On the basis of the seventh department of the National Medical Research Center for Pediatric Traumatology and Orthopedics named after. G.I. Turner conducts a comprehensive examination and treatment of children with congenital radioulnar synostosis. This area is actively developing thanks to the scientific and clinical activities of our specialists. Therapeutic tactics are determined for each patient individually based on the severity of pronation deformity and the degree of limitation of daily activity.
What is congenital radioulnar synostosis?
Congenital radioulnar or radioulnar synostosis is a developmental anomaly of the upper limb, characterized by the presence of a bone (less often fibrous) “bridge” between the radial and ulnar bones of the forearm in the upper third. This pathology is manifested by a sharp limitation of rotational movements of the forearm, which leads to impaired self-care. To code this disease according to ICD-10, codes Q74.0 are used - anomalies as such, Q68.8 - to code the actual pronation deformity of the forearm against the background of this developmental anomaly.
Diagnostics
To make an accurate diagnosis, an x-ray of the bones of the damaged area is used, in the number of projections recommended by the attending physician.
Control images during the recovery process show dynamics and allow you to evaluate the effectiveness of treatment measures. Sometimes computed tomography of the head, brain and MRI of the head are indicated. If a malfunction of the cardiovascular system is suspected, cardiac ultrasound (echocardiography) is used. It is impossible to diagnose on your own. It is necessary to make an appointment with a traumatologist, if increased intracranial pressure, spinal cord damage, neurological disorders, intracerebral circulation or cardiovascular disorders are diagnosed, you should seek advice from a surgeon, neurologist, cardiologist, orthopedist or ophthalmologist.
Causes
This condition is a consequence of disruption of the normal process of separation of the bones of the forearm from a common cartilaginous model in the early stages of embryonic development. At the moment, the exact cause remains unknown, but a frequent combination with other developmental anomalies, genetic syndromes, and chromosomal abnormalities has been noted. Interestingly, according to archaeological excavations, cases of congenital radioulnar synostosis have been encountered since ancient times all over the world - up to 1000-500 BC.
Prevention
There are no measures to prevent the congenital form of this disease. The risk of its development will decrease if the pregnancy takes place under normal conditions, and the influence of negative factors on the body of the expectant mother will be excluded.
Measures to prevent the development of acquired synostosis of the elbow joint are:
- avoiding joint injury, using personal protective equipment (elbow pads);
- prevention of the occurrence of pathologies (primarily endocrine and metabolic in nature) that can lead to the development of excess bone tissue in the area of the elbow joint, and if they have already arisen, their timely diagnosis and adequate treatment;
- optimization of the load on the elbow joint.
Kinds
In approximately half of the cases, this anomaly affects both limbs, but unilateral variants also occur. Boys and girls are affected equally often. According to our Center, the frequency of radioulnar synostosis among boys is somewhat predominant, with type III according to the Cleary-Omer classification occurring in more than 50% of cases.
Currently, the most common x-ray anatomical classification is according to Cleary-Omer:
• Type I: fibrous synostosis without bone changes, but with shortening of the forearm and limited rotational movements; • Type II: bony synostosis, the head of the radius is formed correctly, there are no imbalances in the brachioradial joint; • Type III: bony synostosis, the radial head is hypoplastic and displaced posteriorly; • Type IV: bony synostosis, the radial head is displaced anteriorly.
Prognosis, disability with synostosis of the elbow joint
The prognosis for synostosis of the elbow joint is favorable. The operation will radically relieve the patient from bone fusions of the joint and resume his motor activity. But cases of relapse are not uncommon.
Kovtonyuk Oksana Vladimirovna, medical observer, surgeon, consultant doctor
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How to treat? Modern methods of treatment and surgery
In cases with slight pronation deformity without a pronounced limitation of daily activity, only dynamic monitoring of the patient is required. However, in more severe cases, the child may require surgery to correct the overpronation of the forearm to improve mobility. In the global medical community, the generally accepted standard is the correction of pronation deformity by moving the forearm to the middle position after osteotomy of one or both bones of the forearm. Our department provides surgical treatment in accordance with current global trends, taking into account individual limitations in the rotational movements of the forearm and the degree of dysfunction of the upper limbs. In the most complex cases, preoperative 3D modeling is used.
Trigonocephaly (metopic synostosis)
Trigonocephaly is premature fusion of the metopic suture. Metopic suture is a suture connecting the 2 halves of the frontal bone. Normally, this suture closes between the ages of 8 months and 2 years. Premature fusion of the suture causes deformation of the cranial vault and the more harmful area of the face. It is important to distinguish between the two conditions that appear with metopic craniosynostosis: trigonocephaly and asymptomatic craniosynostosis (“metopic ridge” or “mild metopic”). In the first case, closure of the metopic suture occurs in utero or at 1-2 months of life. In this case, a classic deformation of the skull is formed like trigonocephaly, hypotelorism (reduced distance between the orbits). At the site of the metopic suture, a bone ridge can be identified by palpation; the large fontanel closes by 1-2 months. Since the growth of the frontal bone largely depends on the metopic suture, its premature closure leads to hypoplasia of the frontal bone and upper jaw, which visually looks like a “small” face.
Caption: Sematic representation of trigonocephaly
In the second case, the closure of the metopic suture occurs later - at 3-4 months, which causes the appearance of a bone crest at the site of the metopic suture, moderate deformation of the frontal bone without the development of craniocerebral disproportion. In any of these cases, consultation with a neurosurgeon is necessary.
Signature: Mild metopic without signs of craniocerebral disproportion.
Metopic craniosynostosis occurs with a frequency of 1:8000 newborns and accounts for about 25% of the total number of craniosynostosis. In the last few years, there has been an increase in the incidence of metopic craniosynostosis.
The clinical picture of metopic craniosynostosis is dominated by hypertension syndrome, visual disturbances and mental abnormalities. According to large international studies, a decrease in cognitive functions in metopic craniosynostosis occurs in 50-60% of cases, while various visual impairments occur in 70% of cases.
In most cases, the diagnosis of metopic craniosynostosis can be established during a routine examination of the child by a neonatologist, pediatrician or neurologist: upon palpation of the metopic suture area, a bone ridge is noted throughout, more pronounced at the base of the skull, a “closed” or reduced and deformed large fontanel.
The gold standard in the differential diagnosis of metopic craniosynostosis is computed tomography. It is this diagnostic method that can reveal signs of craniocerebral disproportion and compression of the frontal lobes of the brain.
Caption: SCT with 3D reconstruction of metopic craniosynostosis
Metopic craniosynostosis with the development of trigonocephaly is a direct indication for surgical treatment. The method of choice for metopic craniosynostosis is fronto-orbital reconstruction. Until recently, minimally invasive techniques were used in the treatment of metopic craniosynostosis, but according to recent data they are ineffective.
Caption: Metopic craniosynostosis after minimally invasive correction and repeated reconstructive surgery
Fronto-orbital reconstruction involves remodeling the frontal bone and brow bone, giving them the correct shape and fixing them with plates. The optimal age for surgical intervention is 5-8 months. At an earlier age, surgical intervention is performed only with the early development of craniostenosis.
The recovery period after surgery is about 10 days. At this time, the patient is bandaged and conservative therapy is performed. For the first 3-4 days after surgery, the child continues to have orbital swelling, which does not require specific treatment.
For more detailed consultation, you can refer to the “Contacts” section
Symptoms
The symptoms of synostosis depend on the location of the pathological process. For the ulnar variant of development, the problems remain typical:
- Limitation of function of the affected limb. The patient cannot perform helical movements. He has difficulty getting dressed, brushing his teeth, holding a tray, or writing with a pen.
- Atrophy of the hand and forearm.
Craniostenosis has a more varied clinical picture. Against the background of damage to the bones of the skull, hypertension progresses inside the corresponding cavity. This causes partial inhibition of mental development. Visually you can see a change in the configuration of the skull.
Patients may additionally suffer from nausea, vomiting, and impaired motor function of the eyes. They sometimes protrude (exophthalmos). A typical symptom is headache, which does not respond well to traditional treatment.
If synostosis affects the ribs, the process may occur without any symptoms. The diagnosis is sometimes made only during a routine medical examination. Fusion of the ribs is an incidental finding by the radiologist.
The clinical picture of synostosis depends on the location of the lesion and the volume of functional activity of the structures involved in the pathological process.
Which doctor treats synostosis?
Treatment of pathological bone fusion is traditionally carried out by a traumatologist or orthopedist. The doctor conducts a comprehensive examination of the patient and prescribes appropriate therapy depending on the characteristics of the clinical case.
Sometimes bone damage can be accompanied by pathology of internal organs. Then the orthopedist sends his patient to a neurologist, ophthalmologist, cardiologist, etc. Depending on the predominance of damage to one or another system, an additional specialist is selected.
Craniostenosis
Craniostenosis is a premature process of joining together individual bones of the skull at natural sutures.
As a result of this pathology, the configuration of the head often changes. It may be flattened on the sides and have an irregular characteristic protrusion in the forehead area. It all depends on the localization of the process.
Symptoms of craniostenosis:
- Increased pressure inside the skull.
- Partial mental retardation.
- Headache.
- Dyspepsia, manifested by nausea and vomiting.
- Strabismus.
- Disturbance in sleep rhythm and emotional state.
Craniosynostosis can be treated surgically.