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It is sometimes possible to correct the problem with surgery, though this has high failure rates for treatment of post-traumatic radioulnar synostosis.
Treatment for Jackson–Weiss syndrome can be done through surgery for some facial features and feet. Secondary complications such as hydrocephalus or cognitive impairment, can be averted via prompt surgery.
There is no standard treatment for the hand malformations in Apert due to the differences and severity in clinical manifestations in different patients. Every patient should therefore be individually approached and treated, aiming at an adequate balance between hand functionality and aesthetics.
However, some guidelines can be given depending on the severity of the deformities.
In general it is initially recommended to release the first and fourth interdigital spaces, thus releasing the border rays.
This makes it possible for the child to grasp things by hand, a very important function for the child's development. Later the second and third interdigital spaces have to be released.
Because there are three handtypes in Apert, all with their own deformities, they all need a different approach regarding their treatment:
- Type I hand usually needs only the interdigital web space release. First web release is rarely needed but often its deepening is necessary. Thumb clynodactyly correction will be needed.
- In type II hands it is recommended to release the first and fifth rays in the beginning, then the second and the third interdigital web spaces have to be freed. The clynodactyly of the thumb has to be corrected as well. The lengthening of the thumb phalanx may be needed, thus increasing the first web space. In both type I and type II, the recurrent syndactyly of the second web space will occur because of a pseudoepiphysis at the base of the index metacarpal. This should be corrected by later revisions.
- Type III hands are the most challenging to treat because of their complexity. First of all, it is advised to release the first and fourth webspace, thus converting it to type I hand. The treatment of macerations and nail-bed infections should also be done in the beginning. For increasing of the first web space, lengthening of the thumb can be done. It is suggested that in severe cases an amputation of the index finger should be considered. However, before making this decision, it is important to weigh the potential improvement to be achieved against the possible psychological problems of the child later due to the aesthetics of the hand. Later, the second and/or third interdigital web space should be released.
With growing of a child and respectively the hands, secondary revisions are needed to treat the contractures and to improve the aesthetics.
Each child is different and it entirely depends on which sutures are fused and how it is affecting the child as to how it is treated. Some children have severe breathing issues due to shallow mid face and may require a tracheostomy. All should be treated at a specialist centre. Cranio bands are not used in the UK.
Surgery is typically used to prevent the closure of sutures of the skull from damaging the brain's development. Without surgery, blindness and mental retardation are typical outcomes. Craniofacial surgery is a discipline of both plastic surgery and oral and maxillofacial surgery (OMFS) . To move the orbits forward, craniofacial surgeons expose the skull and orbits and reshape the bone. To treat the midface deficiency, craniofacial surgeons can move the lower orbit and midface bones forward. For jaw surgery, either plastic surgeons or OMFS surgeons can perform these operations.
Crouzon patients tend to have multiple sutures involved, most specifically bilateral coronal craniosynostoses, and either open vault surgery or strip craniectomy (if child is under 6 months) can be performed. In the later scenario, a helmet is worn for several months following surgery.
Once treated for the cranial vault symptoms, Crouzon patients generally go on to live a normal lifespan.
Treatment for Klippel–Feil syndrome is symptomatic and may include surgery to relieve cervical or craniocervical instability and constriction of the spinal cord, and to correct scoliosis.
Failing non-surgical therapies, spinal surgery may provide relief. Adjacent segment disease and scoliosis are two examples of common symptoms associated with Klippel–Feil syndrome, and they may be treated surgically. The three categories treated for types of spinal cord deficiencies are massive fusion of the cervical spine (Type I), the fusion of 1 or 2 vertebrae (Type II), and the presence of thoracic and lumbar spine anomalies in association with type I or type II Klippel–Feil syndrome (Type III).
Adjacent segment disease can be addressed by performing cervical disc arthroplasty using a device such as the Bryan cervical disc prosthesis.
The option of the surgery is to maintain range of motion and attenuate the rate of adjacent segment disease advancement without fusion.
Another type of arthroplasty that is becoming an alternate choice to spinal fusion is Total Disc Replacement. Total disc replacement objective is to reduce pain or eradicate it.
Spinal fusion is commonly used to correct spinal deformities such as scoliosis. Arthrodesis is the last resort in pain relieving procedures, usually when arthroplasties fail.
To treat the trigonocephaly, expanding the distance between orbits using springs seems to work. It allows enough space for the brain to grow and it creates a normal horizontal axis of the orbits and supraorbital bar. The endoscopic surgery started to become popular since the early 90's, but it has some technical limitations (only strip cranictomy is possible). There have been few attempts to go beyond the limits.
Aesthetic outcomes of metopic surgery have been good. Surgery does not have a perfect outcome because there will most likely be minor irregularities. Sometimes reoperations are needed for the severe cases. Trying to hollow out the temporal, and the hypoterlorism are very hard to correct. The hypotelorism usually stays not corrected and in order to correct the temporal hollowing, a second operation is most likely needed.
Galeazzi fractures are best treated with open reduction of the radius and the distal radio-ulnar joint. It has been called the "fracture of necessity," because it necessitates open surgical treatment in the adult. Nonsurgical treatment results in persistent or recurrent dislocations of the distal ulna. However, in skeletally immature patients such as children, the fracture is typically treated with closed reduction.
While there is no cure for BGS, symptoms can be treated as they arise. Surgery shortly after birth can repair craniosynostosis, as well as defects in the hand to create a functional grasp. There are risks associated with untreated craniosynostosis, therefore surgery is often needed to separate and reshape the bones. Since patients with a RECQL4 mutation may be at an increased risk of developing cancer, surveillance is recommended.
Non surgical treatments include steroid injections in the lower back or radiofrequency sensory ablation. Physical therapy interventions are also helpful in early cases and are focused around mobilization, neural stretching, and core strengthening exercises. Surgical intervention is usually a last resort if all conservative methods fail. It can be treated surgically with posterolateral fusion or resection of the transitional articulation.
The key problem is the early fusion of the skull, which can be corrected by a series of surgical procedures, often within the first three months after birth. Later surgeries are necessary to correct respiratory and facial deformities.
The use of surgery to treat a Jefferson fracture is somewhat controversial. Non-surgical treatment varies depending on if the fracture is stable or unstable, defined by an intact or broken transverse ligament and degree of fracture of the anterior arch. An intact ligament requires the use of a soft or hard collar, while a ruptured ligament may require traction, a halo or surgery. The use of rigid halos can lead to intracranial infections and are often uncomfortable for individuals wearing them, and may be replaced with a more flexible alternative depending on the stability of the injured bones, but treatment of a stable injury with a halo collar can result in a full recovery. Surgical treatment of a Jefferson fracture involves fusion or fixation of the first three cervical vertebrae; fusion may occur immediately, or later during treatment in cases where non-surgical interventions are unsuccessful. A primary factor in deciding between surgical and non-surgical intervention is the degree of stability as well as the presence of damage to other cervical vertebrae.
Though a serious injury, the long-term consequences of a Jefferson's fracture are uncertain and may not impact longevity or abilities, even if untreated. Conservative treatment with an immobilization device can produce excellent long-term recovery.
Surgery is needed to prevent the closing of the coronal sutures from damaging brain development. In particular, surgeries for the LeFort III or monobloc midface distraction osteogenesis which detaches the midface or the entire upper face, respectively, from the rest of the skull, are performed in order to reposition them in the correct plane. These surgeries are performed by both plastic and oral and maxillofacial (OMS) surgeons, often in collaboration.
Scheuermann's disease is self-limiting after growth is complete, meaning that it generally runs its course and never presents further complication. Typically, however, once the patient is fully grown, the bones will maintain the deformity. For this reason, there are many treatment methods and options available that aim to correct the kyphosis while the spine is still growing, and especially aim to prevent it from worsening.
While there is no explanation for what causes Scheuermann's Disease, there are ways to treat it. For decades there has been a lot of controversy surrounding treatment options. For less extreme cases, manual medicine, physical therapy and/or back braces can help reverse or stop the kyphosis before it does become severe. Because the disease is often benign, and because back surgery includes many risks, surgery is usually considered a last resort for patients. In severe or extreme cases, patients may be treated through an extensive surgical procedure in an effort to prevent the disease from worsening or harming the body.
In Germany, a standard treatment for both Scheuermann's disease and lumbar kyphosis is the Schroth method, a system of specialized physical therapy for scoliosis and related spinal deformities. The method has been shown to reduce pain and decrease kyphotic angle significantly during an inpatient treatment program.
The heterogeneity of the Klippel–Feil syndrome has made it difficult to outline the diagnosis as well as the prognosis classes for this disease. Because of this, it has complicated the exact explanation of the genetic cause of the syndrome.
The prognosis for most individuals with KFS is good if the disorder is treated early on and appropriately. Activities that can injure the neck should be avoided, as it may contribute to further damage. Other diseases associated with the syndrome can be fatal if not treated, or if found too late to be treatable.
Treatment options for distal radius fractures include non-operative management, external fixation, and internal fixation. Indications for each depend on a variety of factors such as patient's age, initial fracture displacement, and metaphyseal and articular alignment, with the ultimate goal to maximize strength, and function in the affected upper extremity. Surgeons use these factors combined with radiologic imaging to predict fracture instability, and functional outcome in order to help decide which approach would be most appropriate. Treatment is often directed to restore normal anatomy to avoid the possibility of malunion, which may cause decreased strength in the hand and wrist. The decision to pursue a specific type of management varies greatly by geography, physician specialty (hand surgeons vs. orthopedic surgeons), and advancements in new technology such as the volar locking plating system.
Scheuermann's disease can be successfully corrected with surgical procedures, almost all of which include spinal fusion and hardware instrumentation, i.e., rods, pedicle screws, etc. While many patients are typically interested in getting surgery for their correction, it is important to realize the surgery aims to reduce pain, and not cosmetic defect. As always, surgical intervention should be used as a last resort once conservative treatment fails or the patient's health is in imminent danger as any surgical procedure is not without risk; however, the chances of complication are relatively low and the surgeries are often successful.
One of the largest debates surrounding Scheuermann's disease correction is the use of very different correction procedures. There are different techniques to correct kyphosis; usually the differences being posterior/anterior entry or posterior entry (rear) only. The classic surgical procedure partially entails entering two titanium rods, each roughly one and a half feet long (depending on the size of the khyphosis), into the back on either side of the spine. Eight titanium screws and hardware are drilled through the bone and secure the rods onto either side of the spine. On the internal-facing side of the spine, ligaments (which can be too short, pulling the spine into the general shape of kyphosis) must be surgically cut or released, not only stopping part of the cause of the kyphosis, but also allowing the titanium rods to pull the spine into a more natural position. Normally, the damaged discs between the troubled vertebrae (wedged vertebrae) are removed and replaced with bone grafting from the hip or other parts of the vertebrae, which once healed or 'fused' will solidify. The titanium instrumentation holds everything in place during healing and is not necessary once fusion completes. Recovery begins in the hospital and depending on whether the operation is one- or two-stage the patient can expect to be in hospital for minimum of a week, possibly longer depending on recovery.
They will then often be required to wear a brace for several months to ensure the spine heals correctly leaving the patient with the correct posture. The titanium instrumentation can stay in the body permanently, or be removed years later. Patients undergoing surgery for Scheuermann's disease often need physical therapy to manage pain and mobility, however their range of motion is generally not limited very much. Recovery from kyphosis correction surgery can be very long; typically patients are not allowed to lift anything above 5 or 10 pounds for 6 months to a year. Many are out of work for at least 6 months. However, once the fusion is solidified, most patients can return to their usual lifestyle within one to two years.
The majority of distal radius fractures are treated with conservative non-operative management, which involves immobilization through application of plaster or splint with or without closed reduction. The prevalence of non-operative approach to distal radius fractures is approximately 70%. Non-operative management is indicated for fractures that are undisplaced, or for displaced fractures that are stable following reduction. There are variations in immobilization techniques involving the type of cast, position of immobilization, and the length of time required in the cast.
The length of time in the cast varies with different ages. Children heal more rapidly, but may ignore activity restrictions. Three weeks in a cast and 6 weeks off sports may be appropriate for certain fractures. In adults, the risk of stiffness of the joint increases the longer it is immobilized. If callus is seen on x-ray at 4 weeks, the cast may be replaced by a removable splint. However, many hand surgeons leave the patients in the cast for up to 6 weeks. In general, the x-rays will not show any callus until about a month after the fracture is healed; therefore the cast is removed before the x-rays confirm that it is healed.
During the period of follow-up, it is common practice to repeat x-rays at about 1 week to make sure the position is still acceptable. Further followup is needed to determine when the fracture has healed and when rehabilitation is complete. The critical time during the period of attempted treatment with casting is the first 3 weeks. The swelling will reduce during this time and the fracture can displace. If the displacement becomes unacceptable, closed treatment may need to be abandoned and surgery pursued. More than 3 weeks after injury, the fracture will start to heal and further displacement becomes less likely.
Post-traumatic cases are most likely to develop following surgery for a forearm fracture, this is more common with high-energy injuries where the bones are broken into many pieces (comminuted). It can also develop following soft tissue injury to the forearm where there is haematoma formation.
Preiser disease, or (idiopathic) avascular necrosis of the scaphoid, is a rare condition where ischemia and necrosis of the scaphoid bone occurs without previous fracture. It is thought to be caused by repetitive microtrauma or side effects of drugs (e.g., steroids or chemotherapy) in conjunction with existing defective vascular supply to the proximal pole of the scaphoid. MRI coupled with CT and X-ray are the methods of choice for diagnosis.
Preiser's disease is initially treated by immobilising the wrist with a cast. However, in most cases the avascular scaphoid will start to collapse leading to degeneration within the wrist joints. This often requires surgical intervention to prevent the progression of arthris. Two commonly performed procedures are:
1. Proximal row carpectomy (PRC), which involves removing the first row of the carpal bones, i.e. the scaphoid, lunate and triquetrum. The wrist is immobilised in a cast for six weeks after the surgery and then physiotherapy is started.
2. Scaphoid excision and 4-corner fusion, which is a procedure consisting of the removal of the scaphoid and fixation of the remaining wrist bones with a plate (called a "spider plate") or wires in order to provide stability. The plate usually is left inside the patient's wrist, while the wires (usually K-wires) have to be removed in a second surgery. This procedure of partial wrist fusion allows for limited wrist movement, whereas total wrist fusion immobilizes the wrist permanently. Following surgery it can take several months for affected patients to regain strength.
Unfortunately both of these operations are salvage procedures and movements in the wrist will be significantly reduced.
Synostosis (plural: synostoses) is fusion of two bones. It can be normal in puberty, fusion of the epiphysis, or abnormal. When synostosis is abnormal it is a type of dysostosis.
Examples of synostoses include:
- craniosynostosis – an abnormal fusion of two or more cranial bones;
- radioulnar synostosis – the abnormal fusion of the radius and ulna bones of the forearm;
- tarsal coalition – a failure to separately form all seven bones of the tarsus (the hind part of the foot) resulting in an amalgamation of two bones; and
- syndactyly – the abnormal fusion of neighboring digits.
Synostosis within joints can cause ankylosis.
Children with Pfeiffer syndrome types 2 and 3 "have a higher risk for neurodevelopmental disorders and a reduced life expectancy" than children with Pfeiffer syndrome type 1, but if treated, favorable outcomes are possible. In severe cases, respiratory and neurological complications often lead to early death.
Radioulnar synostosis is one of the more common failures of separation of parts of the upper limb. There are two general types: one is characterized by fusion of the radius and ulna at their proximal borders and the other is fused distal to the proximal radial epiphysis. Most cases are sporadic, congenital (due to a defect in longitudinal segmentation at the 7th week of development) and less often post-traumatic, bilateral in 60%, and more common in males. Familial cases in association with autosomal dominant transmission appear to be concentrated in certain geographic regions, such as Sicily.
The condition frequently is not noted until late childhood, as function may be normal, especially in unilateral cases. Increased wrist motion may compensate for the absent forearm motion. It has been suggested that individuals whose forearms are fixed in greater amounts of pronation (over 60 degrees) face more problems with function than those with around 20 degrees of fixation. Pain is generally not a problem, unless radial head dislocation should occur.
Most examples of radioulnar synostosis are isolated (non-syndromic). Syndromes that may be accompanied by radioulnar synostosis include X chromosome polyploidy (e.g., XXXY) and other chromosome disorders (e.g., 4p- syndrome, Williams syndrome), acrofacial dysostosis, Antley–Bixler syndrome, genitopatellar syndrome, Greig cephalopolysyndactyly syndrome, hereditary multiple osteochondromas (hereditary multiple exostoses), limb-body wall complex, and Nievergelt syndrome.
Craniosynostosis (from cranio, cranium; + syn, together; + ostosis relating to bone) is a condition in which one or more of the fibrous sutures in an infant skull prematurely fuses by turning into bone (ossification). Craniosynostosis has following kinds: scaphocephaly, trigonocephaly, plagiocephaly, anterior plagiocephaly, posterior plagiocephaly, brachycephaly, oxycephaly, pansynostosis.
The DDx for this condition includes metopic synostosis, as well as Lambdoida synostosis.
Oxycephaly is a type of cephalic disorder where the top of the skull is pointed or conical due to premature closure of the coronal suture plus any other suture, like the lambdoid, or it may be used to describe the premature fusion of all sutures. It should be differentiated from Crouzon syndrome. Oxycephaly is the most severe of the craniosynostoses.
Treatment is usually conservative in nature. Patient education on lifestyle modifications, chiropractic, nonsteroidal anti-inflammatory drugs (NSAIDs), physical therapy, and osteopathic care are common forms of manual care that help manage such conditions. Other alternative therapies such as massage, trigger-point therapy, yoga and acupuncture may be of limited benefit. Surgery is occasionally performed.
Many of the treatments for cervical spondylosis have not been subjected to rigorous, controlled trials. Surgery is advocated for cervical radiculopathy in patients who have intractable pain, progressive symptoms, or weakness that fails to improve with conservative therapy. Surgical indications for cervical spondylosis with myelopathy (CSM) remain somewhat controversial, but "most clinicians recommend operative therapy over conservative therapy for moderate-to-severe myelopathy" (Baron, M.E.).
Physical therapy may be effective for restoring range of motion, flexibility and core strengthening. Decompressive therapies (i.e. manual mobilization, mechanical traction) may also help alleviate pain. However, physical therapy and osteopathy cannot "cure" the degeneration, and some people view that strong compliance with postural modification is necessary to realize maximum benefit from decompression, adjustments and flexibility rehabilitation.
It has been argued, however, that the cause of spondylosis is simply old age, and that posture modification treatment is often practiced by those who have a financial interest (such as Worker's Compensation) in proving that it is caused by work conditions and poor physical habits. Understanding anatomy is the key to conservative management of spondylosis.