Muscular Dystrophy Amjad Moiffak Moreden, M.D. Department of Orthopaedic Surgery The General...

MuscularMuscularDystrophyDystrophy

Amjad Moiffak Moreden, M.D.Amjad Moiffak Moreden, M.D.Department of Orthopaedic SurgeryDepartment of Orthopaedic Surgery

The General Assembly of Damascus HospitalThe General Assembly of Damascus HospitalMinistry of HealthMinistry of HealthDamascus, SyriaDamascus, Syria

Jun. 26, 2007Jun. 26, 2007

IntroductionIntroduction

Muscular dystrophy (MD) is a group of rare inherited Muscular dystrophy (MD) is a group of rare inherited muscle diseases in which muscle fibers are unusually muscle diseases in which muscle fibers are unusually susceptible to damage. Muscles, primarily voluntary susceptible to damage. Muscles, primarily voluntary muscles, become progressively weaker. In the late muscles, become progressively weaker. In the late stages of muscular dystrophy, fat and connective stages of muscular dystrophy, fat and connective tissue often replace muscle fibers. In some types of tissue often replace muscle fibers. In some types of muscular dystrophy, heart muscles, other involuntary muscular dystrophy, heart muscles, other involuntary muscles and other organs are affected.muscles and other organs are affected.

The most common types of muscular dystrophy The most common types of muscular dystrophy appear to be due to a genetic deficiency of the appear to be due to a genetic deficiency of the muscle protein dystrophin.muscle protein dystrophin.

There's no cure for muscular dystrophy, but There's no cure for muscular dystrophy, but medications and therapy can slow the course of the medications and therapy can slow the course of the disease.disease.

DefinitionDefinition

“muscular dystrophy” (MD) describes a group of primary genetic disorders of muscle that often have a distinctive and recognizable clinical phenotype, accompanied by characteristic, but frequently not pathognemonic, pathological features.

Muscular Dystrophy Types and Genes Duchenne MD DMD Dystrophinopathy Xp21

Becker MD BMD Dystrophinopathy Xp21 Emery-Dreifuss MD EDMD Xq28 Dominant Emery-Dreifuss MD AD-EDMD 1q11 Limb-girdle MD type 1A LGMD1A 5q Limb-girdle MD type 1B LGMD1B 1q11 Limb-girdle MD type 1C LGMD1C p25 Limb-hirdle MD type 1D LGMD1D 6q22 Limb-girdle MD type 1E LGMD1E 7q Limb-girdle MD type 2A LGMD2A Calpainopathy 15q15 Limb-girdle MD type 2B LGMD2B Dysferlinopathy 2p13 Miyoshi myopathy MM Dysferlinopathy 2p13 Miyoshi-type MD MMD 10 α-Sarcoglycanopathy SGCA LGMD2D, SCARMD2 17q21 β-Sarcoglycanopathy SGCB LGMD2E 4q12 γ-Sarcoglycanopathy SGCC LGMD2C, SCARMD1 13q12 δ-Sarcoglycanopathy SGCD LGMD2F 5q33 Limb-girdle MD type 2G LGMD2G 17q11 Limb-girdle MD type 2H LGMD2H 9q31 Limb-girdle MD type 2I LGMD2I 19q13-3 Merosin-negative congenital MD 6q22 Congenital MD with rigid spine 1p35 Fukuyama congenital MD FCMD 9q31 Congenital Myopathy (or ?MD) 12q13 Facioscapulohumeral MD FSHD 4q35 Myotonic dystrophy DM 19q13 Myotonic dystrophy type 2 DM2 3q Oculopharyngeal MD OPMD 14q11 Epidermolysis bullosa simplex MD-EBS 8q24

M.D. TypesM.D. Types

There are nine major types of MD There are nine major types of MD affecting people of all ages, from affecting people of all ages, from infancy to middle age or later. The infancy to middle age or later. The two most common types of MD affect two most common types of MD affect children: children:

Duchenne muscular dystrophy Duchenne muscular dystrophy (DMD) (DMD)

Becker muscular dystrophy (BMD) Becker muscular dystrophy (BMD)

Signs and symptomsSigns and symptoms

They vary according to the type of muscular They vary according to the type of muscular dystrophy. In general, they may include:dystrophy. In general, they may include:

Muscle weakness Muscle weakness Apparent lack of coordination Apparent lack of coordination Progressive crippling, resulting in contractures Progressive crippling, resulting in contractures

of the muscles around your joints and loss of of the muscles around your joints and loss of mobility mobility

Many specific signs and symptoms vary from Many specific signs and symptoms vary from among the different forms of MD. Each type is among the different forms of MD. Each type is different in the age of onset, what parts of the different in the age of onset, what parts of the body the symptoms primarily affect and how body the symptoms primarily affect and how rapidly the disease progresses.rapidly the disease progresses.

Signs and symptomsSigns and symptomsCont..Cont..

DystrophinopathiesDystrophinopathiesThese types of muscular dystrophies These types of muscular dystrophies are due to a genetic deficiency of the are due to a genetic deficiency of the protein dystrophin.protein dystrophin.


Duchenne muscular dystrophyDuchenne muscular dystrophy is the most is the most severe form of dystrophinopathy. It occurs mostly severe form of dystrophinopathy. It occurs mostly in young boys and is the most common form of MD in young boys and is the most common form of MD that affects children. Signs and symptoms of that affects children. Signs and symptoms of Duchenne MD may include:Duchenne MD may include:

Frequent falls Frequent falls Large calf muscles Large calf muscles Difficulty getting up from a lying or sitting position Difficulty getting up from a lying or sitting position Weakness in lower leg muscles, resulting in Weakness in lower leg muscles, resulting in

difficulty running and jumping difficulty running and jumping Waddling gait Waddling gait Mild mental retardation, in some cases Mild mental retardation, in some cases

Gower's maneuverGower's maneuver


Duchenne usually appear between the ages of 2 and Duchenne usually appear between the ages of 2 and 5. It first affects the muscles of the pelvis, upper arms 5. It first affects the muscles of the pelvis, upper arms and upper legs. By late childhood, most children with and upper legs. By late childhood, most children with this form of muscular dystrophy are unable to walk. this form of muscular dystrophy are unable to walk. Most die by their late teens or early 20s, often from Most die by their late teens or early 20s, often from pneumonia, respiratory muscle weakness or cardiac pneumonia, respiratory muscle weakness or cardiac complications. Some people with Duchenne MD may complications. Some people with Duchenne MD may exhibit curvature of their spine (scoliosis).exhibit curvature of their spine (scoliosis).

Becker's muscular dystrophy is a milder form of Becker's muscular dystrophy is a milder form of dystrophinopathy. It generally affects older boys and dystrophinopathy. It generally affects older boys and young men, and progresses more slowly, usually over young men, and progresses more slowly, usually over several decades. Signs and symptoms of Becker's MD several decades. Signs and symptoms of Becker's MD are similar to those of Duchenne. The onset of the are similar to those of Duchenne. The onset of the signs and symptoms is generally later, and those signs and symptoms is generally later, and those affected by Becker's MD usually are able to walk until affected by Becker's MD usually are able to walk until at least age 15, and often well into adulthood.at least age 15, and often well into adulthood.


Myotonic dystrophyMyotonic dystrophy Also known as Also known as Steinert's diseaseSteinert's disease, this form , this form of muscular dystrophy produces stiffness of of muscular dystrophy produces stiffness of muscles and an inability to relax muscles at muscles and an inability to relax muscles at will (myotonia), as well as the muscle will (myotonia), as well as the muscle weakness of the other forms of muscular weakness of the other forms of muscular dystrophy.dystrophy.

Although this form of MD can affect Although this form of MD can affect children, it often doesn't affect people until children, it often doesn't affect people until adulthood. It can vary greatly in its severity. adulthood. It can vary greatly in its severity. Muscles may feel stiff after using them. Muscles may feel stiff after using them. Progression of this form of MD is slow.Progression of this form of MD is slow.


Besides myotonia, signs and symptoms of adult-onset myotonic Besides myotonia, signs and symptoms of adult-onset myotonic dystrophy may include:dystrophy may include:

Weakening of voluntary muscles that control your arms and legs, Weakening of voluntary muscles that control your arms and legs, usually beginning with the limb muscles farthest from the torso — usually beginning with the limb muscles farthest from the torso — the muscles of the feet, hands, lower legs and forearms. the muscles of the feet, hands, lower legs and forearms.

Weakening of head, neck and face muscles, which may result in the Weakening of head, neck and face muscles, which may result in the face having a hollow, drooped appearance. face having a hollow, drooped appearance.

Weakening of muscles involved in breathing and swallowing. Weakening of muscles involved in breathing and swallowing. Weaker breathing muscles may result in less oxygen intake and Weaker breathing muscles may result in less oxygen intake and fatigue. Weaker swallowing muscles increase the risk of choking. fatigue. Weaker swallowing muscles increase the risk of choking.

Fainting or dizziness, which may indicate that the disease is Fainting or dizziness, which may indicate that the disease is interfering with the conduction of electrical signals that keep the interfering with the conduction of electrical signals that keep the heart rate normal. heart rate normal.

Weakening of muscles of hollow internal organs such as those in the Weakening of muscles of hollow internal organs such as those in the digestive tract and the uterus. Depending on which part of the digestive tract and the uterus. Depending on which part of the digestive tract is affected, you may experience problems with digestive tract is affected, you may experience problems with swallowing as well as constipation and diarrhea. Weakness of the swallowing as well as constipation and diarrhea. Weakness of the uterine walls may cause problems during childbirth. uterine walls may cause problems during childbirth.

Difficulty sleeping well at night and daytime sleepiness, and Difficulty sleeping well at night and daytime sleepiness, and inability to concentrate because of the effect of the disease on the inability to concentrate because of the effect of the disease on the brain. brain.

Frontal balding in men. Frontal balding in men. Clouding of the lenses of the eyes (cataracts). Clouding of the lenses of the eyes (cataracts). Mild diabetes. Mild diabetes.


Rarely, infants have this form of muscular Rarely, infants have this form of muscular dystrophy, in which case it's called dystrophy, in which case it's called congenital myotonic dystrophycongenital myotonic dystrophy. The . The infant form is more severe, although infants infant form is more severe, although infants with myotonic dystrophy don't experience with myotonic dystrophy don't experience myotonia. Signs in infants may include:myotonia. Signs in infants may include:

Severe muscle weakness Severe muscle weakness Difficulty sucking and swallowing Difficulty sucking and swallowing Difficulty breathing Difficulty breathing Cognitive impairment Cognitive impairment


Facioscapulohumeral Facioscapulohumeral DystrophyDystrophyAlso known as Also known as Landouzy-Dejerine diseaseLandouzy-Dejerine disease, this form , this form involves progressive muscle weakness, usually in this involves progressive muscle weakness, usually in this order:order:

Face Face Shoulders Shoulders Abdomen Abdomen Feet Feet Upper arms Upper arms Pelvic area Pelvic area Lower arms Lower arms When someone with facioscapulohumeral MD raises When someone with facioscapulohumeral MD raises

his or her arms, the shoulder blades may stick out like his or her arms, the shoulder blades may stick out like wings. Progression of this form is slow, with some wings. Progression of this form is slow, with some spurts of rapidly increasing weakness. Onset usually spurts of rapidly increasing weakness. Onset usually occurs during the teen to early adult years.occurs during the teen to early adult years.


The other major types of muscular The other major types of muscular dystrophy are rare. They include:dystrophy are rare. They include:

Limb-girdle muscular dystrophy Limb-girdle muscular dystrophy Congenital muscular dystrophy Congenital muscular dystrophy Oculopharyngeal muscular Oculopharyngeal muscular

dystrophy dystrophy Distal muscular dystrophy Distal muscular dystrophy Emery-Dreifuss muscular dystrophy Emery-Dreifuss muscular dystrophy

CausesCauses

1- X-linked recessive inheritance pattern with carrier mother

2- Autosomal dominant inheritance pattern

X-linked recessive inheritance pattern with carrier mother

Duchenne and Becker's muscular Duchenne and Becker's muscular dystrophies are passed from mother to son dystrophies are passed from mother to son through one of the mother's genes in a through one of the mother's genes in a pattern called X-linked recessive pattern called X-linked recessive inheritance. Boys inherit an X chromosome inheritance. Boys inherit an X chromosome from their mothers and a Y chromosome from their mothers and a Y chromosome from their fathers. The X-Y combination from their fathers. The X-Y combination makes them male. Girls inherit two X makes them male. Girls inherit two X chromosomes, one from their mothers and chromosomes, one from their mothers and one from their fathers. The X-X one from their fathers. The X-X combination determines that they are combination determines that they are female.female.

Autosomal dominant inheritance pattern

Patterns differ for other types of MDPatterns differ for other types of MDMyotonic dystrophy is passed along in a pattern Myotonic dystrophy is passed along in a pattern called autosomal dominant inheritance. If either called autosomal dominant inheritance. If either parent carries the defective gene for myotonic parent carries the defective gene for myotonic dystrophy, there's a 50 percent chance the dystrophy, there's a 50 percent chance the disorder will be passed along to a child.disorder will be passed along to a child.

Some of the less common types of muscular Some of the less common types of muscular dystrophy are passed along in the same dystrophy are passed along in the same inheritance pattern that marks Duchenne and inheritance pattern that marks Duchenne and Becker's muscular dystrophies. Other types of Becker's muscular dystrophies. Other types of muscular dystrophy can be passed on from muscular dystrophy can be passed on from generation to generation and affect males and generation to generation and affect males and females equally. Still others require a defective females equally. Still others require a defective gene from both parents.gene from both parents.

Screening and diagnosisScreening and diagnosis Blood tests.Blood tests. Damaged muscles release enzymes such as creatine Damaged muscles release enzymes such as creatine

kinase (CK) into the blood. High blood levels of CK suggest a muscle kinase (CK) into the blood. High blood levels of CK suggest a muscle disease such as muscular dystrophy. disease such as muscular dystrophy.

Electromyography.Electromyography. A thin-needle electrode is inserted through your A thin-needle electrode is inserted through your skin into the muscle to be tested. Electrical activity is measured as skin into the muscle to be tested. Electrical activity is measured as you relax and as you gently tighten the muscle. Changes in the you relax and as you gently tighten the muscle. Changes in the pattern of electrical activity can confirm a muscle disease. The pattern of electrical activity can confirm a muscle disease. The distribution of the disease can be determined by testing different distribution of the disease can be determined by testing different muscles. muscles.

Ultrasonography.Ultrasonography. High-frequency sound waves are used to produce High-frequency sound waves are used to produce precise images of tissues and structures within your body. An precise images of tissues and structures within your body. An ultrasound is a noninvasive way of detecting certain muscle ultrasound is a noninvasive way of detecting certain muscle abnormalities, even in the early stages of the disease. abnormalities, even in the early stages of the disease.

Muscle biopsy.Muscle biopsy. A small piece of muscle is taken for laboratory A small piece of muscle is taken for laboratory analysis. The analysis distinguishes muscular dystrophies from other analysis. The analysis distinguishes muscular dystrophies from other muscle diseases. Special tests can identify dystrophin and other muscle diseases. Special tests can identify dystrophin and other markers associated with specific forms of muscular dystrophy. markers associated with specific forms of muscular dystrophy.

Genetic testing.Genetic testing. Blood samples are examined for mutations in the Blood samples are examined for mutations in the gene that produces dystrophin. Standard tests examine just the gene that produces dystrophin. Standard tests examine just the portions of the dystrophin gene responsible for most cases of portions of the dystrophin gene responsible for most cases of Duchenne and Becker's muscular dystrophies. These tests identify Duchenne and Becker's muscular dystrophies. These tests identify deletions or duplications on the dystrophin gene in about two-thirds deletions or duplications on the dystrophin gene in about two-thirds of people with Duchenne and Becker's MDs. The genetic defects of people with Duchenne and Becker's MDs. The genetic defects responsible for Duchenne and Becker's muscular dystrophies are responsible for Duchenne and Becker's muscular dystrophies are harder to identify in other cases of those affected, but new tests that harder to identify in other cases of those affected, but new tests that examine the entire dystrophin gene are making it possible to pinpoint examine the entire dystrophin gene are making it possible to pinpoint tiny, less common mutations. tiny, less common mutations.

TreatmentTreatment

There's currently no cure for any form of There's currently no cure for any form of muscular dystrophy. Research into gene muscular dystrophy. Research into gene therapy may eventually provide treatment therapy may eventually provide treatment to stop the progression of some types of to stop the progression of some types of muscular dystrophy. Current treatment is muscular dystrophy. Current treatment is designed to help prevent or reduce designed to help prevent or reduce deformities in the joints and the spine and deformities in the joints and the spine and to allow people with MD to remain mobile to allow people with MD to remain mobile as long as possible. Treatments may as long as possible. Treatments may include various types of physical therapy, include various types of physical therapy, medications, assistive devices and surgery.medications, assistive devices and surgery.

Treatment Cont..Treatment Cont..

Physical therapy:Physical therapy:As muscular dystrophy progresses and muscles As muscular dystrophy progresses and muscles weaken, fixations (contractures) can develop in weaken, fixations (contractures) can develop in joints. Tendons can shorten, restricting the joints. Tendons can shorten, restricting the flexibility and mobility of joints. Contractures flexibility and mobility of joints. Contractures are uncomfortable and may affect the joints of are uncomfortable and may affect the joints of your hands, feet, elbows, knees and hips.your hands, feet, elbows, knees and hips.

One goal of physical therapy is to provide One goal of physical therapy is to provide regular range-of-motion exercises to keep your regular range-of-motion exercises to keep your joints as flexible as possible, delaying the joints as flexible as possible, delaying the progression of contractures, and reducing or progression of contractures, and reducing or delaying curvature of your spine. Using hot delaying curvature of your spine. Using hot baths (hydrotherapy) also can help maintain baths (hydrotherapy) also can help maintain range of motion in joints.range of motion in joints.


Medications:Medications: For myotonic dystrophy.For myotonic dystrophy. The medications The medications

mexiletine (Mexitil), phenytoin (Dilantin, mexiletine (Mexitil), phenytoin (Dilantin, Phenytek), carbamazepine (Tegretol, Phenytek), carbamazepine (Tegretol, Carbatrol), quinine and procainamide Carbatrol), quinine and procainamide (Procanbid, Pronestyl) may be used to treat (Procanbid, Pronestyl) may be used to treat the delayed muscle relaxation that occurs in the delayed muscle relaxation that occurs in myotonic dystrophy. myotonic dystrophy.

For Duchenne muscular dystrophy.For Duchenne muscular dystrophy. The The anti-inflammatory corticosteroid medication anti-inflammatory corticosteroid medication prednisone may help improve muscle strength prednisone may help improve muscle strength and delay the progression of Duchenne MD. and delay the progression of Duchenne MD.


Assistive devices:Assistive devices:Braces can both provide support for Braces can both provide support for weakened muscles of your hands and lower weakened muscles of your hands and lower legs and help keep muscles and tendons legs and help keep muscles and tendons stretched and flexible, slowing the stretched and flexible, slowing the progression of contractures. Other devices progression of contractures. Other devices such as canes, walkers and wheelchairs can such as canes, walkers and wheelchairs can help maintain mobility and independence. If help maintain mobility and independence. If respiratory muscles become weakened, respiratory muscles become weakened, using a ventilator may become necessary.using a ventilator may become necessary.


Surgery: Surgery: ( three approaches: ambulatory, rehabilitative, ( three approaches: ambulatory, rehabilitative, palliative )palliative )

Surgical release of contracturesSurgical release of contractures: Cut through : Cut through tendons to relieve contractures (tendon release tendons to relieve contractures (tendon release surgery). surgery).

Spinal fusion for scoliosisSpinal fusion for scoliosis: Scoliosis in a : Scoliosis in a wheelchair-dependent child with MD can wheelchair-dependent child with MD can become so severe it causes breathing problems become so severe it causes breathing problems and pneumonia. Having spine surgery before this and pneumonia. Having spine surgery before this happens can preserve breathing function, lessen happens can preserve breathing function, lessen back pain and improve sitting balance. All these back pain and improve sitting balance. All these factors improve the child's quality of life. The factors improve the child's quality of life. The surgery is recommended when the spinal curve surgery is recommended when the spinal curve reaches a certain size (i.e., more than 20 reaches a certain size (i.e., more than 20 degrees).degrees).

Surgical TreatmentSurgical Treatment

Percutaneous Release of Hip Flexion and Percutaneous Release of Hip Flexion and Abduction Contractures and Tendo Abduction Contractures and Tendo Calcaneus Contracture.Calcaneus Contracture.

Open procedure techniqueOpen procedure technique

TECHNIQUE:TECHNIQUE: With the child supine on the operating table, prepare and With the child supine on the operating table, prepare and

drape both lower extremities from the iliac crests to the toes. drape both lower extremities from the iliac crests to the toes. First flex and then extend the hip to be released, holding the First flex and then extend the hip to be released, holding the hip in adduction to place tension on the muscles to be hip in adduction to place tension on the muscles to be released; keep the opposite hip in maximal flexion to flatten released; keep the opposite hip in maximal flexion to flatten the lumbar spine. Insert a no. 15 knife blade percutaneously the lumbar spine. Insert a no. 15 knife blade percutaneously just medial and just distal to the anterosuperior iliac spine. just medial and just distal to the anterosuperior iliac spine. Release the Release the sartorius musclesartorius muscle first, then the first, then the tensor fasciae tensor fasciae femorisfemoris muscle. Push the knife laterally and subcutaneously— muscle. Push the knife laterally and subcutaneously—without cutting the skin—to release the without cutting the skin—to release the tensor fasciae lataetensor fasciae latae completely. Bring the knife to the original insertion point and completely. Bring the knife to the original insertion point and push it deeper to release the push it deeper to release the rectus femorisrectus femoris completely. Take completely. Take care to avoid the neurovascular structures of the anterior care to avoid the neurovascular structures of the anterior thigh. Next, approximately 3 to 4 cm proximal to the upper thigh. Next, approximately 3 to 4 cm proximal to the upper pole of the patella, percutaneously release the fascia lata pole of the patella, percutaneously release the fascia lata laterally through a stab wound in its midportion. Push the knife laterally through a stab wound in its midportion. Push the knife almost to the femur to release the almost to the femur to release the lateral intermuscular lateral intermuscular septumseptum completely. Now perform a percutaneous release of completely. Now perform a percutaneous release of the the tendo calcaneustendo calcaneus. Apply long leg casts with the feet in . Apply long leg casts with the feet in neutral position and with the heels well padded to prevent neutral position and with the heels well padded to prevent pressure ulcers. pressure ulcers.

Surgical Treatment Surgical Treatment Cont..Cont..

Transfer of Posterior Tibialis Tendon to Transfer of Posterior Tibialis Tendon to Dorsum of Foot Dorsum of Foot (posterior tibialis muscle overpull)(posterior tibialis muscle overpull)

TECHNIQUE:TECHNIQUE: make a 3-cm incision starting medially at the neck of the talus and extending to the make a 3-cm incision starting medially at the neck of the talus and extending to the

navicular. Open the sheath of the posterior tibial tendon from the distal extent of the navicular. Open the sheath of the posterior tibial tendon from the distal extent of the flexor retinaculum to the navicular. Release the tendon from its bony insertions, flexor retinaculum to the navicular. Release the tendon from its bony insertions, preserving as much length as possible. Make a second incision 6 to 8 cm long preserving as much length as possible. Make a second incision 6 to 8 cm long vertically between the tendo calcaneus and the medial distal tibia. The tendo vertically between the tendo calcaneus and the medial distal tibia. The tendo calcaneus can be lengthened through the same incision if necessary. Incise the calcaneus can be lengthened through the same incision if necessary. Incise the posterior tibial tendon sheath and pull the distal portion of the tendon through the posterior tibial tendon sheath and pull the distal portion of the tendon through the second operative wound. Make a third incision 6 cm long lateral to the anterior crest second operative wound. Make a third incision 6 cm long lateral to the anterior crest of the tibia and extend it to the superior extensor retinaculum. Incise the anterior of the tibia and extend it to the superior extensor retinaculum. Incise the anterior compartment fascia and retract the tibialis anterior tendon laterally. Carefully incise compartment fascia and retract the tibialis anterior tendon laterally. Carefully incise the interosseous membrane on the lateral aspect of the tibia adjacent to its tibial the interosseous membrane on the lateral aspect of the tibia adjacent to its tibial insertion for a distance of 3 cm. Enlarge the opening by proximal and distal horizontal insertion for a distance of 3 cm. Enlarge the opening by proximal and distal horizontal cuts, extending halfway across the interosseous membrane. Pass a curved clamp close cuts, extending halfway across the interosseous membrane. Pass a curved clamp close to the tibia from the anterior compartment proximally into the second incision. Keep to the tibia from the anterior compartment proximally into the second incision. Keep the curved clamp on the tibia to prevent injury to the peroneal vessels. After grasping the curved clamp on the tibia to prevent injury to the peroneal vessels. After grasping the posterior tibialis tendon and pulling it into the third incision, inspect the tendon the posterior tibialis tendon and pulling it into the third incision, inspect the tendon through the second incision to make sure that it has neither twisted on itself nor through the second incision to make sure that it has neither twisted on itself nor ensnared the flexor digitorum longus tendon. Make a fourth incision 3 cm long on the ensnared the flexor digitorum longus tendon. Make a fourth incision 3 cm long on the dorsum of the foot in the region of the middle cuneiform. Incise the periosteum of the dorsum of the foot in the region of the middle cuneiform. Incise the periosteum of the middle cuneiform and expose the central portion of the bone. Drill a hole 5 to 8 mm to middle cuneiform and expose the central portion of the bone. Drill a hole 5 to 8 mm to insert the tendon through the middle of the cuneiform. Pass a Kelly clamp insert the tendon through the middle of the cuneiform. Pass a Kelly clamp subcutaneously from the third incision to the fourth incision distally to create a subcutaneously from the third incision to the fourth incision distally to create a subcutaneous tract for the posterior tibialis tendon. Pull the tendon through the subcutaneous tract for the posterior tibialis tendon. Pull the tendon through the subcutaneous tract with a tendon passer. Holding on to the sutures tied to the end of subcutaneous tract with a tendon passer. Holding on to the sutures tied to the end of the posterior tibialis tendon, pass the tendon into the hole in the middle cuneiform the posterior tibialis tendon, pass the tendon into the hole in the middle cuneiform and pass the sutures through the dorsum of the foot with the aid of straight needles. and pass the sutures through the dorsum of the foot with the aid of straight needles. Release the tourniquet. Inspect, irrigate, and close the wounds. After the wounds have Release the tourniquet. Inspect, irrigate, and close the wounds. After the wounds have been closed tie the suture over a felt pad and button on the plantar aspect on the foot been closed tie the suture over a felt pad and button on the plantar aspect on the foot with the foot in a neutral position. Apply a long leg cast with the knee extended and with the foot in a neutral position. Apply a long leg cast with the knee extended and the ankle in neutral position. the ankle in neutral position.


Transfer of Posterior Tibialis Tendon to Transfer of Posterior Tibialis Tendon to Dorsum of Base of Second Metatarsal Dorsum of Base of Second Metatarsal (posterior tibialis muscle overpull)(posterior tibialis muscle overpull)

TECHNIQUE:TECHNIQUE: With the patient supine and a tourniquet in place, make a 3-cm incision over the With the patient supine and a tourniquet in place, make a 3-cm incision over the

insertion of the posterior tibialis tendon on the navicular. Open the sheath of the insertion of the posterior tibialis tendon on the navicular. Open the sheath of the posterior tibialis tendon from the anterior aspect of the medial malleolus to the posterior tibialis tendon from the anterior aspect of the medial malleolus to the navicular. Release the tendon from the bony insertions, preserving as much length navicular. Release the tendon from the bony insertions, preserving as much length as possible. Make a second incision in the posteromedial calf in the region of the as possible. Make a second incision in the posteromedial calf in the region of the myotendinous junction of the posterior tibialis tendon. A gastrocnemius recession myotendinous junction of the posterior tibialis tendon. A gastrocnemius recession can be performed through this incision if necessary, but excessive lengthening of can be performed through this incision if necessary, but excessive lengthening of the triceps surae complex should be avoided to prevent the development of a the triceps surae complex should be avoided to prevent the development of a crouched gait postoperatively. Open the posterior tibial tendon sheath and pull the crouched gait postoperatively. Open the posterior tibial tendon sheath and pull the tendon through the sheath into the calf wound. At the myotendinous junction of the tendon through the sheath into the calf wound. At the myotendinous junction of the tibialis posterior, incise the tendon transversely halfway through its width. Extend tibialis posterior, incise the tendon transversely halfway through its width. Extend this incision distally to within 0.5 cm of the cut insertion of the tibialis tendon. this incision distally to within 0.5 cm of the cut insertion of the tibialis tendon. Secure the distal aspect of the tendon with a single suture to prevent the Secure the distal aspect of the tendon with a single suture to prevent the longitudinal cut from extending out to the end of the tendon. This procedure longitudinal cut from extending out to the end of the tendon. This procedure effectively doubles the length of the posterior tibialis tendon. Make a third incision, effectively doubles the length of the posterior tibialis tendon. Make a third incision, 6 cm long lateral to the anterior crest of the tibia, extending it to the superior 6 cm long lateral to the anterior crest of the tibia, extending it to the superior extensor retinaculum. Perform an anterior compartment fasciotomy and retract the extensor retinaculum. Perform an anterior compartment fasciotomy and retract the tibialis anterior tendon laterally. Incise the interosseous membrane of the lateral tibialis anterior tendon laterally. Incise the interosseous membrane of the lateral aspect of the tibia for a distance of 3 cm. Take a Kelly clamp and place it through aspect of the tibia for a distance of 3 cm. Take a Kelly clamp and place it through the anterior compartment wound across the interosseous membrane and into the the anterior compartment wound across the interosseous membrane and into the deep posterior compartment. Grasp the end of the lengthened posterior tibialis deep posterior compartment. Grasp the end of the lengthened posterior tibialis tendon and bring it through the interosseous membrane into the anterior tendon and bring it through the interosseous membrane into the anterior compartment of the calf . Make another incision, 2 to 3 cm long, over the base of compartment of the calf . Make another incision, 2 to 3 cm long, over the base of the second metatarsal. Dissect down to the base of the second metatarsal and the second metatarsal. Dissect down to the base of the second metatarsal and subperiosteally dissect around the base of the second metatarsal circumferentially. subperiosteally dissect around the base of the second metatarsal circumferentially. Take the elongated tibialis posterior tendon and tunnel it subcutaneously into the Take the elongated tibialis posterior tendon and tunnel it subcutaneously into the incision over the dorsum of the second metatarsal. Loop the tendon around the incision over the dorsum of the second metatarsal. Loop the tendon around the base of the second metatarsal as a sling and tie it to itself with the appropriate base of the second metatarsal as a sling and tie it to itself with the appropriate tension on the ankle to hold it into a neutral plantar flexion and dorsiflexion. tension on the ankle to hold it into a neutral plantar flexion and dorsiflexion. Release the tourniquet and inspect the tibial vessels to make sure that they are not Release the tourniquet and inspect the tibial vessels to make sure that they are not being kinked by the transferred tendon. Irrigate the wounds and close them in a being kinked by the transferred tendon. Irrigate the wounds and close them in a standard fashion. standard fashion.


Lengthening of Tendo Calcaneus(Equinus deformity)


Scapulothoracic Fusion Scapulothoracic Fusion (inability to functionally flex and abduct (inability to functionally flex and abduct

the shoulder )the shoulder )

TECHNIQUE:TECHNIQUE: Place the patient prone and manually position the Place the patient prone and manually position the

scapula at 15 to 20 degrees of external rotation. Make scapula at 15 to 20 degrees of external rotation. Make an oblique 12-inch incision over the medial border of an oblique 12-inch incision over the medial border of the scapula and transect the trapezius muscle. Release the scapula and transect the trapezius muscle. Release the rhomboids and levator scapula from their scapular the rhomboids and levator scapula from their scapular insertions and elevate the supraspinatus, insertions and elevate the supraspinatus, infraspinatus, and subscapularis muscles infraspinatus, and subscapularis muscles subperiosteally for 2 cm from the medial border of the subperiosteally for 2 cm from the medial border of the scapula. Excise a 2-cm strip of subscapularis muscles scapula. Excise a 2-cm strip of subscapularis muscles to allow approximation of the scapula to the thoracic to allow approximation of the scapula to the thoracic wall. Usually ribs 3 through 7 are exposed wall. Usually ribs 3 through 7 are exposed subperiosteally for 2 cm. Pass double 16-gauge Luque subperiosteally for 2 cm. Pass double 16-gauge Luque wires subperiosteally under each rib. Use a sharp wires subperiosteally under each rib. Use a sharp towel clip to make holes 1 cm from the medial ledge of towel clip to make holes 1 cm from the medial ledge of the scapular points corresponding from the wired ribs. the scapular points corresponding from the wired ribs. Use a burr to decorticate the posterior surface of the Use a burr to decorticate the posterior surface of the exposed rib margins in the corresponding points along exposed rib margins in the corresponding points along the anterior surface of the scapula. Place strips of the anterior surface of the scapula. Place strips of cancellous allograft taken from the posterior iliac cancellous allograft taken from the posterior iliac crest between the denuded ribs and scapula. Tighten crest between the denuded ribs and scapula. Tighten the wires sequentiallythe wires sequentially


Other treatmentsOther treatmentsBecause respiratory infections may Because respiratory infections may become a problem in later stages of become a problem in later stages of muscular dystrophy, it's important to muscular dystrophy, it's important to be vaccinated for be vaccinated for pneumoniapneumonia and to and to keep up-to-date with keep up-to-date with influenzainfluenza shots.shots.

THE ENDTHE END

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من • تقديمها و إعدادها تم محاضرات سلسلة من هي المحاضرة هذه , دمشق مشفى في العظمية الجراحة شعبة في المقيمين األطباء قبل

. . ميرعلي بشار د إشراف تحت• . المحاضرة هذه في الواردة األخطاء عن مسؤول غير الموقع

•This lecture is one of a series of lectures were prepared and presented by residents in the department of orthopedics in Damascus hospital, under the supervision of Dr. Bashar Mirali.

•This site is not responsible of any mistake may exist in this lecture.

كاظم. مؤيد Dr. Muayad Kadhimد

Muscular Dystrophy Amjad Moiffak Moreden, M.D. Department of Orthopaedic Surgery The General...

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Transcript of Muscular Dystrophy Amjad Moiffak Moreden, M.D. Department of Orthopaedic Surgery The General...