Congenital pseudarthrosis of the clavicle is a rare condition and till
date only about 200 cases have been reported in literature. Once such
rare case of congenital pseudoarthosis affecting right clavicle of a
girl is presented 

Salati, S.A.

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Congenital Pseudarthrosis of the Clavicle

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Congenital pseudarthrosis of the clavicle is a rare condition and till date only about 200 cases have been reported in literature. Once such rare right clavicle of a girl is presented

Salati, SA

AJOL - African Journals Online

  ISSN 2073COSECSA/ASEA Publication        East and Central African Journal of Surgery.  Congenital Pseudarthrosis of the ClavicleS. A. Salati,  College of Medicine, Qassim University, KSA                                                                 Email: docsajad@gmail.com  Congenital pseudarthrosis of the clavicle is a rare condition and till date only about 200 cases have been reported in literature. Once such rare right clavicle of a girl is presented . Introduction Congenital pseudarthrosis of the clavicle is a rare condition and after being reported for the fin 1910, only about 200 cases have been reported inpredominantly right sided and etiology is still unclear. Imaging plays a vital role in diagnosis and subsequent management. Surgical management comprises of excision of pseudarthrosis with internal/external fixation of bony ends with or without bone grafting. Due to rarity of this case of a girl reporting with congenital pseudarthrosis of right clavicle is presented. Case presentation   A 12 years old girl, first in birth order was brought byright clavicular area. The swelling had been noticed by parents since her birth and had been growing in size gradually as the patient grew up with age. The girl was first in birth order and had no other medical, surgical or family history of significance. There was no history of a difficult delivery or injury.  On examination, the patient was of average built and had a non tender bony lump middle of right clavicle. The skin over the lump had no abincreased with abduction of the right Plain images showed characteristic pattern of congenital in the diaphysis and anterior and superior tilting of the sternal half and a smaller acromial portion with bulbous ends. The CTscans with the 3with no evidence of developing callus or soft tissue     Figure 1. Preoperative (A) and two years postoperative followpseudarthrosis of right clavicle.  -9990   East Cent. Afr. J. surg. (Online) 2012  March/ April;17(1)                  case of  congenital pseudoarthosis affecting   literature in last hundred years. The condition is   parents with a unsightly prominence over the normal features. The size ofshoulder. The ranges of motions of right shoulder were normalpseudarthrosis of right clavicle-D reconstruction images showed the bipartite right clavicle mass and confirmed the diagnosis.  -up (B) images of conge text]      Page 126                            irst time condition, a lateral to the  the lump .  with a defect  enital   ISSN 2073-9990   East Cent. Afr. J. surg. (Online) e text] COSECSA/ASEA Publication        East and Central African Journal of Surgery.  2012  March/ April;17(1)      Page 127   Figure 2 . Plain image (A) and 3- D reconstruction CT scans ( B, C, D) showing features of congenital pseudarthrosis of right  clavicle.   Figure 3.  Follow up plain images at 6 months after surgery of congenital pseudarthrosis of right  clavicle.  The patient was transferred to the care of pediatric orthopedic surgeon and underwent operative intervention involving resection of the pseudarthrosis, with iliac bone graft interposition, and internal fixation. The patient was seen after 2 years for a different reason. She was satisfied with the outcome of clavicular surgery. She had full range of movement of the right shoulder and arm, and was pain-free. There was minimal residual prominence over the lateral half of right clavicle and a subclavicular scar which was widened over a length of 3 cm laterally. She was offered the option of scar revision under local anesthesia.    ISSN 2073-9990   East Cent. Afr. J. surg. (Online) e text] COSECSA/ASEA Publication        East and Central African Journal of Surgery.  2012  March/ April;17(1)      Page 128  Discussion  Congenital pseudarthrosis of the clavicle is a rare disorder of the shoulder girdle.  The condition was initially described by Fitzwilliams1 as a distinct variant of cleidocranial dysostosis in 1910 and since then with only approximately 200 individual cases having been reported in the world literature to date2. It presents predominantly on the right side (about 90%) of the patients 2,3. The left sided involvement usually occurs with dextrocardia and situs inversus 4. Bilateral cases can occur but are rare and are typically associated with genetic syndromes 5. The exact etiology remains unknown but it is believed that the lesion is caused by pressure exerted upon the developing clavicle by the pulsating subclavian artery.  The right subclavian artery is normally situated at a higher level, which explains the predominant right clavicular involvement6. Another theory suggests the condition to be due to the separation of the two primary ossification centers 7.  Bilateral involvement is related to an abnormally high subclavian artery on both sides, caused by cervical ribs or high vertically oriented upper ribs. Some cases occurring in members of the same family have been reported in literature, leading to theories that it may be an inherited disease with an autosomal dominant transmission8.     Congenital pseudarthrosis of the clavicle usually presents as a painless prominence lateral to the middle of the clavicle at birth or in early neonatal life.  The ends of the clavicular segments are enlarged at the site of the pseudarthrosis and there is some degree of motion between them. The deformity tends to become more obvious and unsightly as the child grows with age. The skin above the prominence may become atrophic. The vertical border of the scapula on the affected side looks asymmetrical and prominent, while the affected shoulder is lower. The deformity becomes greater when the patient abducts the arm. The condition is generally painless but mild to moderate pain   may be present around the shoulder girdle and upper  arm in some cases. A few patients may also develop functional problems of the upper limb of affected side like weakness, limited shoulder abduction or features of thoracic outlet obstruction9,10 .  The differential diagnoses of congenital pseudarthrosis of the clavicle include obstetric fracture, post-traumatic non-union, cleidocranial dysostosis, or neurofibromatosis11. An obstetric fracture of the clavicle is suggested by history of a difficult delivery, pseudoparalysis of the arm with no voluntary limb movement and pain on passive movement, or when a massive callus is seen radiographically. Traumatic nonunion can be differentiated by plain radiographs as in the non union the bone ends seem attenuated. In cleidocranial dysostosis is there are other skeletal abnormalities such as skull deformities, small size of the facial bones, scoliosis and deficiencies of the pelvis. In neurofibromatosis, the bony ends are attenuated and other characteristic stigmata are present which include axillary / inguinal freckling and cafe-au-lait spots on the skin. In this regard, clavicular pseudoarthrosis is different from tibial pseudoarthrosis. In tibial pseudoarthrosis, 50% of cases are associated with neurofibromatosis and in such cases there may be a lytic lesion seen within the bone.  The deformity is evaluated with imaging which generally include plain radiographs and CT scan. Anterior, posterior, and apical lordotic views usually are sufficient for plain imaging and shows a characteristic pattern with involvement of the middle third of the clavicle, with anterior and superior tilting of the sternal half and a smaller acromial portion ,lack of reactive callus, and bulbous appearance of the lateral segment at the pseudarthrosis without tapering of the bone ends. The CTscans and particularly the 3-D (three dimensional) reconstruction images are being widely used  recently as accurate, noninvasive diagnostic tools and play a vital role  in reaching a diagnosis and formulating a treatment plan . MRI may be indicated in occasional cases (like thoracic outlet syndrome) to determine the extent of the fibrous union, the location of the great vessels, and the space available within the thoracic outlet.  Treatment of this condition varies. When patients are asymptomatic and have no functional disability, some authors do not recommend any treatment12-13. Conversely it has also been reported in literature that, if treated conservatively, the deformity can increase and lead to instability of the shoulder girdle   ISSN 2073-9990   East Cent. Afr. J. surg. (Online) e text] COSECSA/ASEA Publication        East and Central African Journal of Surgery.  2012  March/ April;17(1)      Page 129  and to neuro-vascular complications. Surgical treatment is recommended for symptomatic patients with dysfunction of the arm, when patients are embarrassed by the cosmetic appearance of the lump and to prevent thoracic outlet syndrome in the future. The optimal age for surgery is controversial. Many workers recommend that surgery be done between 3 and 4 years of age. Operative intervention involves resection of the pseudarthrosis, with or without bone graft interposition, and internal11,14 or external fixation2 .  Histopathological analysis of resected specimen confirms the diagnosis of a true congenital pseudarthrosis of the clavicle by revealing features of false joint with the ends of the clavicle covered with hyaline cartilage caps. The appearance of the cartilaginous caps  is considered equivalent to that of developing physes and  were proven to be adding new bone to the bony ends15. These observations are also supporting the hypothesis that the pseudarthrosis is caused by failure of two ossification centers to fuse, as has proposed by many workers in literature 7.  Acknowledgement:   I acknowledge with thanks the help provided by patient and her guardians in allowing us to use the case history and images for publication.  References  1. Fitzwilliams D.C.L. Hereditary craniocleidodysostosis. Lancet 1910; 2:1466-75 2. Beslikas TA , Dadoukis DJ , Gigis IP, Nenopoulos SP, Christoforide JE. Congenital pseudarthrosis of the clavicle: a case report . Journal of Orthopaedic Surgery 2007; 15(1):87-9 3. Sloan A, Paton R. Congenital pseudarthrosis of the clavicle: the role of CT-scanning. Acta Orthop Belg. 2006; 72(3):356-58 4. Mooney JF, Koman LA. Bilateral congenital pseudarthrosis of the clavicle associated with trisomy 22.Orthopedics 1991; 14(2):171-73.  5. Sakkers RJ, Tjin a Ton E, Bos CF. Left-sided congenital pseudarthrosis of the clavicula. J Pediatr Orthop B 1999; 8(1):45-7 6. Hirata S, Miya H, Mizuno K. Congenital pseudarthrosis of the clavicle. Histologic examination for the etiology of the disease. Clin Orthop Relat Res 1995; 315:242–45 7. Lorente Molto FJ, Bonete Lluch DJ, Garrido IM. Congenital  pseudoarthrosis of the clavicle: a proposal for early surgical  treatment. J Pediatr Orthop 2001;21:689-93 8. Akman YE, Dogan A, Uzumcugil O, Azar N, Dalyaman E, Kabukcuoglu YS. Congenital pseudarthrosis of the clavicle in two siblings. Acta Orthop Traumatol Turc. 2008; 42(5):377-81 9. Lozano P, Doaz M, Riera R, Gomez FT. Venous thoracic outlet syndrome secondary to congenital pseudoarthrosis of the clavicle. Presentation in the fourth decade of life. Eur J Vasc Endovasc Surg. 2003 ; 25(6):592-93 10. Sales de Gauzy J, Baunin C, Puget C, Fajadet P, Cahuzac JP. Congenital pseudarthrosis of the clavicle and thoracic outlet syndrome in adolescence. J Pediatr Orthop B. 1999; 8(4):299-301 11. Andrew S, Robin P  Congenital pseudarthrosis of the clavicle : The role of CT-scanning. Acta Orthop. Belg., 2006; 72:356-58 12. Grogan DP, Love SM, Guidera KJ, Ogden JA. Operative treatment of congenital pseudarthrosis of the clavicle. J Pediatr Orthop 1991;11:176–80 13. Cataldo F. A 7-month-old child with a clavicular swelling since birth. Eur J Paediatr 1999 ; 158 :1001-02 14. Cadilhac C, Fenoll B, Peretti A, Padovani JP, Pouliquen JC, Rigault P.   Congenital pseudarthrosis of the clavicle: 25 childhood cases. Rev Chir Orthop Reparatrice Appar Mot. 2000; 86(6):575-80  15. Hirata S, Miya H, Mizuno K.Congenital pseudarthrosis of the clavicle. Histologic examination for the etiology of the disease.  Clin Orthop Relat Res. 1995; 315:242-45   

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Congenital Pseudarthrosis of the Clavicle

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