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I'm going to go through a small set of topics that have picked at random and do not necessarily represent whether they are common or will come up in your F R C S. Your general approach starts with a patient and then focus is on the hand, start with age in any congenital hand discussion. Age is going to be very important because it affects the timing of your treatment. Next, you will go through their general birth, developmental and family history last but not least you have to know their functional level. Remember that enhance pinch grip, tripod grip and grasp. Also known as pretension are important components of how a hand eventually functions. It is classification of congenital upper limb anomalies. The old Swanson's classification was published in the very first general of hand surgery in 1976 and included seven categories. These were failure, formation, failure of differentiation, duplication, overgrowth, undergrowth, constriction bands, and generalized skeletal anomalies. A lot of Swanson's original work was based on an understanding of the proximal distal development of the limb because they knew the existence of the apical ectoderm, a ridge. But they didn't quite understand back then. That there were three access in the development of the upper extremities. The other two being ventral dorsal and radioulnar. After Swanson's, the classification has now been superseded by the Oberg Mansky and Tonkin classification, which has been recommended by the I F S S H. The OMT classification was originally described in 2010 with first updates in 2014 and second update in 2020. Yeah. To make things even easier. There is an app developed by We Lamb who is a congenital hand surgeon up in Edinburgh. If you want to explore the topic further, he has excellent videos up on youtube which are worth watching. This is the QR code to help you download the app. Having been through a general overview of assessment and classification. These next few slides cover several congenital hand conditions. Syndactyly is where the digits are joined up. It is a common congenital hand anomaly which occurs one in every 2000 to 3000 births. It's inherited in an autosomal dominant manner with variable expression. However, it can also occur sporadically. Syndactyly can be classified as complete or incomplete depending on whether it includes a nail folds and simple or complex depending on whether it's purely soft tissue or includes bone infusion between the digits in both these pictures which represent a simple syndactyly. The picture on the left includes a nail folds and therefore is incomplete syndactyly compared to the right, which is incomplete complex syndactyly is where there's bone infusion between the digits and complicated syndactyly is those which are associated with the syndrome and often have multiple fusion points between the digits. The goal of syndactyly is to create a more normal webspace to improve the appearance and function of each finger surgery is generally recommended for all cases because outcomes are better with finger separation. Timing for surgery is controversial but generally performed between 12 and 18 months. If you operate earlier, you risk high incidence of scar contracture. Whereas operating later may result in finger too deformed as a result in differential growth between the fingers. It's worth noting that little and ring finger, differential growth is greater compared to that between ring and middle finger. And therefore we're more likely to separate little and ring fingers earlier. If it syndactyly affects three or more fingers, it is generally recommended to stage your operation. So as not to risk vascular compromise of the flaps and digits from bilateral dissections. The most common procedure for separating the fingers and reconstructing the web space is using what's known as a proximately based dorsal rectangular flab. These clinical photographs show the lateral base flaps at the tips of both fingers which are elevated and then re rotated to recreate the nail folds. You can then harvest a full thickness skin graft from the groin or take a split thickness graft in order to cover the exposed areas where the fingers were. Uh guys can people say if there's no, can you hear anything at, when did you stop hearing things? So I can still hear the audio fine. Okay. So it was working until 10 seconds ago. Correct. I can hear the whole thing. I can't hear it currently. Clifton is also as an ectrodactyly. It's a central longitudinal deficiency which causes suppression of the bone and soft tissues within the central elements of the hand, which includes the index, middle and ring fingers. Classically, this results in the V shaped Clifton hand compared to the U shaped deformity that we see in some bracket activity, which we'll talk about later. Generally, the digital absence. Whereas the metacarpals are still present, even though they're maybe hyperplastic Chlef hands could be described as typical or atypical. Typical clef hands are genetic and can be associated with clef foot as well. Atypical cleft hands are form of Simbarashe. Exactly that involves the index long and ring fingers and are thought to be as a result of sporadic mutation. Atypical cleft hands are rarely associated with syndromes of deformities and the disorders not inherited. In other words, you're more likely to see a family history for those who have typical cleft hands. The functional you issue is usually a small first web space between the thumb and index finger. This leads to an inability to form an effective pinch or tripod grip. For those finer motor movements. Surgery would involve some level of soft tissue releases, tendon transfers and osteotomy ease of the metacarpals in reality. Although the deformity looks quite striking, these patient's actually have a high degree of function and many typically do not choose to undergo surgery. Symbyax exactly is short and webbed digits. It usually occurs sporadically but it might be a feature of Poland Syndrome. The underlying problem is disruption of embryonic formation and differentiation. The results on hand as shorter and smaller compared to the contralateral with underdeveloped, often shorter web digits, digital nubbins or absent digits. As always, treatment should be patient specific and done for the patient rather than to the patient. The technique may involve separation of the syndactyly but also increasing the mobility of the finger by dividing the transverse metacarpal ligaments. Thumb hyperplasia is characterized by a smaller thumb size, metacarpal adduction, MP, joint instability, thena muscle hyperplasia or aplasia, extrinsic tendon dysplasia. And in the most severe cases that could be CMC joint instability or thumb aplasia, it's considered part of a radial deficiency. And so even with normal forum architecture, it's important to screen systemically for those syndromes that you see as below the blood of classification is the most well known for thumb hyperplasia. This goes from 1 to 5 with one being less severe and five being more severe blow with one does not require treatment and is simply a shortened and diminished thumb. And this might even be unnoticed by individuals who are asymptomatic blouse too is where there is more extensive soft tissue involvement including instability of the ulnar collateral ligaments and adducted webspace and underdeveloped or absent thenar muscles, blough three is the same as type to accept that there's now involvement of the extrinsic tendon, which includes E P L and F P L3 A and three B are sub classified depending on whether there is a stable MCP joints or in three B, whether it's an unstable MCP joint. Blouse four is a floating thumb which is attached simply by soft tissue bridge and is completely nonfunctional and blah thigh. There's an absent thumb. This is the summary of the classification. Type one hyperplasia usually does not warrant surgical intervention because the functional impairment is minimal. Type two or three. A hyperplasia does affect function and often requires surgery to improve stability, improve motion or enhance the function of the thumb. The reconstruction involves a combination of soft tissue releases first webspace, local flap coverage on the collateral ligament reconstruction, extrinsic tendon, tina license or muscle tendon transfers type four or five hyperplasia. Normally requires an index finger politization. If the index finger is suitable for becoming a thumb. The politization procedure again is a combination of osteotomies and soft tissue reconstruction and transfers in order to achieve a thumb that can have some level of pinch grip thumb do populations are a failure of formation and or differentiation affecting the radio on the axis of the hand plate. This image shows the muscle description of seven types of thumb duplication, whereby higher numbers represent a more proximal split involving first the phalanx and then the metacarpal. The aim of surgical briefing instruction is to obtain a stable mobile thumb of adequate size and appropriate shape. This normally involves removal of the lesser digit and reconstruction of the dominant digit. The bill ACL okay procedure is a classical method of reconstructing thumb duplication. This is a vessel for duplication with evenly matched thumbs which is ideal for this procedure. The procedure involves central resection of the duplication, followed by fusion of the uh remaining lateral portions in order to obtain a thumb of satisfactory volume. Macro Doctor Lee is a rare condition in which the fingers, hands or limb growth is unregulated resulting in the overgrowth of tissues to the affected extremities. Although it's considered a tumorous condition, it's not considered to be malignant and therefore there have been known known cases of malignant transformation to date the cause for macro. Doctor Lee is thought to be a defect in signaling pathways that dominate the growth of the tissues. Peripheral nerves and the involved territories are usually enlarge and this is particularly clinically significant because the median nerve could develop an overgrowth in what's known as a lipo fibro metis harm automa which can lead to uh carpet tunnel syndrome. In the child. Treatment approaches can vary but the general approach is to maintain the size and alignment uh of the fingers in order to achieve optimal function. This would be through accommodation of uh epifix, CDCs, osteotomy is debulking procedures, uh and even amputations and or two transfers. These pictures show macro doctor affecting the index and middle fingers that were treated by soft tissue debulking and fusion and epic physiolysis. It's helpful to note that many patient's with macro doctorly may have meet the nerve enlargement at the level of the carpal tunnel that could lead to symptomatic carpal tunnel syndrome. If they're young, they may not be able to tell you the typical complaints of pins and needles to the fingers. Instead, the parents may describe frequent scratching, biting the fingers as well, shaking of the hand. The treatment is carpal tunnel decompression and debulking with sometimes removal of enlarged subcutaneous nerves in this region. Arthrogryposis is a huge topic and a spectrum of disorders. It's a nonprogressive congenital disorder of unknown or idiopathic etiology. It can result from environmental or genetic causes, distill arthrogryposis including that which affects the hands are genetic disorders. The diagnosis of arthrogryposis is made clinically with the presence of elbow and the hyper extension contractures, shoulder, internal rotation, contractors and hands which are in in intrinsic plus deformity. There will be severe limitation or absent movement in a range of joints around all four limbs. The treatment is an MD T approach involving the pediatrician. Geneticists. Therapists and surgeons treatment includes things such as bracing and casting and surgically. We have options of soft tissue releases, fusions and osteotomy is in this clinical picture, you will see the characteristic smooth appearance to the skin, which means that even increases around the joints are absent. This table shows some patterns of hand deformities that come in arthrogryposis. In patients with distal arthrogryposis, the risk is often an extension but it can also be in flexion, hand function can be highly variable. We're going into a gentle finish. Clinodactyly is angulation of the digits in the radial ulnar. In other words, corona plain, that's distilled to the MCP joint. It's found in 25% of Children with Down syndrome and 3% of the general population. There is an autosomal dominant inheritance and the middle phalanx of the small finger is most commonly affected, make sure you don't confuse this with a kerner deformity which results in an apex. Also ulnar deformity of the little finger, therefore causing flexion in the sagittal plane plan. It actually has to be angulations more than 10 degrees. If it is less, it is probably just a normal anatomical variant. The shape is due to a result of asymmetric longitudinal growth as always, climate actually can also be seen as part of many syndromes and complex hand anomalies. If miles, the treatment is usually to do nothing but it's severe. There is an option to perform uh an opening wedge osteotomy of the phalanx. Sometimes patient's may have what's known as a delta phalanx which includes the epithets iss in a C shape. Lastly, I'd like to discuss congenital trigger thumb. This is 10 times more common than trigger finger. It occurs at a rate of three cases per 1000 births by age 12 months. Bilateral involvement is present in approximately one quarter to one third of Children. The difficulty in diagnosing it in infants is that they will naturally hold their thumb in a position of flexion into the palm. Anyways, as shown in this slide. Sigma Tau classified trigger thumb according to four stages depending on simply the presence of a swelling known as a not uh not a nodule, triggering on active movements, triggering felt only by passive movements and a rigid joint. Higher Tsukamoto grades makes it more likely that surgical intervention is required. Despite this 30 to 60% would be expected to resolve spontaneously before the age of two years old and when a child has reached two years old, thereafter, it becomes far less likely to resolve spontaneously at less than 10%. The first line of treatment is intermittent extension splinting. Uh and this is for younger kids. The precise timing of surgery is dependent on multiple factors including the age of the child. The Sugimoto grade of trigger thumb and the discussion between the surgeon and the family surgery can be delayed for up to three years of age without any long term detriment to the thumb range of motion. For Sugimoto stage four, which is a painful triggering and rigid deformity. It's recommended to undergo surgery even if less than one years old. Again, the timing of surgery is controversial and depends from unit to unit. In summary, we've just been through a whistle stop tour of congenital hand anomalies. We went through a general approach which includes accurate history and examination of the patient's in particular, considering their age and functional deficit associated with their congenital hand anomaly, we briefly touched upon a few anomalies which have manifestations affecting the thumb, the fingers and the hand. There are some little things to look out for such as Poland syndrome in a typical cleft hand carpal tunnel syndrome in macro doctorly or associated syndromes with some dysplasia such as the vector I'll association francona's anemia or charge syndrome. That concludes our talk. Please feel free to email me if you have any questions. All right. Hopefully that played throughout. It did mostly that's certainly write for trying to be too clever.