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Neonates with Duchenne-Erb palsy may also have facial, diaphragmatic, or hypoglossal paralysis. Facial paralysis occurs because of concomitant traumatic facial nerve lesions (Figure 218.1). Facial weakness involves the upper and lower quadrants and it is minimal or not present when the neonate is quiet or asleep but very noticeable when the neonate is crying (Figure 218.1).

A
B

Figure 218.1. Facial nerve injury in a neonate with Duchenne-Erb palsy. [A] When quiet, the face looks symmetrical. [B] When crying, there is a facial asymmetry that involves the lower and the upper quadrants of the face. The facial asymmetry is on the side opposite from the arm weakness.

Unilateral diaphragmatic paralysis is usually asymptomatic but it should be considered if a neonate with Duchenne-Erb palsy can not be removed from the respirator. Bilateral diaphragmatic paralysis produces inability to sustain effective respiration. Diaphragmatic paralysis is usually diagnosed by inspiratory and expiratory chest radiographs (Figure 218.2 [A and B]) or fluoroscopy. Phrenic nerve conduction studies may be necessary in some cases (Figure 218.2 [C]). The phrenic nerve arises from the anterior roots of C3-C5 spinal segments. The phrenic nerve becomes a single nerve over the brachial plexus and progresses caudally toward the diaphragm. Injuries to the phrenic nerve often occur at the level of the roots or over the brachial plexus. Unilateral diaphragmatic paralysis usually resolves spontaneously in 6 to 12 months. The only necessary management is clinical follow-up. Bilateral diaphragmatic paralysis may require surgical treatment if it persists for more than 2 months. Respiratory support is usually needed from birth. Tongue weakness due to hypoglossal nerve injury may occur. Clavicular fractures often occur with Duchenne-Erb palsy. They may not be noted clinically or by radiographs during the first 10 days. A lump in the clavicle is usually felt after 10 days.

A
B
C

Figure 218.2. Unilateral phrenic nerve injury. [A] Expiration film does not show diaphragmatic paralysis. [B] Inspiratory film demonstrates the presence of a nonfunctional left diaphragm. [C] Decreased amplitude of diaphragmatic contraction during phrenic nerve conduction.

 

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Haenggeli, 1989 Volpe, 1995