Sunday, 14 November 2010

Bifid Ribs


Bifid Rib Clinical Features:
  • Bifid rib is usually unilateral.
  • May occur in isolation.
  • Bifid ribs are usually asymptomatic.
  • May also found with multi-system malformations.
  • They often discovered incidentally by chest x-ray.
Clinical Assessment:
  • Examine the mouth & skin carefully.
  • Physical findings such as odontogenic cysts, subcutaneous calcifications, & palmar pits should prompt more extensive radiological / radiological investigations.
  • With normal physical examination, & asymptomatic patient, further radiological assessment gives a low yield for abnormalities.
Clinical Associations:
  • Gorlin-Goltz (Basal cell nevus) syndrome (65-70% of patients) [2,10]
  • Job's syndrome [3]
  • Kindler syndrome [4]
  • Malignancy in childhood (esp neuroblastoma) [5]
Complications:
  • Asymptomatic (usually)
  • Intercostal nerve entrapment
  • Musculoskeletal pain
  • Thoracic outlet syndrome [6]
Epidemiology:
  • Prevalence of bifid rib ~ 0.15% to 3.4% (~ 2%). [1]
  • Up to 20% of all congenital rib anomalies. [1]
  • Bifid ribs occur in up to 8.4% of Samoans. [8]
  • Bifid ribs on chest Xray occur in 2.8% of Koreans. [9]
Morphological Patterns:
  • Two distinct morphological patterns. [7]
  • 1) - long and slender (fissured) bifid spaces, with the bifid rib appearing as a two-pronged fork (fork type). [7]
  • 2) - more rounded bifid spaces (hole type). [7]
Pathophysiology:
  • Ribs originate from the mesoderm.
  • Bifid ribs may therefore be associated with other mesodermal abnormalities.
  • Look specifically for malformations in other organs of mesodermal origin, i.e. heart & kidneys. [1]
Prognosis:
  • That of underlying cause.
  • There is little information in the literature about the clinical significance of bifid ribs.
References:
  • [1] - Charles I, Scott J. Pectoral girdle, spine, ribs, and pelvic girdle. In: Stevenson RE, Hall JG, Goodmann RM, eds. Human Malformations and Related Anomalies, vol 2. Oxford University Press: New York, 1993:655–697.
  • [2] - Rai S, Gauba K. Jaw cyst-basal cell nevus-bifid rib syndrome: a case report. J Indian Soc Pedod Prev Dent 2007; 25:137–139.
  • [3] - Freeman AF, Holland SM. The hyper-IgE syndromes. Immunol Allergy Clin North Am 2008; 28:277–291.
  • [4] - Sharma RC, Mahajan V, Sharma NL, Sharma AK. Kindler syndrome. Int J Dermatol 2003; 42:727–732.
  • [5] - Schumacher R, Mai A, Gutjahr P. Association of rib anomalies and malignancy in childhood. Eur J Pediatr. 1992;151:432–434. [PubMed]
  • [6] - K. Ca─čli. Thoracic outlet syndrome in an adolescent with bilateral bifid ribs. Clin Anat. 2006 Sep;19(6):558-60.
  • [7] - Wu-Chul Song, Sang-Hyun Kim, Dae-Kyoon Park, and Ki-Seok Koh. Bifid Rib: Anatomical Considerations in Three Cases. Yonsei Med J. 2009 April 30; 50(2): 300–303
  • [8] - Martin EJ. Incidence of bifidity and related rib abnormalities in Samoans. Am J Phys Anthropol. 1960;18:179–187.
  • [9] - Lim CK, Lee KW, Bin JC, Rhee BC. Congenital anomalies of the ribs. J Korean Soc Plast Reconstr Surg. 1982;18:487–495.
  • [10] - Bitar GJ, Herman CK, Dahman MI, Hoard MA. Basal Cell Nevus Syndrome: Guidelines for Early Detection. Am Fam Physician. 2002 Jun 15;65(12):2501-2505.
Image: Bifid Rib (CT Chest)
Image Credit: Dr Frank Gaillard (radpod.org) (cc)
Tags: Bifid Rib - Gorlin-Goltz Syndrome - Job Syndrome - Kindler Syndrome - Mesoderm - Neuroblastoma - Rib - Samoan
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