Left Thenar Hypoplasia
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Dr. Prabhpreet Singh
Left Thenar Hypoplasia
Cavanagh Syndrome(Congenital Thenar Hypoplasia)


Introduction

The case is a rare variant of Cavanagh Syndrome who presented as congenital left thenar hypoplasia, absence of left radial artery along with bilateral absence of dorsalis pedis arteries.

This association has not been reported previously in India. Genetic Mutations have not been reported with this syndrome.

Case Report

A 5 year old child presented with fever, drowsiness and photophobia of 3 days duration. There was no history of any septic foci, trauma, drug intake or exposure to toxic substances. He was born of non-consanguineous marriage and rest of the history was non contributory.

On examination, the child was fully conscious, alert and oriented with normal vitals. It was noted that the left radial and bilateral dorsalis pedis pulses were absent, while other central and peripheral pulses were present. Blood pressure readings in both upper and lower limbs were normal for age. There was no apparent cranial nerve, motor, or sensory deficit. Fundus examination was normal. Cardiovascular examination did not reveal any murmurs or cardiomegaly.
Radiographic findings and orthopedic evaluation ruled out the possibility of Carpel tunnel syndrome.
Neuroimaging of brain and spinal chord was normal. On colour Doppler study, the arteries of the right
Upper limb, that is : brachial, ulnar and interosseous arteries showed low resistance flow, while the left radial artery could not be visualized from the bifurcation. In the lower limbs, anterior tibial artery ended at the level of ankle on both sides while distally it divided into digital branches. The dorsalis pedis artery could not be visualized on either sides. Thus, the study of all the four limbs revealed absence of left radial and both the dorsalis pedis arteries.

Thenar muscle anomalies and thumb hypoplasia is seen as an isolated defect in Cavangarh syndrome. However, it is commonly seen in association with other syndromes such as Holt-Oram, TAR (Thrombocytopenia-absent-radius), Fanconi and radial bone anomalies

Cavanagh Syndrome(Congenital Thenar Hypoplasia)


Introduction

The case is a rare variant of Cavanagh Syndrome who presented as congenital left thenar hypoplasia, absence of left radial artery along with bilateral absence of dorsalis pedis arteries.

This association has not been reported previously in India. Genetic Mutations have not been reported with this syndrome.

Case Report

A 5 year old child presented with fever, drowsiness and photophobia of 3 days duration. There was no history of any septic foci, trauma, drug intake or exposure to toxic substances. He was born of non-consanguineous marriage and rest of the history was non contributory.

On examination, the child was fully conscious, alert and oriented with normal vitals. It was noted that the left radial and bilateral dorsalis pedis pulses were absent, while other central and peripheral pulses were present. Blood pressure readings in both upper and lower limbs were normal for age. There was no apparent cranial nerve, motor, or sensory deficit. Fundus examination was normal. Cardiovascular examination did not reveal any murmurs or cardiomegaly.
Radiographic findings and orthopedic evaluation ruled out the possibility of Carpel tunnel syndrome.
Neuroimaging of brain and spinal chord was normal. On colour Doppler study, the arteries of the right
Upper limb, that is : brachial, ulnar and interosseous arteries showed low resistance flow, while the left radial artery could not be visualized from the bifurcation. In the lower limbs, anterior tibial artery ended at the level of ankle on both sides while distally it divided into digital branches. The dorsalis pedis artery could not be visualized on either sides. Thus, the study of all the four limbs revealed absence of left radial and both the dorsalis pedis arteries.

Thenar muscle anomalies and thumb hypoplasia is seen as an isolated defect in Cavangarh syndrome. However, it is commonly seen in association with other syndromes such as Holt-Oram, TAR (Thrombocytopenia-absent-radius), Fanconi and radial bone anomalies
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Mar 12, 2019Like