- Limb development begins in the 4th week of gestation from lateral plate mesoderm under the guidance of three signaling centers: the AER, ZPA, and dorsal/ventral ectoderm.
- The AER guides proximal-distal growth through FGF signaling, the ZPA patterns anterior-posterior development using SHH, and the ectoderm determines dorsal-ventral fates through WNT7a.
- Failure of limb formation can result in transverse or longitudinal deficiencies, where parts of the limb fail to develop fully, such as in radial club hand where the radius is absent. Classification systems aim to describe the spectrum of abnormalities.
This document discusses congenital vertical talus (CVT), a rare foot deformity. It begins by defining CVT and providing background information. It then describes the anatomy and pathoanatomy of CVT. Key points include that CVT results in an almost vertical talus bone and rigid flatfoot deformity. Treatment involves serial casting and manipulation to prepare for surgery, with the goal of restoring normal anatomical relationships in the foot. Surgical techniques described include open reduction and percutaneous fixation of the talonavicular joint with K-wires. Complications of surgery can include wound issues and stiffness.
This document provides information about Perthes' disease, including:
- It is characterized by avascular necrosis of the femoral head in children.
- Risk factors include being male and between ages 5-10 years old.
- Imaging studies like x-rays are used to diagnose and monitor the stages of avascular necrosis, fragmentation, ossification, and remodeling.
- Differential diagnosis depends on whether the condition is unilateral or bilateral.
- Treatment aims to prevent deformity through nonsurgical or surgical methods depending on the severity.
This document provides information on slipped capital femoral epiphysis (SCFE), including:
- SCFE involves slippage of the femoral epiphysis posteriorly and inferiorly due to weakness of the growth plate.
- It most commonly affects obese adolescent boys and girls during periods of rapid growth.
- Clinical features include pain in the groin or knee and limited range of motion of the hip.
- Treatment involves closed or open reduction and internal fixation using pins or screws to stabilize the slip.
- The goals of treatment are to prevent further slippage and allow healing to occur without complications like avascular necrosis.
This document discusses the clinical examination of the hip joint, including inspection, palpation, range of motion testing, special tests, and gait analysis. Key examination findings are described for various hip pathologies like developmental dysplasia of the hip, arthritis, fractures, and dislocations. Landmark bony anatomy, compensations, and fallacies of certain examination maneuvers are also outlined.
This document discusses fracture diseases that can result from prolonged immobilization following fracture treatment. The principal fracture diseases include musculoskeletal issues like muscle weakness, atrophy, soft tissue contractures, osteoporosis, and joint stiffness. Prolonged immobilization can also lead to cardiovascular complications such as increased heart rate, circulatory dysfunction, orthostatic hypotension, and venous thromboembolism. Prevention of these fracture diseases focuses on proper fixation, early and frequent mobilization, muscle stretching and contraction, range of motion exercises, use of leg stockings, anticoagulant drugs, and changing patient positioning regularly.
This document provides an overview of clubfoot (congenital talipes equinovarus), including:
1. The definition, incidence, causes, and typical deformities seen in clubfoot.
2. Evaluation methods like the Pirani scoring system and radiographic assessment.
3. Treatment approaches like the Ponseti method of serial casting and bracing, as well as surgical options like the McKay procedure when non-operative treatment fails.
4. Post-operative casting protocols and complications that can arise from treatment.
Blount's disease, also known as tibia vara, is a progressive orthopedic condition that causes bowing of the legs in children. It results from a growth disturbance in the proximal medial tibial growth plate. The deformity includes varus alignment of the knees, internal tibial torsion, and leg length discrepancy. Radiographs show a characteristic wedging and irregularity of the proximal medial tibial epiphysis. Treatment depends on age and severity, ranging from bracing to corrective osteotomies. Congenital metatarsus adductus is another common pediatric foot deformity involving inward twisting of the forefoot. It is often mild and resolves with stretching, but more severe cases may require
This document discusses congenital vertical talus (CVT), a rare foot deformity. It begins by defining CVT and providing background information. It then describes the anatomy and pathoanatomy of CVT. Key points include that CVT results in an almost vertical talus bone and rigid flatfoot deformity. Treatment involves serial casting and manipulation to prepare for surgery, with the goal of restoring normal anatomical relationships in the foot. Surgical techniques described include open reduction and percutaneous fixation of the talonavicular joint with K-wires. Complications of surgery can include wound issues and stiffness.
This document provides information about Perthes' disease, including:
- It is characterized by avascular necrosis of the femoral head in children.
- Risk factors include being male and between ages 5-10 years old.
- Imaging studies like x-rays are used to diagnose and monitor the stages of avascular necrosis, fragmentation, ossification, and remodeling.
- Differential diagnosis depends on whether the condition is unilateral or bilateral.
- Treatment aims to prevent deformity through nonsurgical or surgical methods depending on the severity.
This document provides information on slipped capital femoral epiphysis (SCFE), including:
- SCFE involves slippage of the femoral epiphysis posteriorly and inferiorly due to weakness of the growth plate.
- It most commonly affects obese adolescent boys and girls during periods of rapid growth.
- Clinical features include pain in the groin or knee and limited range of motion of the hip.
- Treatment involves closed or open reduction and internal fixation using pins or screws to stabilize the slip.
- The goals of treatment are to prevent further slippage and allow healing to occur without complications like avascular necrosis.
This document discusses the clinical examination of the hip joint, including inspection, palpation, range of motion testing, special tests, and gait analysis. Key examination findings are described for various hip pathologies like developmental dysplasia of the hip, arthritis, fractures, and dislocations. Landmark bony anatomy, compensations, and fallacies of certain examination maneuvers are also outlined.
This document discusses fracture diseases that can result from prolonged immobilization following fracture treatment. The principal fracture diseases include musculoskeletal issues like muscle weakness, atrophy, soft tissue contractures, osteoporosis, and joint stiffness. Prolonged immobilization can also lead to cardiovascular complications such as increased heart rate, circulatory dysfunction, orthostatic hypotension, and venous thromboembolism. Prevention of these fracture diseases focuses on proper fixation, early and frequent mobilization, muscle stretching and contraction, range of motion exercises, use of leg stockings, anticoagulant drugs, and changing patient positioning regularly.
This document provides an overview of clubfoot (congenital talipes equinovarus), including:
1. The definition, incidence, causes, and typical deformities seen in clubfoot.
2. Evaluation methods like the Pirani scoring system and radiographic assessment.
3. Treatment approaches like the Ponseti method of serial casting and bracing, as well as surgical options like the McKay procedure when non-operative treatment fails.
4. Post-operative casting protocols and complications that can arise from treatment.
Blount's disease, also known as tibia vara, is a progressive orthopedic condition that causes bowing of the legs in children. It results from a growth disturbance in the proximal medial tibial growth plate. The deformity includes varus alignment of the knees, internal tibial torsion, and leg length discrepancy. Radiographs show a characteristic wedging and irregularity of the proximal medial tibial epiphysis. Treatment depends on age and severity, ranging from bracing to corrective osteotomies. Congenital metatarsus adductus is another common pediatric foot deformity involving inward twisting of the forefoot. It is often mild and resolves with stretching, but more severe cases may require
The document discusses physeal (growth plate) injuries in children. It notes that physeal injuries represent 15-20% of injuries in children and can cause growth arrest and deformities. The most common sites are the distal radius, distal tibia, and phalanges. It describes the anatomy and blood supply of the physis. It discusses the Salter-Harris classification system for physeal fractures and treatment approaches including casting, splinting, and surgery. Complications of physeal injuries like growth arrest, angular deformity, and limb length discrepancy are also summarized. Long term follow up is needed to monitor bone healing and growth.
This document discusses limb length discrepancy (LLD), including its definition, causes, effects, evaluation, and management. LLD is when one lower limb is noticeably longer than the other. It is classified as structural or functional. LLD of 2.5 cm or more can cause back/hip/knee pain and gait abnormalities. Evaluation involves history, exam including block testing, and imaging like scansograms. LLD can be managed non-surgically with shoe lifts for small discrepancies or surgically with epiphysiodesis or bone lengthening depending on the severity.
This document discusses congenital talipes equino-varus (CTEV), also known as clubfoot. CTEV is a congenital deformity of the foot and ankle characterized by equinus, inversion, adduction and cavus. It occurs in about 1 in 1000 live births. The document describes the types and causes of CTEV, pathological changes, treatment methods including Ponseti technique and surgery, and long-term management with bracing. Non-operative treatment is usually attempted first using serial casting and manipulation techniques.
The document provides information about ulnar nerve injury, including its course through the upper limb, branches and sensory/motor supply. Causes of injury include compression at sites like the elbow (cubital tunnel syndrome) and wrist (Guyon's canal syndrome). Signs and symptoms involve sensory loss and weakness of hand muscles. Clinical tests assess functions like pinching. Investigations include EMG, nerve conduction studies and imaging. Claw hand deformity can occur with severe ulnar nerve injury.
The document describes claw hand, which is a deformity of the hand where the fingers are flexed into the palm at the middle knuckle. It discusses the anatomy, etiology, types, clinical signs, classification, and surgical techniques for correction. The main techniques discussed are static procedures like flexor pulley advancement and dynamic procedures using tendon transfers to restore muscle function. The goal of surgery is to maintain the middle knuckle in slight flexion to allow the extensors to straighten the fingers.
This document discusses congenital anomalies of the upper limb, including definition, incidence, embryology, classification, and specific anomalies such as club hand, Madelung deformity, Apert's syndrome, cleft hand, and thumb abnormalities. Upper limb anomalies can occur due to arrest of development during crucial embryonic stages between 4-8 weeks. Common types include transverse or longitudinal arrest of parts, failure of differentiation, duplication, and overgrowth/undergrowth. Radial and ulnar club hands are described in detail along with associated syndromes and treatment approaches.
The document discusses developmental dysplasia of the hip (DDH), including: definitions; clinical detection from birth to 6 months using tests like Ortolani's and Barlow's; treatment from birth to 6 months using a Pavlik harness or closed reduction and hip spica casting; and treatment from 6 to 18 months also using closed reduction and hip spica casting, with the goal of obtaining and maintaining reduction without damaging the femoral head. Obstacles to reduction like hypertrophic soft tissues are also mentioned.
Cubitus varus, or gunstock deformity, is caused by malunion of supracondylar fractures and results in the forearm being deviated inward at the elbow with loss of the carrying angle. It is a triplanar deformity involving varus, hyperextension, and internal rotation. Treatment options include observation for young children, hemiepiphysiodesis to alter growth, and corrective osteotomy. The lateral closing wedge osteotomy is commonly used to safely correct the varus deformity through removal of a lateral wedge. Other techniques include medial opening wedge, oblique, dome, and step-cut osteotomies. Postoperative management focuses on immobilizing the arm in extension
Limb length discrepancy can be structural or functional. For structural discrepancies between 2-5 cm in growing children, epiphysiodesis is commonly used to modulate growth. Epiphysiodesis involves arresting growth in the long limb's growth plate to allow the short limb time to catch up. It is a relatively simple procedure but risks include under or overcorrection and asymmetric growth arrest. For discrepancies over 5 cm or in skeletally mature individuals, shortening the long limb is preferred over lengthening the short limb.
This document provides an overview of Legg Calve Perthes disease, including its definition, demographics, risk factors, pathogenesis, clinical features, investigations, classifications, management, and surgical procedures. Some key points:
- It is avascular necrosis of the femoral head in children, most common in ages 4-8 years. Positive family history and low birth weight are risk factors.
- Clinical features include hip/thigh pain aggravated by movement. Imaging shows stages from avascular necrosis to fragmentation to regeneration/healing.
- Conservative management includes bracing for young/mild cases. Surgical containment is used for more severe/older cases to encourage spherical remodeling.
- Procedures
The document provides information about the radial nerve including its anatomy, course, branches and clinical presentations of radial nerve palsies. It discusses the radial nerve's origin from the brachial plexus and branches in the arm and forearm. Common causes of radial nerve palsy include fractures and entrapment in the radial tunnel. Clinical features, investigations, treatment including splinting and tendon transfers, and postoperative management are outlined. Surgical techniques for nerve repair and reconstructive procedures are also described.
This document discusses coxa vara, which is a hip deformity characterized by an abnormal decrease in the femoral neck-shaft angle. It classifies coxa vara as congenital, developmental, or acquired. Developmental coxa vara is the most common type and is caused by a primary cartilage defect in the femoral neck. Clinical features include limping and pain. Treatment involves corrective valgus osteotomies to restore the neck-shaft angle and relieve stress on the femoral physis. The document describes several techniques for valgus osteotomy including Pauwel's, Borden's, and subtrochanteric osteotomy. The goal of surgery is to stimulate healing of the femoral neck defect and restore normal
Dr. Jhansi Kusuma presented on the topic of foot drop. The presentation defined foot drop as the inability to dorsiflex the foot due to damage to the common peroneal nerve. It discussed the anatomy of the leg and dorsiflexor muscles innervated by the deep peroneal nerve. The common peroneal nerve was described in detail, including its branches. Causes, symptoms, types, diagnosis and management of foot drop including conservative treatment, surgery, and tendon transfer procedures were covered. Dr. Narasimha Reddy moderated and Dr. Sarath chaired the presentation.
1) Tuberculosis of the knee joint is the third most common site of osteoarticular tuberculosis, accounting for around 10% of skeletal tuberculosis cases.
2) Initial pathology involves hematogenous spread to the synovium or subchondral bone, forming tubercles. Advanced cases involve erosion of joint surfaces and destruction of bones.
3) Clinical features include knee swelling, warmth, effusion, tenderness, and restricted painful movement. Advanced cases develop triple deformity of flexion, adduction, and internal rotation.
Foot drop is the inability to lift the front part of the foot. It can be caused by injuries or conditions that damage the common peroneal nerve. Symptoms include difficulty lifting the foot and dragging the toes. Treatment depends on the underlying cause but may include bracing, nerve stimulation, tendon transfers, or joint fusions. The goal is to improve mobility and gait.
This document discusses avascular necrosis of the femoral head, also known as osteonecrosis. It begins by providing a brief history and definitions. It then discusses the blood supply of the femoral head and covers traumatic vs. non-traumatic causes. Risk factors for atraumatic osteonecrosis like corticosteroids, alcohol abuse, smoking, and others are outlined. The pathophysiology section explores theories of arterial occlusion, fat emboli, and increased bone marrow pressure as causes. Signs and symptoms, diagnostic imaging methods, staging classifications, and non-operative and operative treatment options are summarized.
Tuberculosis of the hip joint is the second most common site of bone and joint TB after the spine. It typically affects people in their first three decades of life. The infection spreads from a primary focus such as the lungs to the hip joint via the bloodstream. It can initially involve different areas of the hip and pelvis before spreading to the joint. Patients present with hip pain, limping, and constitutional symptoms. Treatment involves anti-TB drugs along with rest, traction, and surgery if needed to address complications like joint destruction and deformity. Surgical options depend on the stage of disease and can include synovectomy, arthrodesis, osteotomy, or arthroplasty.
1. The document discusses various congenital hand anomalies classified using the Swanson and modified classifications.
2. Key classifications include failures of formation (transverse arrest, longitudinal arrest), differentiation (syndactyly, contractures), duplication (polydactyly), overgrowth (macrodactyly), undergrowth (hypoplastic thumb), and generalized skeletal abnormalities (Apert syndrome, Poland syndrome).
3. The modified classification organizes anomalies by malformations involving the entire limb, hand plate, or unspecified structures, as well as deformities and dysplasias. Specific conditions like symbrachydactyly, triphalangeal thumb, and brachydacty
The document discusses the embryology and development of the upper limb. It covers topics like:
- The formation of the limb bud and structures involved in outgrowth like the apical ectodermal ridge and progress zone.
- How the limb bud develops and differentiates over time into the hand plate and cartilage models of bones.
- Rotation and ossification of the bones during the 7th week of development.
- Common congenital anomalies of the upper limb like syndactyly and polydactyly.
- Classification systems for various upper limb anomalies.
- Principles and timing of surgical treatment for different anomalies.
The document discusses physeal (growth plate) injuries in children. It notes that physeal injuries represent 15-20% of injuries in children and can cause growth arrest and deformities. The most common sites are the distal radius, distal tibia, and phalanges. It describes the anatomy and blood supply of the physis. It discusses the Salter-Harris classification system for physeal fractures and treatment approaches including casting, splinting, and surgery. Complications of physeal injuries like growth arrest, angular deformity, and limb length discrepancy are also summarized. Long term follow up is needed to monitor bone healing and growth.
This document discusses limb length discrepancy (LLD), including its definition, causes, effects, evaluation, and management. LLD is when one lower limb is noticeably longer than the other. It is classified as structural or functional. LLD of 2.5 cm or more can cause back/hip/knee pain and gait abnormalities. Evaluation involves history, exam including block testing, and imaging like scansograms. LLD can be managed non-surgically with shoe lifts for small discrepancies or surgically with epiphysiodesis or bone lengthening depending on the severity.
This document discusses congenital talipes equino-varus (CTEV), also known as clubfoot. CTEV is a congenital deformity of the foot and ankle characterized by equinus, inversion, adduction and cavus. It occurs in about 1 in 1000 live births. The document describes the types and causes of CTEV, pathological changes, treatment methods including Ponseti technique and surgery, and long-term management with bracing. Non-operative treatment is usually attempted first using serial casting and manipulation techniques.
The document provides information about ulnar nerve injury, including its course through the upper limb, branches and sensory/motor supply. Causes of injury include compression at sites like the elbow (cubital tunnel syndrome) and wrist (Guyon's canal syndrome). Signs and symptoms involve sensory loss and weakness of hand muscles. Clinical tests assess functions like pinching. Investigations include EMG, nerve conduction studies and imaging. Claw hand deformity can occur with severe ulnar nerve injury.
The document describes claw hand, which is a deformity of the hand where the fingers are flexed into the palm at the middle knuckle. It discusses the anatomy, etiology, types, clinical signs, classification, and surgical techniques for correction. The main techniques discussed are static procedures like flexor pulley advancement and dynamic procedures using tendon transfers to restore muscle function. The goal of surgery is to maintain the middle knuckle in slight flexion to allow the extensors to straighten the fingers.
This document discusses congenital anomalies of the upper limb, including definition, incidence, embryology, classification, and specific anomalies such as club hand, Madelung deformity, Apert's syndrome, cleft hand, and thumb abnormalities. Upper limb anomalies can occur due to arrest of development during crucial embryonic stages between 4-8 weeks. Common types include transverse or longitudinal arrest of parts, failure of differentiation, duplication, and overgrowth/undergrowth. Radial and ulnar club hands are described in detail along with associated syndromes and treatment approaches.
The document discusses developmental dysplasia of the hip (DDH), including: definitions; clinical detection from birth to 6 months using tests like Ortolani's and Barlow's; treatment from birth to 6 months using a Pavlik harness or closed reduction and hip spica casting; and treatment from 6 to 18 months also using closed reduction and hip spica casting, with the goal of obtaining and maintaining reduction without damaging the femoral head. Obstacles to reduction like hypertrophic soft tissues are also mentioned.
Cubitus varus, or gunstock deformity, is caused by malunion of supracondylar fractures and results in the forearm being deviated inward at the elbow with loss of the carrying angle. It is a triplanar deformity involving varus, hyperextension, and internal rotation. Treatment options include observation for young children, hemiepiphysiodesis to alter growth, and corrective osteotomy. The lateral closing wedge osteotomy is commonly used to safely correct the varus deformity through removal of a lateral wedge. Other techniques include medial opening wedge, oblique, dome, and step-cut osteotomies. Postoperative management focuses on immobilizing the arm in extension
Limb length discrepancy can be structural or functional. For structural discrepancies between 2-5 cm in growing children, epiphysiodesis is commonly used to modulate growth. Epiphysiodesis involves arresting growth in the long limb's growth plate to allow the short limb time to catch up. It is a relatively simple procedure but risks include under or overcorrection and asymmetric growth arrest. For discrepancies over 5 cm or in skeletally mature individuals, shortening the long limb is preferred over lengthening the short limb.
This document provides an overview of Legg Calve Perthes disease, including its definition, demographics, risk factors, pathogenesis, clinical features, investigations, classifications, management, and surgical procedures. Some key points:
- It is avascular necrosis of the femoral head in children, most common in ages 4-8 years. Positive family history and low birth weight are risk factors.
- Clinical features include hip/thigh pain aggravated by movement. Imaging shows stages from avascular necrosis to fragmentation to regeneration/healing.
- Conservative management includes bracing for young/mild cases. Surgical containment is used for more severe/older cases to encourage spherical remodeling.
- Procedures
The document provides information about the radial nerve including its anatomy, course, branches and clinical presentations of radial nerve palsies. It discusses the radial nerve's origin from the brachial plexus and branches in the arm and forearm. Common causes of radial nerve palsy include fractures and entrapment in the radial tunnel. Clinical features, investigations, treatment including splinting and tendon transfers, and postoperative management are outlined. Surgical techniques for nerve repair and reconstructive procedures are also described.
This document discusses coxa vara, which is a hip deformity characterized by an abnormal decrease in the femoral neck-shaft angle. It classifies coxa vara as congenital, developmental, or acquired. Developmental coxa vara is the most common type and is caused by a primary cartilage defect in the femoral neck. Clinical features include limping and pain. Treatment involves corrective valgus osteotomies to restore the neck-shaft angle and relieve stress on the femoral physis. The document describes several techniques for valgus osteotomy including Pauwel's, Borden's, and subtrochanteric osteotomy. The goal of surgery is to stimulate healing of the femoral neck defect and restore normal
Dr. Jhansi Kusuma presented on the topic of foot drop. The presentation defined foot drop as the inability to dorsiflex the foot due to damage to the common peroneal nerve. It discussed the anatomy of the leg and dorsiflexor muscles innervated by the deep peroneal nerve. The common peroneal nerve was described in detail, including its branches. Causes, symptoms, types, diagnosis and management of foot drop including conservative treatment, surgery, and tendon transfer procedures were covered. Dr. Narasimha Reddy moderated and Dr. Sarath chaired the presentation.
1) Tuberculosis of the knee joint is the third most common site of osteoarticular tuberculosis, accounting for around 10% of skeletal tuberculosis cases.
2) Initial pathology involves hematogenous spread to the synovium or subchondral bone, forming tubercles. Advanced cases involve erosion of joint surfaces and destruction of bones.
3) Clinical features include knee swelling, warmth, effusion, tenderness, and restricted painful movement. Advanced cases develop triple deformity of flexion, adduction, and internal rotation.
Foot drop is the inability to lift the front part of the foot. It can be caused by injuries or conditions that damage the common peroneal nerve. Symptoms include difficulty lifting the foot and dragging the toes. Treatment depends on the underlying cause but may include bracing, nerve stimulation, tendon transfers, or joint fusions. The goal is to improve mobility and gait.
This document discusses avascular necrosis of the femoral head, also known as osteonecrosis. It begins by providing a brief history and definitions. It then discusses the blood supply of the femoral head and covers traumatic vs. non-traumatic causes. Risk factors for atraumatic osteonecrosis like corticosteroids, alcohol abuse, smoking, and others are outlined. The pathophysiology section explores theories of arterial occlusion, fat emboli, and increased bone marrow pressure as causes. Signs and symptoms, diagnostic imaging methods, staging classifications, and non-operative and operative treatment options are summarized.
Tuberculosis of the hip joint is the second most common site of bone and joint TB after the spine. It typically affects people in their first three decades of life. The infection spreads from a primary focus such as the lungs to the hip joint via the bloodstream. It can initially involve different areas of the hip and pelvis before spreading to the joint. Patients present with hip pain, limping, and constitutional symptoms. Treatment involves anti-TB drugs along with rest, traction, and surgery if needed to address complications like joint destruction and deformity. Surgical options depend on the stage of disease and can include synovectomy, arthrodesis, osteotomy, or arthroplasty.
1. The document discusses various congenital hand anomalies classified using the Swanson and modified classifications.
2. Key classifications include failures of formation (transverse arrest, longitudinal arrest), differentiation (syndactyly, contractures), duplication (polydactyly), overgrowth (macrodactyly), undergrowth (hypoplastic thumb), and generalized skeletal abnormalities (Apert syndrome, Poland syndrome).
3. The modified classification organizes anomalies by malformations involving the entire limb, hand plate, or unspecified structures, as well as deformities and dysplasias. Specific conditions like symbrachydactyly, triphalangeal thumb, and brachydacty
The document discusses the embryology and development of the upper limb. It covers topics like:
- The formation of the limb bud and structures involved in outgrowth like the apical ectodermal ridge and progress zone.
- How the limb bud develops and differentiates over time into the hand plate and cartilage models of bones.
- Rotation and ossification of the bones during the 7th week of development.
- Common congenital anomalies of the upper limb like syndactyly and polydactyly.
- Classification systems for various upper limb anomalies.
- Principles and timing of surgical treatment for different anomalies.
The document discusses the use of the Snow and Littler procedure for managing cleft hand deformities. It provides background on classifications of cleft hands and discusses goals of surgical management, which include releasing syndactyly and thumb adduction deformities. The Red Cross Hospital experience from 2003-2007 is described, where 20 cleft hands were treated, including 6 with the Snow and Littler procedure. Complications included finger flexion contractures and rotation of the transferred index metacarpal bone, but patients reported high satisfaction overall.
This document discusses radial club hand, which is a congenital deformity where there is failure of formation along the radial border of the upper extremity. Key points include:
- Radial club hand can range from mild shortening of the radius to total absence. It occurs in about 1 in 30,000 to 100,000 births.
- Surgical treatment involves centralizing the carpus on the distal ulna to provide support. Additional procedures like thumb reconstruction or tendon transfers may be needed.
- Nonsurgical treatment involves serial casting and splinting from birth to gradually correct the deformity. Surgery is usually done at 3-6 months if splinting is insufficient to provide radial support.
The document discusses the use of the Evans Bilobed Flap technique for managing radial club hand at the Red Cross Children's Hospital from 2010-2014. It provides a brief history of incision techniques used previously. The Evans Bilobed Flap technique provides excellent exposure of the radius and ulna as well as dorsal and volar exposure, allowing for one-stage correction. It had no major complications and provided good soft tissue coverage away from the area of potential pollicization with only a 20% minor complication rate of epidermolysis. Results on 7 patients who underwent the procedure are presented.
Common childhood problems of the lower limb (cong. & dev.)Given Sishekano
This document summarizes several congenital orthopedic conditions discussed in an orthopedics tutorial, including: developmental dysplasia of the hip, Perthes disease, recurrent dislocation of the patella, congenital talipes equinovarus (clubfoot), and syndactyly. For each condition, it describes the etiology, key features, diagnosis, and treatment approaches. Common causes of congenital orthopedic conditions include genetic disturbances, drugs, infections, trauma, anoxia, and compression in utero.
Polydactyly is a genetic mutation that results in being born with extra fingers and/or toes, usually occurring in about 1 in 500 births. It is caused by mutations on chromosome 7 and usually inherited in an autosomal dominant pattern. There are two main types: isolated post-axial polydactyly where extra digits are on the pinky side, and pre-axial where they are on the thumb side.
The document discusses upper limb orthosis, devices used to modify the structural and functional characteristics of the upper limb. It covers the objectives of upper limb orthosis including protection, correction, and assistance. It also discusses the classification, biomechanics, principles, and assessment of upper limb orthosis and provides descriptions and examples of specific upper limb orthoses including shoulder orthoses, arm slings, arm abduction orthoses, elbow/forearm/wrist orthoses, and elbow or wrist mobilization orthoses.
A child presented with a 1 month history of swelling around the left elbow associated with evening rise in temperature. They had fallen and injured the elbow 2 months prior. Examination found a 4 by 4 cm soft, swollen area over the proximal forearm with local rise in temperature. Tests revealed elevated ESR and CRP. MRI and biopsy showed evidence of tuberculous inflammation. The child underwent incision and drainage of the collection and was started on anti-tubercular treatment.
The document discusses the rare congenital condition known as windblown hand. It provides details on the condition's history, typical features, possible causes, and surgical treatments based on a doctor's experience at a children's hospital from 2003-2011. The doctor treated 10 patients over 9 years for windblown hand deformities, operating on 9 hands total. Common surgical procedures included volar z-plasty of the thumb, metacarpophalangeal joint chondrodesis, and intrinsic tendon transfers to correct contractures and ulnar deviation. Most results were considered good or fair based on post-operative function and appearance.
This document discusses pediatric flatfoot and whether it should be treated. The general consensus had been that flexible deformities did not require treatment, while rigid deformities warranted surgery. However, the document argues that misaligned feet can lead to a "chain reaction of destruction" even without symptoms. It explains that the talotarsal joint is key to proper foot alignment and discusses how its misalignment causes excessive forces to pass through the inner foot. While external supports cannot correct the underlying problem, internal stabilization of the talotarsal joint via a titanium stent can realign forces. Procedures may also include Achilles lengthening or calcaneal osteotomy. Untreated misalignment only worsens over time and can affect the entire body's
This case report describes a neonate born with fibular hemimelia, which is a rare congenital absence or deficiency of the fibula bone. Prenatal ultrasounds did not detect any abnormalities. After birth, the infant was found to have bowing of the right lower limb and absence of the second toe on the right foot. X-rays confirmed absence of the fibula and other foot bones on the right side. The report discusses the difficulty of detecting this condition prenatally using ultrasound and the treatment options of limb lengthening or amputation.
This document discusses hospital acquired infections (HAIs), also known as nosocomial infections. It provides information on the epidemiology, risk factors, commonly implicated organisms, and infection prevention and control measures for HAIs. It notes that HAIs occur in about 8.7% of hospital patients on average globally, though rates are higher in developing countries. Common types of HAIs include urinary tract infections, ventilator-associated pneumonia, and surgical site infections. Methicillin-resistant Staphylococcus aureus and other drug-resistant organisms are significant causes of HAIs. The document outlines transmission routes and precautions healthcare workers should take like hand hygiene to prevent the spread of HAIs.
This document discusses the principles and management of various congenital hand anomalies. It covers topics such as:
- Surgical management should aim to improve function and appearance with minimal risk. Hand function depends on thumb mobility and pinch grip.
- Timing of surgery depends on technical difficulty and cortical plasticity. Surgery may be delayed to treat other conditions.
- Transverse deficiencies are often managed non-surgically with prosthetics. Longitudinal deficiencies often benefit from surgery.
- Specific procedures are described for conditions like symbrachydactyly, radial hypoplasia, central ray deficiency, thumb and finger hypoplasia/aplasia. Syndactyly and poly
This document summarizes congenital radial club hand deformities. It describes the classification system including types I-IV based on the severity of radial dysplasia. Type IV is the most common, involving total absence of the radius. Associated anomalies can include deficiencies of the thumb, scaphoid, trapezium and other carpal bones. Muscles on the radial side like the brachialis and brachioradialis are often absent or hypoplastic. Associated syndromes that sometimes occur include Holt-Oram syndrome, Fanconi anemia, and thrombocytopenia-absent radius syndrome. Surgical treatment requires consideration of any associated anomalies.
The document discusses several common orthopedic hand problems including congenital anomalies, acquired deformities, infections, and contractures. It describes the pathology, clinical features, and treatment options for conditions like mallet finger, boutonniere deformity, swan neck deformity, trigger finger, Dupuytren's contracture, nail fold infection, pulp infection, and deep fascial space infections. The treatment principles focus on antibiotics, drainage of abscesses, splinting, and rehabilitation.
A congenital anomaly is something unusual or different present at birth. Minor anomalies have no serious medical or cosmetic consequences, while major anomalies like cleft lip are of serious consequence. Mental retardation refers to below-average intelligence and adaptive functioning difficulties. It can be caused by infections, genetic or chromosomal abnormalities, trauma, malnutrition or toxic exposure. While impairments limit functioning, disabilities interfere with abilities, and handicaps disadvantage due to disabilities in adjusting to environments.
The document discusses the gene TBX5 and its role in heart and limb development. It provides information on Holt-Oram syndrome, which results from mutations in the TBX5 gene and affects heart and limb formation. The document also discusses how studies of TBX5 have provided insights into T-box gene family roles in organ development and transcriptional networks that regulate heart and limb formation. It includes images of mutant mice and diagrams of proposed gene networks.
The document provides an overview of the anatomy of the hand and wrist region. It begins with definitions of key terms and descriptions of the bones that make up the hand, including 8 carpal bones, 5 metacarpal bones, and 14 phalanges. It then discusses the dorsal and palmar surfaces in more detail, describing structures like the palmar aponeurosis and flexor retinaculum. The document also provides brief summaries of the embryological development of the hand and some common congenital anomalies that can occur.
The skeletal and muscular systems develop from paraxial mesoderm, lateral plate mesoderm, and neural crest cells. Mesoderm forms somites that differentiate into sclerotome and dermomyotome, with sclerotome cells becoming mesenchyme that migrates and forms cartilage models through endochondral ossification or membraneous bone, while dermomyotome forms myoblasts that fuse into muscle fibers. Limb buds develop from lateral plate mesoderm and rotate as cartilage and bone form through endochondral ossification while surrounding musculature develops from dermomyotome and surrounding nerves
The development of the tetrapod limb involves specification of the limb field and induction of the early limb bud through FGF10 signaling. The proximal-distal axis is established by the AER, which secretes FGF8 to maintain the progress zone. The anterior-posterior axis is specified by SHH expression in the ZPA. The dorsal-ventral axis forms through Wnt7a expression on the dorsal side. Cell death regulated by BMPs then separates the digits and forms joints.
The development of the tetrapod limb involves specification of the limb field and induction of the early limb bud through FGF10 signaling. The proximal-distal axis is established by the AER, which secretes FGF8 to maintain the progress zone. The anterior-posterior axis is specified by SHH expression in the ZPA. The dorsal-ventral axis forms through Wnt7a expression on the dorsal side. Cell death regulated by BMPs then separates the digits and forms joints.
This document discusses pattern formation during embryogenesis. It begins by defining pattern formation as the development of spatial organization that establishes an organism's basic body plan and axes. Key genes that control pattern formation are homeotic genes, which regulate cell processes and contain a homeobox sequence. Hox genes pattern the anteroposterior axis by being expressed sequentially. In Drosophila melanogaster, cytoplasmic determinants, segmentation genes like gap and pair-rule genes, and homeotic genes work together to pattern each segment. Mutations in these pattern formation genes can lead to congenital malformations in humans and other organisms.
Development of Endocrine glands & Muscles (Special Embryology)Dr. Sherif Fahmy
The suprarenal gland develops from both mesodermal and ectodermal tissues. The cortex develops from mesoderm while the medulla develops from neural crest cells. The pituitary gland also has a dual origin, developing from Rathke's pouch and the infundibular process. Skeletal muscle develops from paraxial mesoderm in the somites, while smooth and cardiac muscles develop from splanchnic mesoderm. The diaphragm develops from contributions of the septum transversum, cervical myotomes, pleuro-peritoneal membrane, mesoesophagus, and thoracic wall mesoderm. It can be affected by congenital anomalies involving failure of pleuro-per
Pattern formation arises from the coordination of molecular signaling, gene expression, and cell-cell communication mechanisms across scales. Precise patterning is crucial for organ development and involves homeobox genes which regulate gene expression and cell fate. Vertebrate limb formation occurs along three axes defined by growth factors and transcription factors, which establish progenitor domains and coordinate outgrowth and digit formation.
The paraxial mesoderm lies just lateral to the notochord and gives rise to vertebrae, skeletal muscles, and skin connective tissue. It is divided into somites which then form dermomyotomes and sclerotomes. Dermomyotomes develop into dermatomes that make dermis and myotomes that form back, rib, and body wall muscles. Sclerotomes form the vertebrae and rib cage. Somitogenesis occurs through a clock-wavefront model where somites sequentially segment from cranial to caudal regions under the influence of signaling molecules like retinoic acid and FGF.
This document discusses embryology and the development of the musculoskeletal system. It covers the following key points:
1. Embryology is the study of developmental events during prenatal stages, specifically the embryonic and fetal periods. The embryonic period is the first 8 weeks when the basic body plan takes shape, and the fetal period is the remaining 30 weeks when structures continue growing.
2. Musculoskeletal development begins with the formation of somites from paraxial mesoderm, which give rise to bones, cartilage, and muscles. Bones develop through membranous or endochondral ossification. Long bones are examples of endochondral ossification, forming cartilage models that are later replaced with bone.
3. Lim
1. The Xenopus egg is polarized with a dark animal hemisphere and light vegetal hemisphere containing yolk. After fertilization, rapid cell divisions generate a mid-blastula stage embryo dependent on maternal molecules.
2. The blastula undergoes gastrulation, arranging germ layers with ectoderm outermost, endoderm innermost, and mesoderm between. Fate maps show the animal hemisphere forms ectoderm, vegetal hemisphere forms endoderm, and marginal zone forms mesoderm.
3. Following fertilization, cortical rotation breaks radial symmetry by reorienting microtubules from the vegetal pole towards the future dorsal side, transporting dorsal determinants. Disrupting this leads
coordinated movements of gastrulation begin. This dramatic process t.pdfarjunchetri1
coordinated movements of gastrulation begin. This dramatic process transforms the simple
hollow ball of cells into a multilayered structure with a central gut tube and bilateral
symmetry.many of the cells on the outside of the embryo are moved inside it. Subsequent
development depends on the interactions of the inner, outer, and middle layers of cells thus
formed: the endoderm on the inside, consisting of the cells that have moved into the interior to
form the primitive gut; the ectoderm on the outside, consisting of cells that have remained
external; and the mesoderm between them, consisting of cells that detach from the epithelium to
form a more loosely organized embryonic connective tissue.
eventual pattern of muscles-in the limbs, for example-is determined by the routes that the
migrant cells follow and the selection of sites that they colonize. The embryonic connective
tissues form the framework through which the myoblasts travel and provide signals that guide
their distribution. No matter which somite they come from, myoblasts that migrate into a
forelimb bud will form the pattern of muscles appropriate to a forelimb, and those that migrate
into a hindlimb bud will form the pattern appropriate to a hindlimb.
Limb formation results from series of epithelial-mesenchymal inductions between the
mesenchymal cells of the lateral plate mesoderm and the overlying ectodermal cells. Cells from
the lateral plate mesoderm and the myotome migrate to the limb field and proliferate to create the
limb bud. The lateral plate cells produce the cartilaginous and skeletal portions of the limb while
the myotome cells produce the muscle components. The lateral plate mesodermal cells secrete a
fibroblast growth factor (FGF7 andFGF10, presumably) to induce the overlying ectoderm to
form an important organizing structure called the apical ectodermal ridge(AER).The AER
reciprocatively secretes FGF8 and FGF4 which maintains the FGF10 signal and induces
proliferation in the mesoderm.[citation needed] The position of FGF10 expression is regulated
by Wnt8c in the hindlimb and Wnt2b in the forelimb. The forelimb and the hindlimb are
specified by their position along the anterior/posterior axis and possibly by two T-box containing
transcription factors: Tbx5 and Tbx4, respectively.
DKK1 activation in osteoblasts is the underlying cause of glucocorticoid- and estrogen
deficiency–mediated osteoporosis, and at least partially underlies the teratogenic effects of
thalidomide on limb development.
both thalidomide-induced PCD and limb deformities could be partially inhibited by blocking
Dkk1 or activating Wnt signaling downstream of the ligand-receptor interaction
Solution
coordinated movements of gastrulation begin. This dramatic process transforms the simple
hollow ball of cells into a multilayered structure with a central gut tube and bilateral
symmetry.many of the cells on the outside of the embryo are moved inside it. Subsequent
development depends on the interactions .
This document discusses the structure and growth disorders of the physis (growth plate). It begins by describing the structure of long bones and the two pathways of ossification - intramembranous and endochondral. It then focuses on the structure and microscopic anatomy of the physis, including its three zones - reserve, proliferation, and hypertrophy. Various disorders are then discussed like Gaucher's disease, diastrophic dysplasia, pseudochondroplasia, and others affecting the different zones of the physis. Treatment approaches for these conditions aim to improve function and correct deformities.
The document summarizes key aspects of embryonic development in animals, including:
- The three germ layers (endoderm, mesoderm, ectoderm) and what tissues each produces. The endoderm forms digestive tract linings, the mesoderm forms muscles and circulatory tissues, and the ectoderm forms the epidermis and nervous system.
- Cleavage patterns in early embryos, including holoblastic vs meroblastic cleavage. Cleavage planes like meridional, vertical, equatorial and latitudinal are also defined.
- Fate maps show the positions of presumptive tissues in early embryos, like those of amphioxus which mark areas that will form
This document discusses embryonic and fetal development from 3-8 weeks (embryonic period) and 9 weeks to birth (fetal period). During the embryonic period, the three germ layers give rise to specific tissues and organs as the main organ systems are established. Neurulation occurs as the neural tube forms from the neural plate. Neural crest cells migrate throughout the body. The mesoderm forms somites which differentiate into muscle, bone and skin tissues. Blood islands form and later hematopoietic stem cells arise. The endoderm forms the gastrointestinal tract. During the fetal period, organs mature and the fetus grows rapidly in the third, fourth and fifth months.
1. The primitive streak forms in the blastula and establishes bilateral symmetry and the site of gastrulation. It initiates formation of the three germ layers - ectoderm, endoderm, and mesoderm.
2. The notochord induces neural plate formation and provides organizational signals for head development. It lies along the embryo's axis and gives rise to the vertebral column.
3. Neural crest cells migrate extensively and give rise to many tissues, including those that form the skeleton and connective tissues of the head.
This document summarizes limb development in vertebrates using the chicken wing and mouse forelimb as models. It describes how the limb develops axes and patterns of differentiated tissues over time. The early limb bud consists of undifferentiated mesenchymal cells covered by ectoderm. As the bud elongates, the thickened apical ectodermal ridge and differences in expansion across the limb axes influence patterning and contribute to formation of proximal versus distal structures. While chicken wings and mouse forelimbs follow similar developmental processes, there are some differences in morphology and growth.
Development of the musculoskeletal systemSahar Hafeez
The document summarizes the development of the musculoskeletal system from early embryonic stages. It discusses how the skeletal and muscular systems originate from mesoderm and the processes of ossification and myogenesis. Key events include the formation of somites which differentiate into sclerotome and dermomyotome, the development of limb buds and their rotation, and the segmentation of axial musculature into epimere and hypomere. The document provides an overview of the embryonic development of the skeletal and muscular systems.
Development of the musculoskeletal systemSahar Hafeez
In this presentation development of the Musculoskeletal system which is one of the largest systems of human body has been described. The viewer would be able to learn about the concept of Intrauterine bone formation in general and the role of embryonic connective tissue. Also, the origin of the two muscle groups of the , Extensors & Flexors along with their motor innervation pattern has been described in this presentation.
Similar to Congenital anomalies of upper limb (20)
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About this webinar: This talk will introduce what cancer rehabilitation is, where it fits into the cancer trajectory, and who can benefit from it. In addition, the current landscape of cancer rehabilitation in Canada will be discussed and the need for advocacy to increase access to this essential component of cancer care.
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Hypertension and it's role of physiotherapy in it.Vishal kr Thakur
This particular slides consist of- what is hypertension,what are it's causes and it's effect on body, risk factors, symptoms,complications, diagnosis and role of physiotherapy in it.
This slide is very helpful for physiotherapy students and also for other medical and healthcare students.
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Hypertension, also known as high blood pressure, is a serious medical condition that occurs when blood pressure in the body's arteries is consistently too high. Blood pressure is the force of blood pushing against the walls of blood vessels as the heart pumps it. Hypertension can increase the risk of heart disease, brain disease, kidney disease, and premature death.
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3. • Limb formation begins relatively late in gestation
after the basic body plan has taken shape. The
limb buds are first visible along the embryonic
flanks in the middle (upper) and end(lower) of
the 4th week.
• The activation of mesenchymal cells derived from
lateral mesoderm (to form the limb field) appears
to be the crucial step.
• Indeed the skeletal structures of the limbs are
derived from this mesoderm as opposed to the
somitic sclerotomes that form the rest of the
skeletal system from the neck down.
4.
5. EMBRYOLOGY
• Onset of development of arm bud - 27 days
4th week
• Well-developed arm bud - 28-30 days
• Elongation of arm bud - 34-36 days
5th week
• Formation of hand paddle - 34-38 days
• Onset of finger separation - 38-40 days
• Full separation of fingers - 50-52 days
7th week
6. • Limb buds have a specialized distal
ectodermal region at the
dorsal/ventral margin called the
apical ectodermal ridge (AER), which
signals to the underlying mesoderm
called the Progress zone.
• Limb development occurs as a result
of the continuous interactions
between the ectodermal and
mesodermal components of the bud.
7. • It is not currently known what is the primary
signal for initiation of limb buds, but based on
model systems that can be manipulated
experimentally FGF 10 is a good candidate.
• FGF 10, a member of the FGF family of growth
factors is secreted locally from axial structures in
the specific regions adjacent to the limb buds,
apparently dictated by the HOX gene expression
pattern of those segments.
• FGF 10 when implanted ectopically can induce
the formation of accessory limbs. Moreover,
targeted gene ablation of FGF10 results in amelia
(No limbs).
8. • The AER is required for limb outgrowth.
• Removal of AER results in a stunted limb whose
development is arrested shortly thereafter. However
segments that have begun to form continue to do so
reasonably normally.
• The AER produces FGF 8 at an early stage, and thus
seem to take over the function from the axial
structures that initiate limb bud formation.
• Later, production of several different FGFs including
FGF2, 4, 9 and 17 by the AER appears to be necessary
for maintenance of limb development.
9. • Once initiation has occurred and migration of
mesoderm has begun, the limb field develops
independently of any signals from the axial
region.
• The AER is though to take over the function of
maintaining the limb bud.
10. • The mesoderm in the PROGRESS ZONE, just
underneath the AER is thought to be the most
important determinant of the actual limb
structures that will form.
• These mesodermal cells determine whether
for example, leg or arm structures will form.
• The formation of arm vs. leg structures
appears to be set in the progress zone
mesoderm very early by cues from the axial
skeleton.
11. • Finally, there is a time dependent change in
the cells of the progress zone that determines
the type of structures that can be formed in
the proximal-distal axis.
• Superimposed on all of these is the expression
of specific transcription factors that mediate
the cellular programs of differentiation, one of
which is the retinoic acid receptor family of
transcriptional regulators.
12. • A family of transcription factors, one specific
for arm and a related one for leg, are turned
on in the limb buds and appear to be
important in the arm/leg distinctions.
• In general, most of the GFs and TFs produced
are similar in the Upper and lower limb buds.
• The mesoderm in the progress zone is also
thought to set the time clock for the proximal
distal axis. Cells leaving this region are fixed in
their developmental potential and those
leaving earlier have different capabilities than
those leaving later on.
13. • Limb development occurs in dimensions, each
controlled by a separate process and different
growth factors.
• First is the proximal distal Axis, initiated by
signals from axial structures, including the
secretion of FGF, and later maintained by the
AER.
• FGF gene: Produced at apical ectodermal
ridge. Stimulates mitosis of underlying
mesoderm, providing for lengthening of limbs.
14.
15. • Second there is the anterior posterior axis that
specifies the position of the thumb versus little
finger, for example
• A region located along the posterior side of the
limb bud called the Zone of polarizing activity
(ZPA), controls the anterior-posterior process.
This zone produces SHH gene which is critical for
its function.
• Sonic hedgehog gene: Produced at base of limbs
in zone of polarizing activity. Involved in
patterning along anterior-posterior axis. Involved
in CNS development; mutation can cause
holoprosencephaly.
16. • The ZPA is located in the posterior mesoderm.
• It is responsible for establishing polarization along the
A-P axis.
• SHH Gene produced by the ZPA is necessary for its
function.
• SHH production is sustained by FGF produced by AER.
• The signals from the ZPA promote a shift from the
proximal to distal pattern of the HOX gene transcripts
to an anterior-to-posterior pattern.
• It is clear that the HOX gene expression is critical for
cells to understand the positional information they are
receiving during development.
17.
18. • Third there is the dorsal/ventral axis, which
involves the ectoderm and a growth factor of
the WNT family.
• Wnt-7 gene Produced at apical ectodermal
ridge (thickened ectoderm at distal end of
each developing limb). Necessary for proper
organization along dorsal-ventral axis.
19. .
• Directed by the ectoderm skin from back of palm
is different from that on the back.
• It is possible to detach the ectoderm from the
mesoderm in a limb bud.
• The dorsal ectoderm produces the growth factor
Wnt7, a member of the Wnt family which has
been shown to be necessary for dorsalization.
• Ectopic expression of Wnt7 can dorsalize a ventral
surface and ablation of Wnt7a results in
ventralization of the dorsal limb.
20. • Wnt7a signaling from the ectoderm is critical for
the dorsal expression of the transcription factor
Lmx1b in the distal mesenchyme.
• Lmx1b is the gene defective in human nail-patella
syndrome - xterised by abnormal development of
dorsal limb structures, such as nails and patella.
• Engrailed-1, another TF, is expressed in the
ventral ectoderm and is required for specifying
ventral limb fates by repression of the dorsalizing
genes WNT7a and indirectly Lmx1b.
21. • Perhaps most important is the regional
expression of HOX(Homeobox) gene family
members (paralogues). As was true in the
axial region of the embryo, HOX genes encode
transcription factors that control the
formation of particular structures (e.g. type of
digit) in specific regions of the limb.
• Homeobox (Hox) genes: Involved in segmental
organization of embryo in a craniocaudal
direction. Hox mutations = appendages in
wrong locations.
22. • Finally, the differentiation of the mesoderm
into the various tissues inside the limb= bone,
cartilage, muscle, CT, and blood vessels, are
controlled by another series of growth factors.
• Bone Morphogenic Proteins (BMP’s) initially
play a role downstream of SHH in patterning
the A-P axis of the limb and then later
function to induce cartilage and bone
formation.
• It is fairly obvious that the development of the
limb has implications not only for congenital
malformation of the limbs, but also for tissue
regeneration and wound healing processes
23. • These cells have received specific positional
information that will determine the type of
tissue they will become.
• This is best illustrated when an older limb bud
is substituted for a younger one. Only distal
structures are produced. It is too late for the
proximal structures to form.
• Conversely when a young bud is grafted into
an old stump, the proximal structure are
duplicated.
24. Axis Signaling Center Molecular signal
Proximal/Distal
FGF 17
Apical Ectodermal Ridge
Limb mesenchyme
FGF2, FGF4,FGF8, FGF9
FGF10
Anterior/Posterior Zone of polarizing
activity
Sonic Hedgehog, BMP’s
Dorsal-Ventral Dorsal ectoderm
Dorsal mesenchyme
Ventral ectoderm
WNT7a
LMX1b
Engrailed-1
25. Summary of Embryology
• Upper limb bud
Develops from lateral wall of embryo .
On 4th wk after fertilization .
Consists of mesodermal cells covered by
ectoderm .
Under guidance of three signaling centers:
1. AER (apical ectodermal ridge) - proximal-
distal.
2. ZPA (zone of polarizing activity) – anterior-
posterior growth.
3. NRE (non ridge ectoderm)(wing less type) -
dorso-ventral growth.
26. • Mesoderm: blood vessels, muscle, bone,
connective tissue and cartilage.
• Lateral plate mesoderm migrating into the
region can only make certain tissue: blood
vessels, connective tissue, bone, cartilage
• Somitic Myotomes make the muscle masses.
• All bones in the limbs initially form as cartilage
models and ossify as endochondral bones.
27. • ASSH: American society for surgery of the hand
• IFSSH: International federation of societies for
surgery of the hand
• ISPO: International society of prosthetics and
orthotics
• Pre-axial: refers to the lateral/radial aspect of the
forearm
• Post-axial: refers to the medial/ulnar aspect of
the forearm
• Central: when there is absence of digits or
metacarpals in the central region of the hand.
28. Diagnosis before birth
Antenatal diagnosis by ultrasound is
increasingly possible, particularly in cases
of aplasia.
This will prepare the parents for the
deficiency before birth.
29. SWANSON Classification
• The IFSSH proposed a 7 categories
classification based on the proposed
classification of Swanson et al in 1976.
• This classification was based on
etiopathogenic pathways.
30. SWANSON CLASSIFICATION
• Accepted by IFSSH & ASSH.
• Based on their embryologic origin &
morphological appearance.
• Expanded by Knight & Kay in 2000, & Upton
in 2006.
• But recently , adequacy of this classification
has been questioned.
31. Swanson classification
• Failure of formation of parts (Transverse or
Longitudinal)
• Failure of differentiation or separation of parts
• Duplication (Polydactyl)
• Overgrowth
• Undergrowth
• Congenital constriction ring syndrome (Amniotic
band syndrome).
• Generalized skeletal abnormalities & syndromes
32. 1. Failure of formation of parts
• Detected prenatally.
2 types:
A. Transverse arrest
Complete deficiencies
B. Longitudinal arrest:
radial club hand (pre-axial arrest)
ulnar club hand (post-axial arrest)
cleft hand (central arrest)
phocomelia (intercalary arrest) – an intervening
segment of limb is absent.
33. A. Congenital transverse arrest
• Rare, always U/L.
• Sporadic / environmental.
• Level defined by skeletal absence.
• Commonly at level of proximal forearm.
• Defect in AER signaling
• Lt > Rt
• 2 groups:
defect in limb formation
Intrauterine amputation after limb formation
• Most will not require surgery, but benefit from
prosthesis if referred early. Recently developed
myoelectric prostheses enable muscle action to trigger
the prosthesis.
34. B. Longitudinal arrest
I) Phocomelia (seal limb)
• Intercalary arrest
• Absence of the entire UL Is termed Amelia.
• Phocomelia on the other hand is used when a functional
terminal unit is present.
• Intervening segment of limb is absent (arm/forearm)
• Thalidomide in 1st trimester (an anti-emetic)
• Type I (complete) - hand directly attached to trunk
(absence of forearm and arm)
Type II (proximal) – short forearm(with the hand attached to
it) attaches to trunk (absence of arm).
Type III (distal) – short humerus attached to hand i.e. hand is
attached to the arm at the elbow (absence of forearm).
• Surgery – very little role.
35.
36. Pre-axial Deficiencies
• Include a variety of conditions varying from minor
abnormalities of the thenar muscles to complete absence of
pre-axial (radial/lateral structures.
• Most common presentation of pre-axial deficiency is radial
club hand.
• The term radial club hand comprises a spectrum of disorders
consisting of a
Dysplastic radial bone including a radially deviated flexed
hand
Absence of scaphoid and trapezium
And either a Hypoplastic or absent thumb
• Unilateral or bilateral but rarely occurs symmetrically on both
sides.
• Incidence 1:30,000 (M) and 1:100,000 (F)live births.
37.
38. ii) Radial ray dysplasia (aka: Radial club hand/Pre-axial
deficiency/Longitudinal radial deficiency)
• Clinical features of radial club hand include:
Radially deviated, flexed hand with pronated and
shortened forearm
The radius is either partially or completely absent,
being replaced by fibrous band (anlage)
The thumb is either absent or Hypoplastic
Hypoplasia of other radial structures such as the
scaphoid, trapezium, 1st metacarpal, radial artery,
superficial branch of the radial nerve and radial-sided
musculature.
Stiffness of the elbow due to synostosis (bony fusion)
Stiffness of the fingers due to stretching of the tendons
caused by radial deviation of the hand.
39. • Normal ulnar two digits
• Median nerve subluxed towards concave side.
• U/L , M>F , Rt>Lt .
• Radial club hand (and pre-axial deficiencies in general)
is associated with a variety of syndromes affecting the
CVS, GIT and hematological systems:
Holt-Oram syndrome: cardiac abnormalities,
commonly septal defects and radial club hand
VATERR Syndrome: also referred to as VACTERL;
consists of Vertebral anomalies, anal atresia, Cardiac,
Esophageal, Renal and Limb anomalies.
TAR syndrome: thrombocytopenia @ birth which
improves with time and radial club hand
Fanconi Anemia: Aplastic anemia which develops at
around 6 years of age and radial club hand.
40. Classification…(O’Rahilly, Bayne &
Klug)
• I –Short distal radius with no radial bowing or
deviation; Mildest type; No Rx.
II – Hypoplastic radius (Rare); No Rx.
III – Partial absence of radius (replaced by
anlage); Most common; centralization
procedure required
IV – Complete absence of radius – most severe
& second most common. Requires soft tissue
release and centralization.
45. Bayne & Klug classification of radial
longitudinal deficiency
46. • Maternal drug exposure, compression of the
uterus, vascular injury, ?Apical Ectodermal Ridge
(AER) Defect.
• Treatment of radial club hand (and pre-axial
deficiencies in general) is dependent on the Px. Age,
severity of deformity and extent of functional deficit.
• Conservative Mgt. Vs. Surgical mgt.
• Conservative mgt. which include Splints, casts and
passive stretching techniques which helps to reduce
stiffness and maintain soft tissue length.
• Surgical Mgt. which includes soft tissue release,
centralization and radialization procedures, thumb
reconstruction via pollicization and tendon transfers.
47. iii) Ulnar ray dysplasia (aka: Ulnar club hand
/Postaxial deficiency)
• Rarest of longitudinal ray deficiency.
• Compared to radial club hand, has a larger spectrum of
abnormalities ranging from minimal hypoplasia of the
ulnar digits to total absence of the ulna.
• Short, bowed radius with a Hypoplastic or absent ulna.
• AD pattern of inheritance.
• Association with syndromes – uncommon
• Disruption of ZPA signaling.
• Elbow severely affected (with a relatively stable wrist).
• M>F , Lt>Rt , U/L>B/L.
48. Ulnar club hand differs from radial club
hand in a number of key ways..
Ulnar Club hand Radial Club Hand
• Elbow joint is unstable
while the wrist is stable
• Ulnar is typically only
partially absent
• Wrist is unstable while
elbow is either stiff or
stable.
• Radius is usually totally
absent.
• Syndromic associations
are rare, but if they do
occur involve the MSS
• Syndromic associations
are common and always
involve either the
cardiac, GIT or
hematological systems.
49. Classification
• The most commonly used classification for ulnar
club hand is that which has been proposed by
Bayne.
Type 1: hypoplasia of the ulna with minimal
deformity.
Type 2: partial absence of the ulnar
Type 3: total absence of ulnar with anlage and a
normal radiohumoral joint.
Type 4: radius is fused to the humerus with no
elbow joint (radiohumoral synostosis)
50. Bayne
classification
Paley & Herzenberg
classification
• I – Ulnar hypoplasia
• II – Partial ulnar
aplasia
• III- Total ulnar aplasia
• IV – Radiohumeral
synostosis
• I – Ulnar hypoplasia with intact distal
epiphysis
• II – Partial ulnar aplasia (distal 1/3rd )
• III –Partial ulnar aplasia (distal 2/3rd )
• IV – Total ulnar aplasia
• V – Radiohumeral synostosis
51. Bayne & Klug classification of ulnar
longitudinal ray deficiency
52. • Involves hand therapy and splinting to
improve the range of motion.
• Surgical intervention where appropriate
• Surgery usually involves release of the fibrous
anlage and realignment of the carpus and
forearm.
53. iv) Central ray deficiency /cleft
hand
• Involves an absence of digits or
metacarpals of the central portion of the
hand.
• Most common longitudinal deficiency.
• Defect in AER signaling.
• B/L (frequently).
• Divided into typical and atypical types.
• Structures proximal to wrist – normal.
• Little finger – always present
• Associated syndactyly & narrow web
space.
54. • Complex syndactyly (thumb & index) – in
severe case.
• Hand – “functionally good but aesthetically a
disaster’’.
• Association with cleft feet in 1/3rd cases
(absence of the 3rd toe of the foot) (SHSF)
• Other Syndromic associations: EEC
syndrome(ectrodactyly, ectodermal dysplasia,
cleft lip/palate)
55. Typical cleft hand Atypical cleft hand
• Deep V shaped central defect
• Bilateral
• Inherited (AD)
• Cleft feet associated (3rd toe of
foot)
• Partial or complete absence of
the middle finger (central ray) in
the hand.
• Thumb involved
• Associated cleft lip/palate
• No chest wall involvement
• Little finger – only digit
• No finger nubbins (buds)
• Shallow U shaped defect
• Unilateral
• Sporadic
• Not associated with cleft feet
• Rays of central 3 digits(index,
long and ring fingers)
• Rarely involves thumb
• No associated cleft lip/palate
• Seen in Poland syndrome
• Thumb - only digit
• Finger nubbins may occur
• Now recognized as a form of
symbracydactyly.
57. Manske’s classification of cleft hand
• Type I – Normal 1st web
• Type II
A – mildly narrowed web
B – severely narrowed
• Type III – Syndactylised web
• Type IV – Merged web
• Type V – Absent web
A – Partial suppression of
radial ray
B – Complete suppression of
radial ray
58. • Where possible is via surgical intervention to
improve cosmesis and function.
59. • Soft tissue
1. Syndactyly
2. Camptodactyly
3. Trigger thumb
4. Clasped thumb
• Skeletal
1. Clinodactyly
2. Symphalangism
3. Synostosis
4. Arthrogryposis
5. Windblown hand
60. • The term “SYN” is derived from Greek and
means “together” while “dactyly” is also
derived from the Greek term “dactylos” which
means “digits”.
• Both of them used together describes a
condition where the fingers of the hand are
joined together.
• Syndactyly is thought to be caused by a failure
of programmed cell death (apoptosis) in the
interdigital tissue.
61. • When all the different varieties of Syndactyly
are counted together, it becomes the most
common congenital deformity of the hand.
• Incidence of 1 in 1000-2000 live births.
• Males > female
• 30% Bilateral
• 20% of cases have a positive family history.
• Thought to be associated with at least 28
other syndromes including Apert’s and
Poland’s syndrome.
62. • 3rd web > 4th web > 2nd web
• Foot may also be affected, with the commonest form being
an incomplete second web space syndactyly.
• Syndactyly is classified as follows:
1. Complete: digits are fused together up to the level of the
tips of the fingers
2. Incomplete: fusion of the digits does not reach up to the
level of the fingers.
3. Simple: connection between the digits only involves soft
tissue.
4. Complex: in addition to soft tissue connections, there are
also bony connections between the fingers.
5. Acrosyndactyly: there is distal soft tissue and bony
connections (i.e. complex Syndactyly) but proximally the
web space is present to a degree, resulting in small spaces
referred to as fenestrations.
63. • Complicated Syndactyly
More than only distal bony fusion
Abnormal bone structure inside (fusion
,missing bone, abnormal joints, rudimentary
bones ,cross bones)
Seen in ▪Apert syndrome,
▪Central synpolydactyly
▪Typical cleft hand
66. • Involves surgical correction of deformity.
• Goals of surgery include:
a. Separation of the digits
b. Creation of a web space
c. Skin coverage
d. Immobilization and avoidance of scars.
The timing of surgery depends on the deformity.
• Surgery to release fingers of unequal length, such
as the ring and the little finger, thumb and index
finger, maybe carried out as early as 6 months.
67. • Complex and Acrosyndactyly are also often
released early as the bone to bone
connections between the digits may adversely
affect growth.
• Most other less severe deformities can be
deferred to age 12-36 months.
68. A number of surgical techniques exist but most
share the following principles
• If several digits are involved, the border digits are
released first.
• If a digit is joined on both sides a single stage
release procedure to separate that finger should
be avoided as this may adversely impact finger
vascularity.
• Zigzag incisions should be used and straight line
incisions avoided along the borders of digits to
reduce the likelihood of scar contracture which
may lead to digital deformity.
69. • A proximally based dorsal flap is usually used
to reconstruct the web space as skin grafts in
this area may cause a partial recurrence of
Syndactyly
• Defects along the borders of the digits are
closed via volar and dorsal interdigitating
flaps.
• Any other resultant defects are closed via
FTSG
• Skeletal abnormalities need to be corrected.
• Reconstruction of a normal nail fold.
70. )
• Congenital flexion deformity of the PIPJ.
• Painless, progressive flexion Contracture of
PIPJ( antero-posteriorly).
• Most commonly involves the little finger (70%)
although other fingers could be involved.
• Presents at two distinct time periods:
Infancy – 1-4 yrs. with an equal sex incidence
Adolescence – 10-14 yrs. Occurs more
commonly in girls.
71. • 3 types:
type I . newborn (M=F)
type II. adolescent females
type III. Multiple digits/with
syndromes
72. • Almost every structure in the region of
PIPJ has been implicated in the
pathogenesis of camptodactyly although
the most common causes seem to be:
Either an abnormal lumbrical insertion
An abnormal flexor digitorum
superficialis (FDS) insertion.
Or simply Due to imbalance in flexors &
extensors
73. .
• Initial Rx. Is conservative – splinting and
stretching.
• If conservative mgt. fails or there is a rapidly
progressing deformity of the finger, then surgical
intervention is considered.
• Surgery can be deceptively complex and may
involve
1. Release of any anomalous lumbricals or
lengthening of a tight FDS
2. Agulation osteotomy or arthrodesis of the joint.
74. • Presents in infants with similar clinical features to
that in adults
• Includes a thumb that clicks, catches or locks as the
Interphalangeal joint moves from a flexed to an
extended position.
• IP joint is usually held in a flexed position by the
child.
• 25% present at birth; 25% are B/L
• 30% resolve spontaneously by 1 yr. of age
• Nodule known as “Notta’s node” is usually palpable
over the flexor aspect of the MCP joint of thumb
proximal to A1 pulley.
75. • Snapping/popping as the nodule passes beneath
A1 pulley.
• Stenosing tenosynovitis of FPL tendon at A1
pulley
• Compensatory hyperextension at MCPJ.
76.
77.
78.
79. • Initially stretching exercises + splintage are
used.
• If above fails, surgical release of the A1 pulley
is carried – provides excellent result with few
complications.
80. Congenital clasped thumb
• Deficient thumb extensor mechanism.
• Mild clasped (type I) – deficiency of EPB .
Extension lag at MCPJ.
• Severe clasped (type II) – deficiency of EPB &
EPL - Extension lag at both MCPJ & IPJ.
• Type III – clasped associated with
arthrogryposis
81. • Splintage in ext. for up to 6 months
• If conservative Rx. Fails or there is significant
adduction contraction, release of the first web
space may be indicated.
• Tendon transfers, tendon grafts and
arthrodesis may be indicated in more severe
cases.
82. • Progressive Palmar radial curvature of the
distal phalanx of little finger.(deviation in 2
planes)
• Distortion & widening of epiphyseal plate along
with curvature of the diaphysis of the distal
phalanx.
83. • Xterised by curvature of a digit in a radio ulnar
plane (>100) distal to MCPJ.
• Most common presentation is inward (radial)
deviation of the little finger @ DIP. (Middle
phalanx of little finger - last bone to ossify)
• Proximal phalanx of thumb – 2nd most common
• The pathoanatomical basis of clinodactyly rests
with an abnormally shaped middle phalanx
known as a DELTA phalanx.
84. • Due to an abnormality of its epiphysis, the
delta phalanx assumes an abnormal
Trapezoidal shape, which in turn gives rise to
curving of the digit.
85. • Most patients with this condition do not have
any functional deficits and hence surgery is
not indicated purely for aesthetic purposes.
• Wedge osteotomy techniques can be
employed In cases of severe angulation and
interference with function.
86. • Harvey Cushing coined the term
• To describe the congenital stiffness of the
PIPJs. (most commonly)
• Has now been extended to include stiffness of
the DIPJs as well.
• Caused by failure of differentiation of the
Interphalangeal joint – there is absence of the
joint capsule.
• Occurs in assoc. with a number of other
conditions including, Apert’s syndrome,
Poland’s Syndrome and Radial club hand.
87. • Usually conservatively as the hand functions
quite well.
• Mainstay of treatment involves hand therapy
and dynamic splintage.
• Arthroplasty and Arthrodesis may be
considered following skeletal maturity to
obtain a more functional position.
88. • This involves the fusion of 2 bones
• Either in a partial manner permitting some
degree of movt. Or in a complete manner
permitting absolutely no movement at the
joint.
• Can occur at any site along the UL where there
are two bones adjacent to one another.
• Can occur between phalanges (complex
Syndactyly), metacarpal bones, carpal bones
and the radius and ulnar.
90. • Non progressive multiple congenital joint contractures
• An abnormality of the nerves in the motor unit is
thought to be the cause of this condition.
• Elbow – most commonly with lack of flexion.
• Amyoplasia – classic type
▪symmetric limbs
▪shoulder- adducted , internally rotated
▪elbow –extension
▪forearm- pronation
▪wrist – flexion
▪hand – club-like, ulnar deviation
▪thumb – flexed, adducted
▪fingers - flexed
91. Treatment is usually confined to dynamic and static splinting
with surgery being indicated to correct specific functional
problems.
92. • Fingers are deviated in an ulnar direction
• Severe hyper flexion of fingers at MCPJ with
ulnar deviation .
• Due to metacarpal bone shortening.
• “Whistling face” syndrome
• Usually B/L
• Progressively worse as the child matures
• Treatment is surgical and involves reconstruction
of the deformed hand.
93.
94.
95. • In duplication, the entire limb or part of the limb may
be duplicated. Polydactyly refers to the presence of
more than 5 fingers in the hand. It is the most common
congenital anomaly in Upper extremity.
• It can occur on its own or as a condition associated
with a syndrome.
• Classed into:
Ulnar polydactyly (Post-axial)
Central polydactyly
Radial polydactyly (Pre-axial)
Ulnar dimelia
96. • Post axial polydactyly; Africans; syndromic
• 8x as common as polydactyly of the other fingers
• Stelling has divided ulnar polydactyly (duplication
of the little finger) into 3 types:
1. Type 1: a mass of soft tissue lacking a skeletal
structure attached to the post axial region.
2. Type 2: an extra digit with all the normal
components articulating with a normal or bifid
Metacarpal or phalanx.
3. Type 3; an extra digit articulating with an extra
metacarpal.
Treatment involves removal of the extra digit in 2
and 3 and removal of skin tag in 1.
98. • Duplication of the index, middle and ring fingers
• Seen in association with varying degrees of
Syndactyly, and in this case it is referred to as
POLYSYNDACTYLY.
• Surgical intervention usually involves release of
the Syndactyly, removal of the extra digit and
augmentation of the retained digit via
reconstruction.
• Complex operative procedure; requires more
than one operation.
99.
100. • Generally thumb duplication
• Usually U/L; Asians; isolated
• Wassell classified it into 7 types
i. Bifid distal Phalanx
ii. Duplicated distal phalanx
iii. Bifid proximal phalanx with duplicated distal phalanx
iv. Duplicated proximal and distal phalanx (Most
Common; 44% of cases)
v. Bifid metacarpal with duplication of the proximal and
distal phalanx.
vi. Duplicated metacarpal, proximal and distal phalanx
vii. Triphalangeal thumb
101.
102.
103. • Rare condition
• Duplication of the ulna, absence of the radius and
7 or 8 fingers; A central digit with 3 digits (long,
ring, little) on either side with no thumb.
• Sometimes referred to as Mirror hand.
• Due to transplantation/replication of ZPA
• There is usually stiffness of the elbow joint with
reduced forearm rotation
• Treatment involves excision of the Olecranon (to
improve elbow stiffness) and excess fingers.
104. • Macrodactyly: Only deformity in this group
• Enlargement of all structures within a digit
• True macrodactyly needs to be differentiated
from other causes of enlarged digits such as
vascular malformations, osteoid osteomas,
fibrous dysplasia and lipomatoses.
• Most common presentation is macrodactyly
associated with nerve-orientated
lipofibromatosis.
105. • In this condition, there is adipose tissue and
fibroblast deposition in the cutaneous nerve
supplying the digit (leading to its
enlargement) and hence it is alternatively
described as nerve territory orientated
macrodactyly.
• Most commonly occurs in the median nerve
distribution supplying the index finger and is
usually U/L.
106. Classification
• Flatt’s classification -
Type I – lipofibromatosis
Type II- neurofibromatosis
Type III- hyperostosis
Type IV- Gigantism and hemihypertrophy
• Majorly into 2 types
1. Static Macrodactyly: enlarged digit grows in
proportion with the rest of the child’s body
2. Progressive Macrodactyly: the enlarged digit
grows progressively out of proportion with the
rest of the child’s body
108. • Fingers that are shorter than normal
• Occurs in association with symbrachydactyly
where the fingers are replaced by small
nubbins
• Treated either via distraction, free phalangeal
transfers from the toes or free microvascular
joint or bone transfer.
109. Thumb Hypoplasia
• The thumb is the digit that is most commonly
hypoplastic.
classified thumb hypoplasia into 5
types
Type 1: small thumb with normal
components. This deformity does not affect
function adversely and hence in the vast
majority of cases does not require surgical
correction.
110. Type 2: the thenar muscles are hypoplastic. The
first web space is narrowed due to adduction
contracture and the MCP joint is unstable due to
laxity of the ulnar collateral ligament. The skeletal
structure of the thumb is however normal.
Treated via release of the 1st web space and an
opponensplasty (an operation where a muscle is
transferred to restore thumb opposition)
Type 3: there is hypoplasia of the skeletal
structure + intrinsic muscles with abnormalities
of the extrinsic tendons. A - stable CMC joint, B-
unstable CMC joint. It is treated by amputation
and pollicization of the index finger (index finger
is removed and used to create thumb) or by soft
tissue and skeletal re-construction.
111. Type 4: A small thumb is attached to the hand
via a skin bridge; floating thumb (pouce
Flottant)
Type 5: Aplasia. Both types 4 and 5 are treated
via pollicization of the index finger.
112. Madelung’s deformity
• Radial & palmar angulation of distal radius
• Ulnar & palmar part of distal radial physis –
growth disturbance point.
113. A growth disturbance in
the volar-ulnar distal
radial physis
volar and ulnar tilted
distal radial articular
surface,
volar translation of the
hand and wrist,
a dorsally prominent
distal ulna.
114.
115. • Adolescent females
• Pain, Decreased range of motion, and
deformity.
• Genetic etiology and is associated with
mesomelic dwarfism and a mutation on
the X chromosome.
• Surgery involves addressing the deforming
bony and ligamentous lesions, correcting
the abnormal position of the radial articular
surface, and equalizing the longitudinal
levels of the distal radius and ulna
116. )
• Involves the presence of tight bands around any
part of a limb or digit.
• May encircle entire circumference or partially
encircle.
• 1:15000 live births.
• Two hypotheses to its etiology
1. First one proposes that it is caused by extrinsic
amniotic bands encircling the limb in utero
2. Proposes that it is caused by an intrinsic defect
in the germ cell layer.
117. • Categorized by Paterson into 4 main groups
A. Type 1: simple constriction ring involving only
a groove in the skin (partial/circumferential)
B. Type 2: Constriction ring with distal
lymphedema
C. Type 3: constriction ring associated with
fusion of digits distally (known as
Acrosyndactyly – fenestrated Syndactyly)
D. Type 4: intrauterine amputation.
118.
119.
120. • Typically involves excision of the ring via
multiple Z-plasty or W-plasty.
• If Acrosyndactyly is present, it should be
released within the 1st year of life to enable
the digits to grow normally.
121. Generalized skeletal abnormalities
• Most common – multiple exostoses.
• Others –
Poland syndrome(symbrachydactyly)
Apert syndrome(complex syndactyly)
Haas syndrome
Freeman Sheldon syndrome (wind blown hand)
Mohr Wriedt syndrome (radial clinodactyly of
index finger)
Pierre – Robin syndrome (clasped thumb).
122. • Craniofacial
anomalies with
mitten hands.
• Apert syndrome is
a form of
acrocephalosyndac
tyly, a congenital
disorder
characterized by
malformations of
the skull, face,
hands and feet
123. • It is classified as a branchial arch
syndrome, affecting the first branchial (or
pharyngeal) arch, the precursor of the
maxilla and mandible
• Disturbances in the development of the
branchial arches in fetal development
create lasting and widespread effects.
124.
125. Apert syndrome (acrocephalosyndactyly)
• Characterized by
1. Craniosynostosis .
2. Acrosyndactyly.
3. Symphalangism
(2nd , 3rd , 4th finger)
4. Radial clinodactyly of
thumb.
5. Simple Syndactyly of 5th
finger (4th web)
• Upton classification –
Type I – Spade / obstetrician hand
(thumb & little fingers are free)
Type II – Mitten / spoon hand
(only thumb is free)
Type III – Rosebud / hoof hand
( all fused)
126. B. Haas syndrome
• Presence of 6 metacarpals is characteristic
• >5 digits
• All having 3 phalanges
127. Modified classification of congenital
anomalies of hand & upper limb
I. Malformation
A.Failure in axis formation & differentiation – entire upper limb
B.Failure in axis formation & differentiation – hand plate
C.Failure in hand plate formation & differentiation – unspecified axis
II. Deformations –
1. Constriction ring syndrome
III. Dysplasias
1.Macrodactyly
2.Limb hypertrophy
3.Tumorous conditions
129. Symbrachydactyly
• Type 1- triphalangeal type
• Type 2 – diphalangeal type
• Type 3 - monophalangeal type
• Type 4 – aphalangeal type
• Type 5 – ametacarpia type
• Type 6 – acarpia type
• Type 7 – forearm amputation type
132. B. Failure in axis formation &
differentiation – hand plate
1.Radial polydactyly
2.Ulnar polydactyly
3.Triphalangeal thumb
1.Dorsal dimelia
(palmar nail)
2.hypoplastic/aplastic
nail
Radio-ulnar(AP) axis Dorsal ventral axis
133. Dorsal Dimelia of little finger
• Failure in axis formation & differentiation in
hand plate
• Involves dorso-ventral axis.
• Non ridge ectoderm – signaling centre.
• Palmar nail.
134. Triphalangeal thumb
• AD
• Extra phalanx of variable size, variable
shape(triangular/trapezoid/rectangular)
normal appearing thumb
• Fully developed extra phalanx lying in the finger plane
- considered as five fingered hand
- absent thumb with index polydactyly
136. Brachydactyly
• Bell’s classification-
A. Brachymesophalangy
B. Apical dystrophy
C. Drinkwater type
D. Brachymegalodactyly
(stub thumb)
E. Brachymetacarpia
• Mohr – Wriedt syndrome –
Radial deviation (clinodactyly) of index finger
due to brachydactyly(middle phalanx of index)