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The use of orthotic bracing, pioneered by Sydney Haje as of 1977, is finding increasing acceptance as an alternative to surgery in select cases of pectus carinatum. In children, teenagers, and young adults who have pectus carinatum and are motivated to avoid surgery, the use of a customized chest-wall brace that applies direct pressure on the protruding area of the chest produces excellent outcomes. Willingness to wear the brace as required is essential for the success of this treatment approach. The brace works in much the same way as orthodontics (braces that correct the alignment of teeth). The brace consists of front and back compression plates that are anchored to aluminum bars. These bars are bound together by a tightening mechanism which varies from brace to brace. This device is easily hidden under clothing and must be worn from 14 to 24 hours a day. The wearing time varies with each brace manufacturer and the managing physicians protocol, which could be based on the severity of the carinatum malformation (mild moderate severe) and if it is symmetric or asymmetric.
Depending on the manufacturer and/or the patient's preference, the brace may be worn on the skin or it may be worn over a body 'sock' or sleeve called a Bracemate, specifically designed to be worn under braces. A physician or orthotist or brace manufacturer's representative can show how to check to see if the brace is in correct position on the chest.
Bracing is becoming more popular over surgery for pectus carinatum, mostly because it eliminates the risks that accompany surgery. The prescribing of bracing as a treatment for pectus carinatum has 'trickled down' from both paediatric and thoracic surgeons to the family physician and pediatricians again due to its lower risks and well-documented very high success results. The pectus carinatum guideline of 2012 of the American Pediatric Surgical Association has stated: "As reconstructive therapy for the compliant pectus [carinatum] malformation, nonoperative compressive orthotic bracing is usually an appropriate first line of therapy as it does not preclude the operative option. For appropriate candidates, orthotic bracing of chest wall malformations can reasonably be expected to prevent worsening of the malformation and often results in a lasting correction of the malformation. Orthotic bracing is often successful in prepubertal children whose chest wall is compliant. Expert opinion suggests that the noncompliant chest wall malformation or significant asymmetry of the pectus carinatum malformation caused by a concomitant excavatum-type malformation may not respond to orthotic bracing."
Regular supervision during the bracing period is required for optimal results. Adjustments may be needed to the brace as the child grows and the pectus improves.
For patients with severe pectus carinatum, surgery may be necessary. However bracing could and may still be the first line of treatment. Some severe cases treated with bracing may result in just enough improvement that patient is happy with the outcome and may not want surgery afterwards.
If bracing should fail for whatever reason then surgery would be the next step. The two most common procedures are the Ravitch technique and the Reverse Nuss procedure.
A modified Ravitch technique uses bioabsorbable material and postoperative bracing, and in some cases a diced rib cartilage graft technique.
The Nuss was developed by Donald Nuss at the Children's Hospital of the King's Daughters in Norfolk, Va. The Nuss is primarily used for Pectus Excavatum, but has recently been revised for use in some cases of PC, primarily when the malformation is symmetrical.
Surgery is usually only used if the non-surgical treatments have failed. Bone abnormalities may need surgical attention. The most common surgery for snapping scapula requires the surgeon to “take out a small piece of the upper corner of the scapula nearest to the spine.”
Doctors often recommend physical therapy in order to strengthen the subscapularis muscle, and prescribe anti-inflammatory medications. For extreme cases, cortisone injections would be utilized.
The goals of surgical treatment are: reducing length of the thumb, creating a good functioning, a stable and non deviated joint and improving the position of the thumb if necessary. Hereby improving function of the hand and thumb.
In general the surgical treatment is done for improvement of the thumb function. However, an extra advantage of the surgery is the improvement in appearance of the thumb. In the past, surgical treatment of the triphalangeal thumb was not indicated, but now it is generally agreed that operative treatment improves function and appearance. Because an operation was not indicated in the past, there’s still a population with an untreated triphalangeal thumb. The majority of this population doesn’t want surgery, because the daily functioning of the hand is good. The main obstacle for the untreated patients might not be the diminished function, but the appearance of the triphalangeal thumb.
The timing of surgery differs between Wood and Buck-Gramcko. Wood advises operation between the age of six months and two years, while Buck-Gramcko advises to operate for all indications before the age of six years.
- For TPT types I and II of the Buck-Gramcko classification, the surgical treatment typically consists of removing the extra phalanx and reconstructing the ulnar collateral ligament and the radial collateral ligament if necessary.
- For type III of Buck-Gramcko classification proposable surgical treatments:
- For type IV of Buck-Gramcko classification the surgical treatment typically consists of an osteotomy which reduces the middle phalanx and arthrodesis of the DIP. This gives a shortening of 1 to 1.5 cm. In most cases, this technique is combined with a shortening, rotation and palmar abduction osteotomy at metacarpal level to correct for position and length of the thumb. The extensor tendons and the intrinsic muscles are shortened as well.
- For type V of the Buck-Gramcko classification the surgical treatment proposably consists of a "pollicization". With a pollicization the malpositioned thumb is repositioned, rotated and shortened, the above-described rotation reduction osteotomy of the first metacarpal can be performed as well.
- For type VI of the Buck-Gramcko classification, the surgical treatment typically consists of removing the additional mostly hypoplastic thumb(s). Further procedures of reconstruction of the triphalangeal thumb are performed according to the shape of the extra phalanx as described above.
The surgery takes place under general anaesthesia and lasts less than 1 hour. The surgeon prepares the locus to the size of the implant after performing a 8-cm axillary incision and inserts the implant beneath the skin. The closure is made in 2 planes.
The implant will replace the pectoralis major muscle, thus enabling the thorax to be symmetrical and, in women, the breast as well. If necessary, especially in the case of women, a second operation will complement the result by the implantation of a breast implant and / or lipofilling.
Lipomodelling is progressively used in the correction of breast and chest wall deformities. In Poland syndrome, this technique appears to be a major advance that will probably revolutionize the treatment of severe cases. This is mainly due to its ability to achieve previously unachievable quality of reconstruction with minimal scaring.
The complete or partial absence of the pectoralis muscle is the malformation that defines Poland Syndrome. It can be treated by inserting a custom implant designed by CAD (computer aided design). A 3D reconstruction of the patient's chest is performed from a medical scanner to design a virtual implant perfectly adapted to the anatomy of each one. The implant is made of medical silicone unbreakable rubber. This treatment is purely cosmetic and does not make up for the patient's imbalanced upper body strength.
The Poland syndrome malformations being morphological, correction by custom implant is a first-line treatment. This technique allows a wide variety of patients to be treated with good outcomes. Poland Syndrome can be associated with bones, subcutaneous and mammary atrophy: if the first, as for pectus excavatum, is successfully corrected by a custom implant, the others can require surgical intervention such as lipofilling or silicone breast implant, in a second operation.
Because neither of the two thumb components is normal, a decision should be taken on combining which elements to create the best possible composite digit. Instead of amputating the most hypoplastic thumb, preservation of skin, nail, collateral ligaments and tendons is needed to augment the residual thumb. Surgery is recommended in the first year of life, generally between 9 and 15 months of age.
Surgical options depend on type of polydactyly.
This type of procedure is recommended for Wassel types 1 and 2 (in which both thumbs are severely hypoplastic) by some congenital hand surgeons. The technique contains a composite wedge resection of the central bone and soft-tissue. This will be achieved with approach of the lateral tissue of each thumb. The goal is to achieve a normal thumb, what concerns the size, which is possible. If the width of the nail bed is greater than 70% of the contralateral thumb, it may be split. Then the nail bed will be repaired precisely.
Since there is a variety of classifications of winged scapula, there is also more than one type of treatment. Massage Therapy is an effective initial approach to relax the damaged muscles. In more severe cases, Physical Therapy can help by strengthening affected and surrounding muscles. Physical therapy constitutes treatment options if there is weakness of the glenohumeral joint muscles, but if the muscles do not contract clinically and symptoms continue to be severe for more than 3–6 months, surgery may be the next choice. Surgery by fixation of the scapula to the rib cage can be done for those with isolated scapular winging. Some options are neurolysis (chordotomy), intercostal nerve transfer, scapulothoracic fusion, arthrodesis (scapulodesis), or scapulothoracis fixation without arthrodesis (scapulopexy).
There is no specific treatment for rib fractures, but various supportive measures can be taken. In simple rib fractures, pain can lead to reduced movement and cough suppression; this can contribute to formation of secondary chest infection. Flail chest is a potentially life-threatening injury and will often require a period of assisted ventilation. Flail chest and first rib fractures are high-energy injuries and should prompt investigation of damage to underlying viscera (e.g., lung contusion) or remotely (e.g., cervical spine injury). Spontaneous fractures in athletes generally require a cessation of the cause, e.g., time off rowing, while maintaining cardiovascular fitness.
Treatment options for internal fixation/repair of rib fractures include:
- Judet and/or sanchez plates/struts are a metal plate with strips that bend around the rib and then is further secured with sutures.
- Synthes matrixrib fixation system has two options: a precontoured metal plate that uses screws to secure the plate to the rib; and/or an intramedullary splint which is tunneled into the rib and secured with a set screw.
- Anterior locking plates are metal plates that have holes for screws throughout the plate. The plate is positioned over the rib and screwed into the bone at the desired position. The plates may be bent to match the contour of the section.
- U-plates can also be used as they clamp on to the superior aspect of the ribs using locking screws.
Non specific thoracic spine pain is usually treated by one or a combination of the following:
- Exercise/Active and passive physical therapy
- Deep massage or massage therapy
- Ice and/or heat therapy
- Analgesics such as non-steroidal anti-inflammatory drugs
- Joint manipulation, as commonly performed by physical therapists, chiropractors or osteopathic physicians (D.O.).
- If there is a specific tender spot, then trigger point massage or injections can be helpful.
A painful vertebral compression fracture may be treated with pain medication and rest, or with vertebroplasty or kyphoplasty surgery. If the cause is thought to be osteoporosis, oral or intravenous bisphosphonates may be administered to reduce further fracture risk.
Evidence for the treatment of thoracic outlet syndrome as of 2014 is poor.
Surgical approaches have also been used successfully in TOS. Microsurgery can be used approaching the area from above the collar bone (supraclavicular) followed by neurolysis of the brachial plexus, removal of the scalene muscle (scalenectomy), and the release of the underlying (subclavicular) blood vessels. This approach avoids the use of resection, and has been found to be an effective treatment. In cases where the first rib (or a fibrous band extending from the first rib) is compressing a vein, artery, or the nerve bundle, part of the first rib and any compressive fibrous tissue, can be removed in a first rib resection surgical procedure; scalene muscles may also need to be removed (scalenectomy). This allows increased blood flow and the reduction of nerve compression. In some cases there may be a rudimentary rib or a cervical rib that can be causing the compression, which can be removed using the same technique.
Physical therapy is often used before and after the operation to improve recovery time and outcomes. Potential complications include pneumothorax, infection, loss of sensation, motor problems, subclavian vessel damage, and, as in all surgeries, a very small risk of permanent serious injury or death.
Treatment of a laryngeal cleft depends on the length and resulting severity of symptoms. A shallow cleft (Type I) may not require surgical intervention. Symptoms may be able to be managed by thickening the infant's feeds. If symptomatic, Type I clefts can be sutured closed or injected with filler as a temporary fix to determine if obliterating the cleft is beneficial and whether or not a more formal closure is required at a later date. Slightly longer clefts (Type II and short Type III) can be repaired endoscopically. Short type IV clefts extending to within 5 mm below the innominate artery can be repaired through the neck by splitting the trachea vertically in the midline and suturing the back layers of the esophagus and trachea closed. A long, tapered piece of rib graft can be placed between the esophageal and tracheal layers to make them rigid so the patient will not require a tracheotomy after the surgery and to decrease chances of fistula postoperatively. Long Type IV clefts extending further than 5 mm below the innominate artery cannot be reached with a vertical incision in the trachea, and therefore are best repaired through cricotracheal resection. This involves separating the trachea from the cricoid cartilage, leaving the patient intubated through the trachea, suturing each of the esophagus and the back wall of the trachea closed independently, and then reattaching the trachea to the cricoid cartilage. This prevents the need for pulmonary bypass or extracorporeal membrane oxygenation.
Treatment is only required if the occlusion or bite of the person is compromised and causing other dental problems. Multiple long-term clinical problems can arise such as occlusal interferences, aesthetic disturbances, loss of pulp vitality, irritation of tongue during mastication and speech, caries and displacement of the affected tooth. Most people with talon cusp will live their normal lives unless the case is severe and causes a cascade of other dental issues that lead to additional health problems. This dental anomaly would not be considered fatal. Generally talon cusps on lower teeth require no treatment, but talon cusps on upper teeth may interfere with the bite mechanics and may need to be removed or reduced.
Small talon cusps that produce no symptoms or complication for a person can remain untreated. However large talon cusps should not.
Some common treatments include:
- Fissure sealing
- Composite resin restoration
- Reduction of cusp
- Pulpotomy
- Root canal (endodontic treatment)
- Extraction
The condition is usually benign, but it can cause mild irritation to soft tissues around the teeth and the tongue, and if large enough, may pose an aesthetic problem. Talon cusps that are too large are filed down with a motorized file, and then endodontic therapy is administered.
In order to prevent any future dental complications, when talon cusp is present due to an early diagnosis it would be best to see a dentist regularly every six months for routine dental checkups, remain under observation, brush and floss properly and undergo regular topical applications of fluoride gel to prevent caries and to promote enamel strength.
X-rays of the chest are taken in people with chest trauma and symptoms of sternal fractures, and these may be followed by CT scanning. Since X-rays taken from the front may miss the injury, they are taken from the side as well.
Management involves treating associated injuries; people with sternal fractures but no other injuries do not need to be hospitalized. However, because it is common for cardiac injuries to accompany sternal fracture, heart function is monitored with electrocardiogram. Fractures that are very painful or extremely out of place can be operated on to fix the bone fragments into place, but in most cases treatment consists mainly of reducing pain and limiting movement. The fracture may interfere with breathing, requiring tracheal intubation and mechanical ventilation.
Patients who have experienced a pathologic fracture will be investigated for the cause of the underlying disease, if it is unknown. Treatment of any underlying disease, such as chemotherapy if indicated for bone cancer, may help to improve the pain of a sternal fracture.
In order to begin a rehabilitation program for a flail chest it is important to treat the person's pain so they are able to perform the proper exercises. Due the underlying conditions that the flail segment has caused onto the respiratory system, chest physiotherapy is important to reduce further complications. Proper positioning of the body is key, including postural alignment for proper drainage of mucous secretions. The therapy will consist of a variety of postural positioning and changes in order to increase normal breathing. Along with postural repositioning, a variety of breathing exercises are also very important in order to allow the chest wall to reposition itself back to normal conditions. Breathing exercises will also include coughing procedures. Furthermore, range of motion exercises are given to reduce the atrophy of the musculature. With progression, resistance exercises are added to the regimen to the shoulder and arm of the side containing the injury. Moreover, trunk exercises will be introduced while sitting and will progress to during standing.
Hip flexion exercises can be done to expand the thorax. This is done by lying supine on a flat surface, flexing the knees and hips and bringing them in toward the chest. The knees should come in toward the chest while the person inhales, and exhale when the knees are lowered. This exercise can be done in 3 sets of 6-8 repetitions with a pause in between sets. The person should always make sure to maintain controlled breaths.
Eventually, the person will be progressed to walking and posture correction while walking. Before, the person is discharged from the hospital the person should be able to perform mobility exercises to the core and should have attained good posture.
Treatment of the flail chest initially follows the principles of advanced trauma life support. Further treatment includes:
- Good pain management includes intercostal blocks and avoiding opioid pain medication as much as possible. This allows much better ventilation, with improved tidal volume, and increased blood oxygenation.
- Positive pressure ventilation, meticulously adjusting the ventilator settings to avoid pulmonary barotrauma.
- Chest tubes as required.
- Adjustment of position to make the person most comfortable and provide relief of pain.
- Aggressive pulmonary toilet
Surgical fixation can help in significantly reducing the duration of ventilatory support and in conserving the pulmonary function.
A person may be intubated with a double lumen tracheal tube. In a double lumen endotracheal tube, each lumen may be connected to a different ventilator. Usually one side of the chest is affected more than the other, so each lung may require drastically different pressures and flows to adequately ventilate.
Costochondritis may be treated with physical therapy (including ultrasonic, TENS, with or without nerve stimulation) or with medication. Treatment may involve the use of nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen or other pain relief medications (analgesics) such as acetaminophen. Severe cases of costochondritis may call for the use of opioid medications such as hydrocodone or oxycodone, tricyclic antidepressant medications such as amitriptyline for pain from chronic costochondritis, or anti-epileptic drugs such as gabapentin may be used. Oral or injected corticosteroids may be used for cases of costochondritis unresponsive to treatment by NSAIDs; however, this treatment has not been the subject of study by rigorous randomized controlled trials and its practice is currently based on clinical experience. Rest from stressful physical activity is often advised during the recovery period.
Manipulative physiotherapy, therapeutic exercises and chiropractic manipulative therapy shows beneficial results for decreasing pain and increasing spinal range of motion. As areas of the spine and tendons can become inflamed NSAIDs such as ibuprofen and Naproxen can be helpful in both relieving pain and inflammation associated with DISH. It is hoped that by minimizing inflammation in these areas, further calcification of tendons and ligaments of the spine leading to bony outgrowths (enthesophytes) will be prevented, although causative factors are still unknown.
There is no cure or approved treatment for FOP. Attempts to surgically remove the bone result in explosive bone growth. While under anesthesia, people with FOP may encounter difficulties with intubation, restrictive pulmonary disease, and changes in the electrical conduction system of the heart. Activities that increase the risk of falling or soft tissue injury should be avoided, as even minor trauma may provoke heterotopic bone formation.
The procedure is performed in general anesthesia. It is useful to place pulse oximeter probes on "both hands" and "one foot" so that test occlusion of one arch or its branches will allow confirmation of the anatomy. In addition blood pressure cuffs should also be placed on one leg and both arms to confirm the absence of a pressure gradient when the intended point of division of the lesser arch is temporarily occluded with forceps.
A bifid rib (bifurcated rib or sternum bifidum) is a congenital abnormality of the rib cage and associated muscles and nerves which occurs in about 1.2% of humans. Bifid ribs occur in up to 8.4% of Samoans. The sternal end of the rib is cleaved into two. It is usually unilateral.
Bifid ribs are usually asymptomatic, and are often discovered incidentally by chest X-ray. Effects of this neuroskeletal anomaly can include respiratory difficulties, neurological difficulties, limitations, and limited energy from the stress of needing to compensate for the neurophysiological difficulties. Another association is with odontogenic keratocysts (OKC [a.k.a keratocystic odontogenic tumor (WHO terminology)]) of the jaw which may behave aggressively and have a high propensity to recur when treated with simple enucleation and curettage. When seen together, the patient is likely to have Nevoid Basal Cell Carcinoma Syndrome (a.k.a. Gorlin-Goltz syndrome) and should be evaluated with this in mind.
Surgical correction is indicated in all double aortic arch patients with obstructive symptoms (stridor, wheezing, pulmonary infections, poor feeding with choking). If symptoms are absent a conservative approach (watchful waiting) can be reasonable. Children with very mild symptoms may outgrow their symptoms but need regular follow-up.