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  • Updated 09.16.2021
  • Released 06.02.2003
  • Expires For CME 09.16.2024

Brachial plexus palsy in neonates

Introduction

Overview

Neonatal brachial plexus palsy can be a debilitating injury that leads to long-term disability in the infant. Although the majority of cases do recover full function of their arm, others must undergo surgery or other treatments to improve function. This article describes the pathology and anatomy of brachial plexus palsy, provides tips to examine an affected patient, and reviews the available treatment options.

Key points

• The most common brachial plexus palsy involves the upper roots, C5 and C6, and is called Erb palsy.

• The spontaneous recovery rate is 60%, and some children suffer permanent impairment.

• Surgical treatment is usually offered between 3 to 6 months of age for primary repair; otherwise, patients can undergo secondary procedures around 12 to 18 months of age.

• A multidisciplinary approach with the pediatrician, physical and occupational therapists, and the surgeon is very helpful when treating an infant with brachial plexus palsy.

Historical note and terminology

Neonates who have neurologic deficits have been observed since the time of the ancient Greeks and Hippocrates (65). Brachial plexus palsy related to the birth process, “obstetric brachial palsy,” was described by Scottish obstetrician William Smellie (1697–1763) in a textbook originally published in 1754 titled A sett of anatomical tables, with explanations, and an abridgment, of the practice of midwifery (134; 135; 136; 137; 95; 114; 127; 133).

William Smellie (1697–1763)
Scottish obstetrician William Smellie (1697–1763). Smellie described obstetrical brachial plexus palsy in 1654. Line engraving by R Anderson. (Courtesy of Wellcome Images and Wikimedia Commons. Creative Commons Attribution 4.0 Int...

German neurologist and neurophysiologist Wilhelm Heinrich Erb (1840–1921) first compared neonatal brachial plexus palsy with that found in adults and concluded that it involved the upper roots (157; 124).

Wilhelm Heinrich Erb (1840–1921)
German neurologist and neurophysiologist Wilhelm Heinrich Erb (1840–1921). Erb first compared neonatal brachial plexus palsy with that found in adults and concluded that it involved the upper roots. (Courtesy of the U.S. National ...

The term “Duchenne-Erb palsy” was coined because French neurologist Guillaume-Benjamin-Amand Duchenne (de Boulogne) (1806–1875) received credit for describing the brachial plexus palsy following delivery of affected newborns (41; 19; 120; 73; 57; 109; 121; 124).

Guillaume-Benjamin-Amand Duchenne (de Boulogne) (1806–1875)
French neurologist Guillaume-Benjamin-Amand Duchenne (de Boulogne) (1806–1875). Duchenne described the brachial plexus palsy following delivery of affected newborns. (Courtesy of Wikimedia Commons. Public domain.)

In addition, brachial plexus injury to the lower roots (C8-T1) was first described by American-born French neurologist Augusta Klumpke (1859–1927) in 1885 (80; 112; 17; 18; 57; 130; 161; 45).

Augusta Klumpke (1859–1927)
American-born French neurologist Augusta Klumpke (1859–1927) (right) and her husband Jules Dejerine (1849–1917). (Courtesy of Wellcome Images and Wikimedia Commons. Creative Commons Attribution 4.0 International License.)

Erb palsy in Kaiser Wilhelm II of Germany. In 1859 Wilhelm was born in Berlin to Victoria (or "Vicky"; 1840-1901), Princess Royal, the eldest daughter of Britain's Queen Victoria (1819-1901), and Prince Frederick William of Prussia (the future Frederick III; 1831-1888)--a marriage intended to unite the royal families of Britain and Germany. A traumatic breech birth resulted in an Erb palsy, which left Wilhelm as an adult with a withered left arm about 6 inches (15 centimeters) shorter than his right. In a letter to her mother, Queen Victoria, Vicky wrote, "The idea of his remaining a cripple haunts me." She felt a cure had to be found, and subjected Wilhelm to various bizarre procedures that served only to traumatize the boy. Beginning at 6 months of age and continuing for years, a hare was slaughtered in his presence twice a week to provide "animal baths"; the still-warm flesh of the dead hare was then tied around boy's left arm, with the hope that the vitality of the wild animal would somehow be transferred into the boy's dysfunctional arm. As the boy became more mobile, they tied his good arm behind his back thinking that this would force him to use and strengthen his weak arm, but this was of course a plan destined to fail and one that only heaped frustration and agony on the growing boy. In another letter to her mother, Queen Victoria, Vicky wrote: "It [He] gets so fretful and cross and violent and passionate that it makes me quite nervous sometimes." For most of his childhood, Wilhelm's withered arm was also subjected to electrotherapy treatments that were not only painful but that succeeded only in provoking a nervous reaction in the young boy. It was only at age 12 that the many, varied, and painful attempts to cure his disability were finally abandoned. Even then, Wilhelm's deformity was considered an embarrassment that had to be kept hidden from public view, but the deformity was, of course, still present, to his mother's shame. Vicky viewed the delivery of a less than physically perfect heir to the Prussian throne as a personal failure and, moreover, one that raised concerns about the ability of her son to thrive in the militant court atmosphere that was his destiny. In still another letter to her mother, Vicky confessed that the deformity "spoils all the pleasure and pride I should have in him." Although her rejection of her son stemmed in part from her failure to bring forth a strong male heir, no doubt compounded by the responsibility she felt (or blame she perceived) as the English mother of a German heir, it was nevertheless a quite terrible reaction by a mother toward her own son and one that Wilhelm reacted to in anger and resentment.

In most of the extant photographs and paintings, the royal photographers and painters concocted a remarkable diversity of subterfuges to obscure the left hand or to otherwise conceal its deformity (although some paintings and sculptures adopted an altogether different approach: just portray the arm and hand as if they were normal!). Photographs and paintings show Wilhelm holding a pair of white gloves in his left hand to make the arm seem longer, hiding his left hand under crossed arms, hiding his left arm behind his back, covering his left hand with his right on the hilt of a sword, putting his left hand in a pocket, holding his left hand with his right, holding a cane to give the illusion of a useful limb posed at a dignified angle, or hiding his hand and arm under a cape. There are also some officially sanctioned paintings and sculptures that portray his left arm and hand as if they were normal.

The disability from obstetric brachial plexus palsy clearly affected Wilhelm’s emotional development, and seemingly caused him to over-compensate through aggressiveness and competitiveness. Instead of his life providing a bridge to strengthen the ties of Britain and German, he ultimately initiated actions that split them apart.

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