Congenital pseudarthrosis of the tibia (CPT) is a rare pathology with reported incidence between 1:140,000 and 1:250,000. The natural history of the disease is extremely unfavourable and once a fracture occurs, there is a little or no tendency for the lesion to heal spontaneously. Histologically tibia shows area of segmental dysplasia resulting in anterolateral bowing of the bone Treatment is mainly surgical. The key to get primary union is to excise hamartomatous tissue and pathological periosteum. Each child with CPT must be followed up till skeletal maturity to identify and rectify residual problems after primary healing. We are illustrating a case of severe neonatal form with posterior bowing at birth.

Keywords- Congenital pseudarthrosis, Fibrous dysplasia, Hamartoma

1. Hefti F, Bollini G, Dungl P, Fixsen J, Grill F, Ippolito E, et al. Congenital pseudarthrosis of the tibia: History, etiology, classification, and epidemiologic data. J Pediatr Orthop B. 2000; 9: 11–5.
2. Andersen KS. Radiological classification of congenital pseudarthrosis of the tibia. Acta Orthop Scand. 1973;44:719–27.
3. Cho TJ, Seo JB, Lee HR, Yoo WJ, Chung CY, Choi IH. Biologic characteristics of  fibrous  hamartoma  from  congenital  pseudarthrosis  of  the  tibia  associated   with neurofibromatosis    J  Bone Joint Surg  Am  2008;90—A:2735—44.
4. Crawford AH, Schorry EK. Neurofibromatosis update. J PediatrOrthop 2006;26:413—23.
5. Crawford Jr AH, Bagamery N. Osseous manifestations of neurofibromatosis in childhood. J Pediatr Orthop 1986;6:72—88.
6. Rogez JM. Courbures et pseudarthroses congénitale de jambe. Cahiers d’enseignement de la SOFCOT, 34: 237—244.  Paris: L’ Expansion Scientifique éd.; 1989.
7. Apoil A. Les pseudarthroses congénitales de jambe. À proposde 13 observations. Rev Chir Orthop 1970;56:120—38.

Priyanka Sharma

Department of OBG, Bhandari Hospital Hamirpur (H.P), India