Birth palsy in congenital varicella syndrome: A lesson in an
Brachial plexus palsy presenting from birth is almost always attributed to trauma sustained during childbirth. Rarely, however, the paralysis may be due to in utero causes including agenesis of the cervical spinal nerve roots and various maternal infections. Maternal varicella (chickenpox) infection is well established to be a cause of various foetal deformations that are grouped under the moniker of ‘Congenital Varicella Syndrome’ (CVS). CVS involves the skin, the eyes, the limbs and the nervous system. Published in the Indian Journal of Plastic Journal the authors describe a child with CVS with associated upper brachial plexus palsy and Horner's syndrome.

Of note was a characteristic set of features that defined the patho-anatomy of the viral infection clearly. The possible site of origin of, and the literature on, each feature is then reviewed.

A 17-month-old first-born female child presented to the brachial plexus clinic with complaints of weakness of the right shoulder and elbow, noticed from birth. Hand and wrist movements were present at birth. No improvement in the movements over these 17 months was reported.

The paresis was accompanied by a prominent scar over the entire limb from the shoulder to the thumb. Both the thumb and index finger were also hypoplastic, apart from the scarring. The skin scar was reported to be bright red at birth (no pictures were available) that had lightened over time.

The child also had a ptosis of the right eye with lighter pigmentation of the iris as compared to the other eye. The iris was reported to be paler at birth and had apparently improved in pigmentation with growth. The mother had developed chickenpox after the 3rd month of pregnancy (week 13) that had been treated symptomatically. No antivirals or immunisation had been administered.

On examination, the general development was confirmed appropriate for age. The following clinical features were noted:


• The eye: Ptosis, miosis and enophthalmos were present in the right eye. The iris was light brown in colour compared to black on the left side

• The skin: The skin over the entire pre-axial border of the upper limb was scarred from the shoulder to the hand, including the hypoplastic thumb and index fingers

• Right upper limb: The limb was held in the classic ‘waiter's tip’ position described for an upper brachial plexus palsy. No active shoulder abduction or external rotation was present. Adduction and internal rotation were present but weak. The elbow was held fully extended with no active flexion and had good extension power. The wrist and the fingers showed full movements.

Given the age of the child, an exploration of the brachial plexus was not indicated. However, an Oberlin's nerve transfer could possibly be performed to attempt restoration of elbow flexion. Hence, the arm was explored for the status of the musculocutaneous nerve.

During surgery, the biceps was found to be completely pale and had undergone a severe fatty degeneration. Hence, the plan of nerve transfer was abandoned. The thumb was made more functional by phalangisation. The first web was released by a Gilles' hatchet flap, and a full-thickness graft was placed.

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