Indian Journal of Radiology and Imaging Indian Journal of Radiology and Imaging

MUSCULOSKELETAL
Year
: 2006  |  Volume : 16  |  Issue : 4  |  Page : 609--610

Radial club-hand: A case report


SV Phatak 
 Prathima Institute of Medical Sciences, Nagunur Road, Karimnagar (A.P) Pincode-505417, India

Correspondence Address:
S V Phatak
Consultant Radiologist, Prathima Institute of Medical Sciences, Nagunur Road, Karimnagar (A.P) Pincode-505417
India




How to cite this article:
Phatak S V. Radial club-hand: A case report.Indian J Radiol Imaging 2006;16:609-610


How to cite this URL:
Phatak S V. Radial club-hand: A case report. Indian J Radiol Imaging [serial online] 2006 [cited 2019 Sep 15 ];16:609-610
Available from: http://www.ijri.org/text.asp?2006/16/4/609/32281


Full Text

 Introduction



Club hand deformities are classified into two main categories radial and ulnar. Radial club hand includes a wide spectrum of disorders that encompass absent thumb, thumb hypoplasia, thin first metacarpal and absent radius. Ulnar club hand is much less frequent than radial clubhand and ranges from mild deviations hand on the ulnar side of forearm to complete absence of ulna. Where as radial club hand is frequently syndromatic ulnar club hand is usually isolated anomaly. [1] Radial club hand is a deficiency along the preaxial or radial side of the extremity. Although considerable forearm and hand anomalies are the classic findings proximal deficiencies can occur through out arm and shoulder girdle. Frequency of this anomaly is between 1:55000 to 1:100000live births. Most cases are sporadic without definable cause. [2]

 Case Report



A twenty six years old primi delivered a baby with abnormally short left forearm, radial club hand, absent thumb and index finger. Antenatal ultrasonography indicated severe IUGR and oligohydramnios. Radiograph of left forearm showed Radial aplasia. Baby was evaluated for other systemic malformations but no other anomaly was found.

 Discussion



Current theory relates the etiology of radial club hand to the apical ectodermal ridge (AER). This structure is a thickend layer of ectoderm that directs differentiation of underlying mesenchymal tissue and limb formation. Removal of a portion of AER in chick embryos has produced anomalies similar to radial club hand. Therefore a defect of AER is the most probable cause of radial club hand. The extent of deformity is related to the degree and extent of AER absence. Radial club hand is classified into four types based on the amount of radius present. Type one is mildest type characterized by mild radial shortening of radius with out considerable bowing, minor radial deviation is apparent although considerable hypoplasia of thumb may be evident. Type two means a miniature radius with distal and proximal physeal abnormalities and moderate deviation of the wrist. A type three deficiency is partial absence of radius, most commonly in distal portion and severe wrist radial deviation. Type four is complete absence of radius. This is the most common variant. [2] Abnormalities of bone and joint, muscles and tendons, nerves and arteries are seen with this condition. [3] Many anomalies can have association with Radial dysplasia. Cardiovascular anomalies include VSD, PDA, Coarctation, Dextrocardia, and pulmonary stenosis. Genitourinary includes ectopic kidney, hypoplastic kidney, urethral valve, horseshoe kidney and duplication. GIT anomalies seen are Esophageal atresia, TE fistula, anal atresia, small bowel atresia and malrotation. Skeletal conditions like scoliosis, hemivertebrae, Klein filter syndrome, sacral agenesis and hip dislocation. [4] Whenever a club hand is identified it is important to conduct a thorough examination of fetus and new born to delineate associated anomalies that may suggest a syndrome. Fetal blood sampling procedures and fetal echocardiography are recommended A complete blood cell count including platelets is important to diagnose hematological conditions like Fanconi's anemia, TAR syndrome, Aase syndrome. Fetal karyotype is indicated because several chromosomal anomalies may be associated (Trisomy 18 and 21,delition of long arm of chromosome 13 and ring formation of chromosome 4.Isolated radial club hand can present with other anomalies. [1] Radial defects may be unilateral or bilateral. Generally speaking this is a useful sign in differentiating isolated form from that associated with syndromes. It appears that most patients with unilateral radial dysplasia can not be easily classified as having the well defined inherited syndromes.even though they do have associated abnormalities .On the other hand cases associated with familial malformation syndromes usually have bilateral involvement. [5],[6] Syndromes associated with absent or hypoplastic thumb with radial dysplasia include Holt-Oram, DeLange, Daune, Ives Houston, and Roberts, RothmundThompson and Shokeir syndromes. [5]

References

1Roberto Romero Et al Fetal skeletal anomalies. RCNA vol.28, No.1, January 1989:94-95.
2Scott H.Kozin Radial Clubhand e medicine specialities .>orthopedic surgery>Hand and upper extremities Last updated Sept 5,2003.
3Sheron K.Skerik et al the anatomy of congenital radial dysplasia Its surgical and functional implications clinical orthopedics No.66, Sept-oct 1969:125-143.
4Jocyline Ledesma Medina Et al Radiographic manifestations of anomalies of the limb RCNA Vol.29, No.2, March 1991:383-405.
5Andrew K.Poznaski The hand in radiologic diagnosis with gamuts and pattern profiles WB Saunders company 1984 vol.1: 244-248.
6David Sutton Radial defects Textbook of Radiology and Imaging Churchill Livingstone Seventh edition, Vol.2, 2003:1108.