Presentation
Clinical presentation Usually presents as a midclavicular swelling in the neonate or young child 1. Pathology Congenital pseudoarthrosis of the clavicle is more common on the right, although it can be bilateral 2. [radiopaedia.org]
This is the form that presents a better prognosis. [scielo.br]
Epidemiology Incidence rare Anatomic location right side most common situs inversus is the exception bilateral in <10% Etiology Pathophysiology postulated to be due to extrinsic compression by the subclavian artery Genetics no clear genetic involvement Presentation [orthobullets.com]
It usually presents as a swelling in the clavicular region at birth or soon after birth. [ijoonline.com]
In congenital pseudoarthrosis of the clavicle, there are no other skeletal abnormalities present, a feature that distinguishes it from cleidocranial dysostosis. [boneandspine.com]
Treatment
Operative treatment succeeds best among children aged two to four years and these patients have the highest chance of achieving consolidation. The age for surgical treatment is very variable. [scielo.br]
Treatment is observation in the case of minimal symptoms and cosmetic deformity. Surgical management is indicated in the event of persistent pain or functional limitations. [orthobullets.com]
Treatment of Congenital Pseudoarthrosis Of Clavicle No conservative treatment achieves the union and not all the cases require surgical treatment. [boneandspine.com]
Management and treatment No treatment is required when functional capacity is satisfactory. Surgery (excision of the pseudarthrosis, internal fixation and bone grafting) is indicated to correct shoulder deformities interfering with function. [orpha.net]
Prognosis
[…] and cosmetic deformity Operative - very uncommmon ORIF with iliac crest bone grafting at age 3-6 years indications pain functional impairment cosmesis outcomes successful union is usually obtained avoid bone graft substitute, higher rates of non-union Prognosis [orthobullets.com]
Complications: Complications Stiffness of the Ankle and Hind foot Re-fracture Valgus Ankle Deformity Tibial Shortening prognosis: prognosis Varies with type Good with type III & IV Worse with II & V Worse with congenital or early onset shortening sclerosis [authorstream.com]
Pseudarthrosis develops through a congenital cyst Type IV : Pseudarthrosis develops in a sclerotic segment of bone without narrowing, stress fracture Type V : Pseudarthrosis of the tibia occurs with a dysplastic fibula, pseudarthrosis of one or both bones (prognosis [orthopaedicsone.com]
The prognosis of nonunion if treated depends on many factors including the age and general health of the patient, the time since the original injury, the number of previous surgeries, smoking history, the patient's ability to cooperate with the treatment [en.wikipedia.org]
Involvement of the left side usually occurs with dextrocardia and situs inversus. [13] Bilateral cases can occur but are rare and are typically associated with genetic syndromes. [14, 15] Prognosis The outcome is usually excellent, with prompt healing [emedicine.medscape.com]
Etiology
The etiology is unknown but an intrauterine pathogenesis is favored over non-union of birth trauma. Associations cleidocranial dysostosis trisomy 21 neurofibromatosis type 1 References Promoted articles (advertising) [radiopaedia.org]
Involvement of the left side usually occurs with dextrocardia and situs inversus. [11] Bilateral cases can occur but are rare and are typically associated with genetic syndromes. [12, 13] Etiology The etiology is unknown, and no relationship with neurofibromatosis [thehealthscience.com]
Etiology The etiology is unknown. The condition is not associated with trauma, neurofibromatosis or fibrous dysplasia. A family history is reported occasionally. [orpha.net]
Epidemiology Incidence rare Anatomic location right side most common situs inversus is the exception bilateral in <10% Etiology Pathophysiology postulated to be due to extrinsic compression by the subclavian artery Genetics no clear genetic involvement [orthobullets.com]
[…] synonyms: clavicle pseudoarthrosis Clavicle Pseudoarthrosis ICD-10 Q74.0 Other congenital malformations of upper limb(s), including shoulder girdle Clavicle Pseudoarthrosis Etiology / Epidemiology / Natural History rare etiology unknown, theories=intrinsic [eorif.com]
Epidemiology
Epidemiology Incidence rare Anatomic location right side most common situs inversus is the exception bilateral in <10% Etiology Pathophysiology postulated to be due to extrinsic compression by the subclavian artery Genetics no clear genetic involvement [orthobullets.com]
Summary Epidemiology Approximately 200 cases have been reported worldwide. Clinical description In the majority of cases, the abnormality occurs on the right clavicle and shoulder and arm movements are normal. [orpha.net]
[…] synonyms: clavicle pseudoarthrosis Clavicle Pseudoarthrosis ICD-10 Q74.0 Other congenital malformations of upper limb(s), including shoulder girdle Clavicle Pseudoarthrosis Etiology / Epidemiology / Natural History rare etiology unknown, theories=intrinsic [eorif.com]
Epidemiology Frequency Congenital pseudoarthrosis is rare. Fewer than 200 cases have been reported in the English literature. The exact incidence and prevalence are unknown. The abnormality occurs almost entirely on the right side. [thehealthscience.com]
335 21 Developmental Anomalies of the Hand 339 22 The Shoulder and Elbow 356 238pt Brachial Plexus Injuries Peripheral Nerve Injuries 365 Section 2 The Lower Limb 387 25 The Limping Child 423 37 Spondylolisthesis 638 Part V Fractures 647 39 Fracture Epidemiology [books.google.com]
Pathophysiology
Epidemiology Incidence rare Anatomic location right side most common situs inversus is the exception bilateral in <10% Etiology Pathophysiology postulated to be due to extrinsic compression by the subclavian artery Genetics no clear genetic involvement [orthobullets.com]
Pathophysiology Congenital pseudoarthrosis of the mid portion of the clavicle occurs when an environmental insult or anatomic or mechanical event disrupts diaphyseal membranous ossification. [thehealthscience.com]
Most authors have proposed an abnormal coalescence of the two clavicle ossification centers as the underlying pathophysiology for CPC [2-4]. Thus, none of the currently known theories can completely reveal the underlying cause of CPC. [journals.eco-vector.com]
Appropriate interpretation will improve understanding of the natural history and pathophysiology of neurofibromatosis type 1. [nature.com]
Pathophysiology The clavicle is the first bone to undergo membranous ossification. It connects the sternum to the acromion and provides support for shoulder function. [emedicine.medscape.com]
Prevention
Management: Indications for surgical intervention includeprogressive pain, esthetic deformity, functional limitation and to prevent future onset thoracic outlet syndrome.Controversies exist about the optimal age for surgery; intervening during infancy [dailyrounds.org]
[…] mineralisation, if normal osteoid can be produced Cartilage formation is entirely lacking or very scant Treatment Bowing of the tibia pre-fracture or a pre-pseudarthrosis lesion is best treated initially with a total contact orthosis, which may delay or prevent [orthopaedicsone.com]
Treatment is appropriate to create a unified bone that will balance the forearm and prevent progressive deformity and angulation. [congenitalhand.wustl.edu]
In children To prevent this deformity or halt its progression a synostosis is created between distal tibia and fibular metaphyses. [authorstream.com]
[…] ends are not apposed (that is, they are not next to each other) infection (particularly osteomyelitis ) the fracture is not fixed (that is, the two ends are still mobile) soft-tissue imposition (there is muscle or ligament covering the broken ends and preventing [en.wikipedia.org]