelonephritis. Post-transplant urologic complications included pyelonephritis (n = 6), vesicoureteral reflux (n = 5), and graft hydronephrosis (n = 4). We conclude that pediatric patients with VACTERL association can be safely transplanted-careful patient selection with vigilance and intervention for pre- and post-transplant urologic complications is essential.

Polymelia in a chimeric Simmental calf: nociceptive withdrawal reflex, anaesthetic and analgesic management, anatomic and genetic analysis.

BACKGROUND: Polymelia is a congenital defect characterized by one or more supernumerary legs. The genetics and aetiology of this condition in cattle have not yet been thoroughly investigated even though several case reports do exist. The model of the nociceptive withdrawal reflex (NWR) has been characterized in several species to study spinal nociceptive processing. It is a polysynaptic spinal reflex that can be elicited by noxious electrical stimulation and recorded by electromyography. Thorough nociceptive examination and preventive analgesic management has not yet been an aspect in the perioperative management of polymelia cases. CASE PRESENTATION: A 4-month-old female Simmental calf was presented with notomelia. The animal was in good health and showed no neurologic deficiencies. Preoperatively, computed tomography was performed to gain more detailed anatomical information. To evaluate the sensitivity of the accessory limb, NWR testing was performed and revealed a connection of the afferent reflex pathway of the accessory limb to the efferent of the normal limb. The accessory limb was surgically removed under general anaesthesia. Intensive care included multimodal pain therapy adapted to the pain intensity scored during regular pain assessment. A gross anatomical dissection as well as a genetic analysis of the accessory limb were performed postoperatively. The calf was identified as a chimera. CONCLUSION: This calf was successfully relieved of its accessory limb. Chimerism has not been described in the congenital defect polymelia. As the accessory limb was pain sensitive and a common nociceptive reflex pathway was identified, thorough perioperative pain management was performed with the intention to prevent chronic neuropathic pain development.

Unicornuate uterus with a rudimentary non-communicating cavitary horn in association with VACTERL association: case report.

BACKGROUND: The unicornuate uterus is caused by abnormal or failed development of one Müllerian duct. Unicornuate uteri with functioning non-communicating rudimentary horns are susceptible to many gynaecologic and obstetric complications such as hematometra, endometriosis and ectopic pregnancy and thus surgical resection is usually recommended.. CASE PRESENTATION: We report a rare case of a unicornuate right uterus with rudimentary non-communicating (functional) cavitary left horn (class U4a) in a 17-year-old girl who was diagnosed with VACTERL association. She was presented to our centre with 3 years history of secondary sever dysmenorrhea. Pelvic magnetic resonance imaging revealed a normal uterus on the right side, a 7 × 8 cm left endometrioma, a tortuous dilated fluid-filled structure in the left hemipelvis, mostly represented left-sided hematosalpinx, and a well-defined lesion with thick enhancing wall in the left hemipelvis measuring 6.7 × 5.7 × 5.6 cm with a similar enhancement to the uterus in the right. She underwent laparotomy that showed a right unicornuate uterus with a normal cervix and a rudimentary non-communicating distended left horn. In addition, there was a left endometrioma and left hematosalpinx. Resection of the left communicating horn, left salpingectomy and left ovarian cystectomy were performed. The right tube and both ovaries were preserved. At 9-months follow up, the patient had a regular period and the pain subsided completely. CONCLUSION: We report yet the second case of VACTERL association and unicornuate uterus with non-communicating functional rudimentary horn, in hope of expanding the knowledge of a rare occurrence. This case also highlights the importance of considering the diagnosis of Müllerian duct anomalies in patients with a history of other anomalies, and/or history of early-age secondary dysmenorrhea.

Recurrence of carpal tunnel syndrome in isolated non-syndromic macrodactyly: DTI examination of a giant median nerve.

Macrodystrophia lipomatosa, a hamartomatous enlargement of soft tissues leading to gigantism of a part or a whole extremity, generally affects the territory of distribution of a single nerve. In some cases, this condition may cause an entrapment neuropathy. We report the clinical, radiological, and surgical findings of a patient with isolated non-syndromic macrodactyly and giant median nerve presenting recurrent carpal tunnel syndrome (CTS). In this case, conventional magnetic resonance imaging (MRI) and diffusion tensor imaging (DTI) supported the presence of fibrohamartomatous infiltration, determining an enlargement of the median nerve and consequently an increased susceptibility to compression damage. A 57-year-old man presenting macrodactyly of the second and third finger of the right hand showed recent onset of severe hypoesthesia of the first three fingers of the right hand. He also underwent surgery for right CTS 23 years before. The electromyography/electroneurography confirmed the diagnosis of CTS. The ultrasonography showed a massive enlargement of the median nerve within the carpal tunnel, while MRI confirmed the enlargement of the median nerve with thickened hypointense bundles and interposed tissue, with increased mean diffusivity and decreased fractional anisotropy at DTI. We discuss about the use of a relatively novel imaging technique, investigating for the first time an uncommon cause of a very common entrapment neuropathy.

Treatment of Hand Macrodactyly With Resection and Toe Transfers.

Macrodactyly is an uncommon congenital hand condition that can be difficult to treat and that can have a profound negative impact on patients and their families. Although many treatments have been described, results tend to be inconsistent. The authors report a case in which a combination of ray resection, partial finger resection, and toe transfer resulted in a 4-digit hand with acceptable function and cosmesis.

Angular Limb Deformities: Growth Retardation.

Angular limb deformities are common in foals; however, the importance of the deformity and if treatment is required depend on the degree of deformity relative to normal conformation for stage of growth, the breed and discipline expectations, age, and response to conservative therapies. This article addresses the importance of the foal conformation examination to determine which foals need surgical intervention to correct an angular deformity and when. Techniques for surgical growth retardation include the transphyseal staple, screw and wire transphyseal bridge, and transphyseal screw. Appropriate timing for intervention for each location and complications associated with each procedure are discussed.

[Domestic external fixator application in the treatment of limb deformities: 7 289 cases application report].

Objective: To discuss the clinical application and effects of domestic external fixator in the treatment of patients with malformations of limbs. Methods: A total of 7 289 patients with malformation of limbs who had been operated in Qin Sihe orthopedic surgery team from January 1989 to June 2016 were retrospective analyzed. The patients were treated with domestic external fixator, including 4 033 males and 3 256 females, aging from 2 to 82 years with a mean age of 23.4 years. There were 2 732 patients using Ilizarov external fixator, 4 713 patients using hybrid external fixator, 57 patients using monobrachial external fixator, 232 patients using Ilizarov external fixator and hybrid external fixator. The Ilizarov, hybrid and monobrachial external fixator were used in 67, 65 and 0 patients on the upper limbs and in 2 665, 4 616 and 57 patients on the lower limbs. There were 3 028 patients operated on the left limbs, 3 260 patients operated on the right limbs and 1 001 patients operated on the bilateral limbs. The top three types of diseases were sequelae of poliomyelitis, cerebral palsy and post-traumatic stress disorder peromely. Deformity types inclued talipes equinovarus, knee flexion deformity, cavus foot and so on. Results: All the patients were followed up for a period of 2.5 months to 22.4 years, with an average follow-up time of 5.4 years. All of the external fixators were used for single once, and there was no substitute for external fixator quality problem. All the patients were completed surgery goal until removing external fixation except 1 patient gave up treatment and 1 removed the fixator because of metal allergy. The common complications included wire or pin infection and joint movement limitation and so on. Conclusions: The domestic external fixator developed and produced based on the characteristics of Chinese limb deformity disability. The domestic external fixator can be used to treat kinds of limb deformities with the advantages of practical, economical, adjustable, universal and portable. The domestic external fixator could meet the clinical demand for fixation of the osteotomy end of the limbs, the correction of the deformity, the repair of the defects and the limb lengthening.

Surgical Treatment of Macrodactyly.

Macrodactyly, enlargement of one or multiple digits, was described in the literature nearly 200 years ago. This is an exceptionally uncommon diagnosis that has led to a paucity of descriptive literature on the treatment options. Because the literature is scarce, and the frequency with which hand surgeons encounter macrodactyly is even scarcer, treatment can be a formidable task often left exclusively to those trained in congenital hand deformity. This article presents our algorithm and surgical techniques for dealing with children with macrodactyly in such a way that should make a complex problem more easily approachable.

Standing placement of transphyseal screw in the distal radius in 8 Thoroughbred yearlings.

This retrospective study describes placement of distal radial transphyseal screws in Thoroughbred yearlings with carpal varus deformities while standing, and identifes short- and long-term complications following the procedure. Data gathered from 2009 to 2013 identified 8 yearlings that met the inclusion criteria. Horses were sedated intravenously and a single 4.5-mm cortical screw was placed in the distal lateral radial physis following application of local anesthetic and surgical preparation of a pre-placed hole. All horses were evaluated weekly after surgery and screw removal was performed standing and under sedation when correction of the angular limb deformity was achieved. The mean time for screw removal was 46 days. No short- or long-term complications were identified. Findings indicate that placing a single transphyseal screw in the lateral aspect of the distal radial physis with the horse standing is a viable option to treat varus angular limb deformity of the carpus in horses.

Placement debout d'une vis transphysaire dans le radius distal chez 8 Thoroughbred âgés d'un an. Cette étude rétrospective décrit le placement d'une vis transphysaire dans le radius distal de chevaux Thoroughbred âgés d'un an ayant des difformités du varus carpien, lorsqu'ils sont debout, et elle identifie les complications à court et à long terme après l'intervention. Les données recueillies de 2009 à 2013 ont identifié 8 chevaux âgés d'un an qui satisfaisaient aux critères d'inclusion. Les chevaux ont été mis sous sédation par intraveineuse et une seule vis corticale de 4,5 mm a été placée dans le cartilage diaphyso-éphysaire après l'application d'anesthésie locale et de la préparation chirurgicale d'un trou pratiqué au préalable. Tous les chevaux ont été évalués une fois par semaine après la chirurgie et l'enlèvement de la vis a été réalisé debout et sous sédation lorsque la correction de la difformité angulaire du membre a été obtenue. Le temps moyen de l'enlèvement des vis était de 46 jours. Aucune complication à court ou à long terme n'a été identifiée. Les résultats indiquent que le placement d'une seule vis transphysaire dans l'aspect latéral du cartilage diaphyso-éphysaire distal radial, lorsque le cheval se tient debout, est une option viable pour traiter la difformité angulaire de type varus du carpe chez les chevaux.(Traduit par Isabelle Vallières).

DIAGNOSIS AND SURGICAL TREATMENT OF A COMPLEX ANGULAR HIND LIMB DEFORMITY IN A SERVAL (FELIS SERVAL).

A 2-yr-old neutered male pet serval (Felis serval) was presented for progressive hind limb lameness that started at 6 mo of age. Previous therapy included only nutritional supplementation. Direct and video gait analysis confirmed bilateral hind limb lameness, more severe on the right. Physical examination and radiography revealed a multifocal complex bilateral angular deformity with a significant rotational component. A right tibial corrective osteotomy was followed by internal rotation and stabilization with a 2.7-mm eight-hole locking compression plate and locking screws. Other deformities were not corrected. Clinical improvement was noted immediately and has been maintained over the 16-mo follow-up.

A child with split-hand/foot associated with tibial hemimelia (SHFLD syndrome) and thrombocytopenia maps to chromosome region 17p13.3.

We describe a-2-year-old boy who presented with a neonatal history of thrombocytopenia associated with a constellation of limb malformations mimicking split hand/foot malformation with long bone deficiency (SHFLD) syndrome. Limb malformations consisted of unilateral monodactyly with radial aplasia, unilateral split foot and bilateral club foot. Tibial aplasia of one limb and tibial hypoplasia of the other limb were notable. Partial agenesis of the sacrum was additional skeletal malformation. Craniofacial features included dense thick scalp hair, narrow frontal area, thick eye-brows, deep-set eyes, depressed nasal bridge, and small overhanging nasal tip, full-cheeks, and large ears. Array-CGH showed duplication of the short arm of chromosome 17p13.3 in the boy and his father, respectively. The father was free from any skeletal abnormalities, though he shares similar craniofacial dysmorphic features like his son. In addition, a paternal sib (uncle of the proband) manifested a phenotype similar to that of the proband. To the best of our knowledge the overall phenotypic and genotypic characterizations were consistent but not completely compatible with the traditional type of TAR syndrome or with SHFLD syndrome. We report on what might be a novel variant of SHFLD associated with transient thrombocytopenia, dysmorphic facial features, and a constellation of bone malformations.

Orthopedics management of acromicric dysplasia: follow up of nine patients.

UNLABELLED: Acromicric dysplasia (AD) is an autosomal dominant disorder characterized by short stature, short extremities, stiff joint and skeleton features including brachymetacarpia, cone-shaped epiphyses, internal notch of the femoral head, and delayed bone age. Recently, we identified fibrillin 1 (FBN1) as the disease gene of AD. The aim of our study was to further describe the long-term follow up of AD patients with an emphasis on orthopedic management. Nine patients with FBN1 mutations were included in the study ranging in age from 5.5 to 64 years. For all, detailed clinical and radiological data were available. RESULTS: Birth parameters were always normal and patients progressively developed short stature <-3 SD. Carpal tunnel syndrome was observed in four patients. We found discrepancy between the carpal bone age and the radius and ulna epiphysis bone ages, a variable severity of hip dysplasia with acetabular dysplasia, epiphyseal and metaphyseal femoral dysplasia resembling Legg-Perthes-Calvé disease and variable pelvic anteversion and hyperlordosis. Orthopedic surgery was required in two patients for hip dysplasia, in one for limb lengthening and in three for carpal tunnel syndrome. Our observations expand the AD phenotype and emphasize the importance of regular orthopedic survey.

Amputation of polymelia in a layer chicken.

Polymelia was rarely recorded in birds; therefore, this report records the clinical and radiographic findings in a case of polymelia in a 7-mo-old layer Fayoumi chicken and the outcome of its amputation. The hen had two pericloacal extra limbs located caudal to the normal ones in an inverted position. The extra limbs were immovable and loosely attached to the underlying tissues. The supernumerary limbs had normal skin with ill-developed feathers. The extra limbs were fused proximally and crossed distally. Plain radiographic examination showed unequal numbers of bones and digits in both extra limbs. The right extra limb had a femur and tarsometatarsus while the left extra one had femur, tibiotarsus, and tarsometatarsus bones. The extra limbs appeared smaller than the normal ones and had neither muscles nor tendons. Amputation of the extra limbs under local infiltration analgesia was easy, safe, and curative.

VACTERL association with a rare vertebral anomaly (butterfly vertebra) in a case of monochorionic twin.

The VATER/VACTERL association is typically defined by the presence of at least three of the following congenital malformations: vertebral anomalies, anal atresia, cardiac malformations, tracheo-esophageal fistula, renal anomalies, and limb abnormalities (13). We report a rare case of a monochorionic twin gestation in which one of the infants had VACTERL association. Antenatal ultrasound showed bilateral renal dysplasia and cardiac anomaly (ASD) in twin A. Twin A was noted to have the following anomalies: a single umbilical artery, limb anomaly (right hand preaxial polydactyly), vertebral anomalies (T9 and T11 butterfly vertebras, bilateral renal agenesis, bladder agenesis, anal and urethral atresia. A normal-sized stomach and normal amount of amniotic fluid were observed during the prenatal period with no other anomalies. Twin B (male) was healthy and no cardiac, renal, or congenital anomalies were demonstrated on ultrasound and physical examination. Infant A was also diagnosed as having VACTERL association because he had five of the core anomalies (V, A, C, R, L) of VACTERL association. Butterfly vertebra is an uncommon congenital spinal anomaly. To the best of our knowledge, our patient is the second case VACTERL association with butterfly vertebra in the literature.

Long-finger pollicization for macrodactyly of the thumb and index finger.

Pollicization of the long finger is rarely performed, and previously described for treating traumatic thumb and index finger loss. Because the long finger lacks the independence of motion and muscular attachments of the index finger, pollicization of the long finger requires modifications of the technique. We present the case of a 3-year-old girl with progressive macrodactyly of the thumb and index finger associated with a lipofibromatous hamartoma of the median nerve. The involved digits were severely enlarged, stiff, and nonfunctional. The child was treated with first and second ray resection followed by long-finger pollicization. Surgical pearls and pitfalls are discussed.