Abstracts

Neonatal brachial plexus palsy (NBBP) is an ancient disease. There are references to this condition back to the the Old Testament, and Galen’s histories. The first scientific description was made by the Scottish obstetrician William Smellie, in 1768¹1 . Shenaq SM, Bullocks JM, Dhillon G, Lee RT, Laurent JP. Management of infant brachial plexus injuries. Clin Plast Surg. 2005;32(1):79-98. doi:10.1016/j.cps.2004.09.001. The classical neurologic description of the upper brachial plexus lesion was done by Duchenne in 1872 and Erb in 1874²2 . Terzis JK, Kokkalis ZT. Pediatric brachial plexus reconstruction. Plast Reconstr Surg. 2009;124:370e-85e. doi:10.1097/PRS.0b013e3181bcf01f. Augusta Klumpke, the first woman in France to be interne des hôpitaux, described the lower plexus lesion in 1885, including the ocular autonomic involvement³3 . Kay SP. Obstetrical brachial palsy. Br J Plast Surg. 1998;51(1):43-50. doi:10.1054/bjps.1997.0166. The most famous patient with this condition was Kaiser Wilhelm II, who ruled Germany during the First World War. Kennedy performed the first surgery in 1903²2 . Terzis JK, Kokkalis ZT. Pediatric brachial plexus reconstruction. Plast Reconstr Surg. 2009;124:370e-85e. doi:10.1097/PRS.0b013e3181bcf01f, but it was not until the convincing results reported by Gilbert in the 80’s that surgical treatment became an option for these patients⁴4 . Gilbert A, Razaboni R, Amar-Khodja S. Indications and results of brachial plexus surgery in obstetrical palsy. Orthop Clin North Am. 1988;19(1):91-105..

The incidence of NBBP varies from 0.5 to 3.0 cases per 1000 live births⁵5 . Evans-Jones G, Kay SP, Weindling AM, Cranny G, Ward A, Bradshaw A et al. Congenital brachial palsy: incidence, causes, and outcome in the United Kingdom and Republic of Ireland. Arch Dis Child Fetal Neonatal Ed. 2003;88:f185-9. doi:10.1136/fn.88.3.F185,⁶6 . Gilbert WM, Nesbitt TS, Danielsen B. Associated factors in 1611 cases of brachial plexus injury. Obstet Gynecol. 1999;93(4):536-40. doi:10.1016/S0029-7844(98)00484-0,⁷7 . Mollberg M, Hagberg H, Bager B, Lilja H, Ladfors L. High birthweight and shoulder dystocia: the strongest risk factors for obstetrical brachial plexus palsy in a Swedish population-based study. Acta Obstet Gynecol Scand. 2005;84(7):654-9. doi:10.1111/j.0001-6349.2005.00632.x. Despite the advances in modern obstetrics, its incidence has not declined during the last decades. In Sweden, there has been actually an increase in its incidence for unknown reasons, but higher birth weight in the population has been probably contributed to this fact⁷7 . Mollberg M, Hagberg H, Bager B, Lilja H, Ladfors L. High birthweight and shoulder dystocia: the strongest risk factors for obstetrical brachial plexus palsy in a Swedish population-based study. Acta Obstet Gynecol Scand. 2005;84(7):654-9. doi:10.1111/j.0001-6349.2005.00632.x. There is no data of Brazilian incidence, but it is probably in the lower spectrum due to the high proportion of cesarean sections in our country. Nevertheless, we have seen over 400 cases in our hospital in the last 14 years. The recent efforts of Brazilian government to increase the proportion of vaginal birth may actually increase our incidence of NBPP.

RISK FACTORS AND PREVENTION

There are several well-known risk factors for NBPP, however, its occurrence remains an essentially unpredictable event⁸8 . Wolf H, Hoeksma AF, Oei SL, Bleker OP. Obstetric brachial plexus injury: risk factors related to recovery. Eur J Obstet Gynecol Reprod Biol. 2000;88(2):133-8. doi:10.1016/S0301-2115(99)00132-3. Most cases have no recognizable cause and just a minority of deliveries with identifiable risk factors will result in brachial plexus lesions.

The first point relates to the existence of congenital lesions⁹9 . Alfonso DT. Causes of neonatal brachial plexus palsy. Bull NYU Hosp Jt Dis. 2011;69(1):11-6.. Although there are several convincing reports, these seem to account for a very small portion of cases. They have a different natural history, since limb atrophy is usually present since birth. Needle electromyography was once used to identify this situation based on the false assumption that an acute lesion would take about two weeks to generate abnormal muscle spontaneous activity¹⁰10 . Koenigsberger MR. Brachial palsy at birth: intrauterine or due to delivery trauma? Ann Neurol. 1980;8(2):228., but there is experimental evidence that this time frame is considerably shorter in newborns¹¹11 . Gonik B, McCormick EM, Verweij BH, Rossman KM, Nigro MA. The timing of congenital brachial plexus injury: a study of electromyography findings in the newborn piglet. Am J Obstet Gynecol. 1998;178(4):688-95. doi:10.1016/S0002-9378(98)70478-8.

The majority of NBPP is related to brachial plexus stretching during the delivery. The relative contributions of obstetrics maneuvers and uterine propulsion have been fervently debated due to its legal implications¹²12 . Malessy MJ, Pondaag W. Obstetric brachial plexus injuries. Neurosurg Clin N Am. 2009;20(1):1-14. doi:10.1016/j.nec.2008.07.024,¹³13 . Doumouchtsis SK, Arulkumaran S. Are all brachial plexus injuries caused by shoulder dystocia? Obstet Gynecol Surv. 2009;64(9):615-23. doi:10.1097/OGX.0b013e3181b27a3a. There are documented cases of NBPP without fetal head traction and the term “obstetric paralysis” has been condemned by several authors¹⁴14 . Jennett RJ, Tarby TJ, Kreinick CJ. Brachial plexus palsy: an old problem revisited. Am J Obstet Gynecol. 1992;166(6 Pt 1):1673-6. doi:10.1016/0002-9378(92)91555-O. The main risk factor for NBPP is shoulder dystocia which is reported in at least half of the cases¹⁵15 . Christoffersson M, Rydhstroem H. Shoulder dystocia and brachial plexus injury: a population-based study. Gynecol Obstet Invest. 2002;53(1):42-7. doi:10.1159/000049410. The fetal shoulder gets stuck under the pubic symphysis, opening the angle between the clavicle and cervical spine, and creating an upward tension gradient. This explains the higher incidence of upper brachial plexus lesions.

Birth weight is the most important fetal factor for NBPP, and it is clearly related to shoulder dystocia. A birth weight higher than 4.5 kg carries a ten-fold risk increase for brachial plexus lesions⁶6 . Gilbert WM, Nesbitt TS, Danielsen B. Associated factors in 1611 cases of brachial plexus injury. Obstet Gynecol. 1999;93(4):536-40. doi:10.1016/S0029-7844(98)00484-0. Maternal diabetes mellitus is also related to this, but also seems to have some independent risk contribution. Other maternal risk factors include obesity, short stature, and previous shoulder dystocia¹³13 . Doumouchtsis SK, Arulkumaran S. Are all brachial plexus injuries caused by shoulder dystocia? Obstet Gynecol Surv. 2009;64(9):615-23. doi:10.1097/OGX.0b013e3181b27a3a.

Forceps extractions are related to a higher risk for NBPP⁶6 . Gilbert WM, Nesbitt TS, Danielsen B. Associated factors in 1611 cases of brachial plexus injury. Obstet Gynecol. 1999;93(4):536-40. doi:10.1016/S0029-7844(98)00484-0; however, it is not clear if this is due to fetal traction or just an associated factor present in a difficult delivery situation. Pelvic deliveries are related to severe and often bilateral lesions, which are probably caused by cervical hyperextension¹⁶16 . Ubachs JM, Slooff AC, Peeters LL. Obstetric antecedents of surgically treated obstetric brachial plexus injuries. Br J Obstet Gynaecol. 1995;102(10):813-7. doi:10.1111/j.1471-0528.1995.tb10848.x. Cesarean sections have a protective effect, but cannot avoid NBPP completely⁶6 . Gilbert WM, Nesbitt TS, Danielsen B. Associated factors in 1611 cases of brachial plexus injury. Obstet Gynecol. 1999;93(4):536-40. doi:10.1016/S0029-7844(98)00484-0.

The indication of cesarean section for macrosomic babies would be a rational approach for prevention; however, fetal ultrasound is not very accurate for detection of large fetus⁷7 . Mollberg M, Hagberg H, Bager B, Lilja H, Ladfors L. High birthweight and shoulder dystocia: the strongest risk factors for obstetrical brachial plexus palsy in a Swedish population-based study. Acta Obstet Gynecol Scand. 2005;84(7):654-9. doi:10.1111/j.0001-6349.2005.00632.x,¹1 . Shenaq SM, Bullocks JM, Dhillon G, Lee RT, Laurent JP. Management of infant brachial plexus injuries. Clin Plast Surg. 2005;32(1):79-98. doi:10.1016/j.cps.2004.09.001⁷7 . Mollberg M, Hagberg H, Bager B, Lilja H, Ladfors L. High birthweight and shoulder dystocia: the strongest risk factors for obstetrical brachial plexus palsy in a Swedish population-based study. Acta Obstet Gynecol Scand. 2005;84(7):654-9. doi:10.1111/j.0001-6349.2005.00632.x. A cost-effective analysis indicated that it would take 3695 cesarean sections to prevent a single permanent NBPP in patients with estimated birth weight higher than 4.5 kg¹⁸18 . Rouse DJ, Owen J, Goldenberg RL, Cliver SP. The effectiveness and costs of elective cesarean delivery for fetal macrosomia diagnosed by ultrasound. JAMA. 1996;276(18):1480-6. doi:10.1001/jama.1996.03540180036030. The American College of Obstetricians and Gynecologists recommends cesarean section for estimated birth weight higher than 5 kg¹³13 . Doumouchtsis SK, Arulkumaran S. Are all brachial plexus injuries caused by shoulder dystocia? Obstet Gynecol Surv. 2009;64(9):615-23. doi:10.1097/OGX.0b013e3181b27a3a, which correspond to less than 4% of our cases.

CLINICAL PICTURE

The clinical presentation can be classified according to the anatomic structures compromised. NBPP is a closed supraclavicular lesion that affects sequentially the upper (C5-C6), middle (C7) and lower (C8-T1) brachial plexus trunks. The right side is affected in two thirds of the cases due to the most common fetal presentation. Bilateral cases are seen in up to 5%, but are usually asymmetric¹⁹19 . Dijk JG, Pondaag W, Malessy MJ. Obstetric lesions of brachial plexus. Muscle Nerve. 2001;24(11):1451-61. doi:10.1002/mus.1168.

Isolated lesion of the upper trunk (C5-C6), also known as Erb’s palsy or Narakas grade I injury, occurs in about half of cases. The typical limb posture is called “waiter’s tip”, in which the arm is adducted and internally rotated, the elbow is extended, and the wrist is flexed²⁰20 . Alfonso I, Alfonso DT, Papazian O. Focal upper extremity neuropathy in neonates. Semin Pediatr Neurol. 2000;7(1):4-14. doi:10.1016/S1071-9091(00)80005-4 (Figure 1). The Moro reflex is absent in the affected side, but the grasp reflex is normal. Motor deficit includes shoulder abduction, external rotation and elbow flexion. Biceps tendon reflex is lost, but pain sensibility is usually preserved.

Upper and middle trunk (C5-C7) lesions, or Narakas grade II injury, accounts for one third of the cases. In addition to the motor deficits seen in Erb´s palsy, elbow and wrist extension are also compromised¹⁹19 . Dijk JG, Pondaag W, Malessy MJ. Obstetric lesions of brachial plexus. Muscle Nerve. 2001;24(11):1451-61. doi:10.1002/mus.1168. Finger flexion is present, but usually weaker than the healthy side. All tendon reflexes are absent in the affected limb. Pain sensibility may be lost in the thumb or middle finger, and this is related to a poor prognosis²¹21 . Heise CO, Martins RS, Foroni LH, Siqueira MG. Prognostic value of thumb pain sensation in birth brachial plexopathy. Arq Neuropsiquiatr. 2012;70(8):590-2. doi:10.1590/S0004-282X2012000800006.

Total plexus lesions (C5-T1) are seen in the remaining 17% of the cases. Some patients can still show minor finger movements and are classified as Narakas grade III injury. Narakas grade IV picture is of a complete flail arm, with abnormal sensibility, and sympathetic ocular involvement known as Claude-Bernard-Horner syndrome³3 . Kay SP. Obstetrical brachial palsy. Br J Plast Surg. 1998;51(1):43-50. doi:10.1054/bjps.1997.0166,²⁰20 . Alfonso I, Alfonso DT, Papazian O. Focal upper extremity neuropathy in neonates. Semin Pediatr Neurol. 2000;7(1):4-14. doi:10.1016/S1071-9091(00)80005-4 (Figure 2). Isolated lower plexus lesions, known as Klumpke’s palsy, are extremely rare²²22 . Al-Qattan MM, Clarke HM, Curtis CG. Klumpke’s birth palsy. Does it really exist? J Hand Surg Eur Vol. 1995;20(1):19-23. doi:10.1016/S0266-7681(05)80008-7. Most reported cases were probably total plexus lesions which recovered upper plexus function after a while. These patients develop a late posture of elbow flexion, wrist extension and supination known as “beggar’s hand”²³23 . Al-Qattan MM, Al-Khawashki H. The “beggar’s” hand and the “unshakable” hand in children with total obstetric brachial plexus palsy. Plast Reconstr Surg. 2002;109(6):1947-52. doi:10.1097/00006534-200205000-00026.

ANCILLARY EXAMS

The diagnosis of NBPP is clinically obvious and no ancillary exam is necessary³3 . Kay SP. Obstetrical brachial palsy. Br J Plast Surg. 1998;51(1):43-50. doi:10.1054/bjps.1997.0166. Plain X rays can be helpful for detection of concurrent lesions, such as clavicular fracture or phrenic paralysis²2 . Terzis JK, Kokkalis ZT. Pediatric brachial plexus reconstruction. Plast Reconstr Surg. 2009;124:370e-85e. doi:10.1097/PRS.0b013e3181bcf01f. Electrodiagnosis and image studies can be useful for prognostic and surgical planning providing data to characterize the root viability. To that extend the lesion can be divided in preganglionic or postganglionic injury according to the localization related to dorsal root ganglion (DRG). In the preganglionic injury the lesion is proximal to the DRG and is associated with root avulsion or intraforaminal root injury. Accordingly, the root cannot be used as donor to reconstruct the brachial plexus. The postganglionic injury is distal to the DRG and the repair can be performed by interposing nerve grafts from the viable root (or roots) to the distal plexus.

Nerve conduction studies and electromyography were commonly performed at three months of age as part of preoperative investigation. Technical issues and overly optimistic results led several surgeons to abandon this procedure⁶6 . Gilbert WM, Nesbitt TS, Danielsen B. Associated factors in 1611 cases of brachial plexus injury. Obstet Gynecol. 1999;93(4):536-40. doi:10.1016/S0029-7844(98)00484-0,²⁴24 . Clarke HM, Curtis CG. An approach to obstetrical brachial plexus injuries. Hand Clin. 1995;11(4):563-80.,²⁵25 . Heise CO, Siqueira MG, Martins RS, Gherpelli JL. Clinical-electromyography correlation in infants with obstetric brachial plexopathy. J Hand Surg Am. 2007;32:999-1003. doi:10.1016/j.jhsa.2007.05.002. However, recent reports have shown that electrodiagnosis can be useful for prognostic estimation if performed earlier. Motor nerve conduction studies can estimate the percentage of motor axonal degeneration, which correlates with the functional outcome²⁶26 . Heise CO, Siqueira MG, Martins RS, Gherpelli JL. Motor nerve-conduction studies in obstetric brachial plexopathy for a selection of patients with a poor outcome. J Bone Joint Surg Am. 2009;91(7):1729-37.. Preservation of sensory potentials in a patient with severe paralysis is indicative of pre ganglionar lesion, which carries a grim prognosis²⁷27 . Vanderhave KL, Bovid K, Alpert H, Chang KW, Quint DJ, Leonard JA Jr et al. Utility of electrodiagnostic testing and computed tomography myelography in preoperative evaluation of neonatal brachial plexus palsy. J Neurosurg Pediatr. 2012;9(3):283-9. doi:10.3171/2011.12.PEDS11416. Biceps needle electromyography at one month of age has been used as part of Leiden´s University algorithm for surgical indication in these patients²⁸28 . Malessy MJ, Pondaag W, Yang LJ, Hofstede-Buitenhuis SM, Cessie S, Dijk JG. Severe obstetric brachial plexus palsies can be identified at one month of age. PLoS One. 2011;6(10):e26193. doi:10.1371/journal.pone.0026193.

Detection of nerve root avulsions is the main indication for image studies²⁹29 . O’Brien DF, Park TS, Noetzel MJ, Weatherly T. Management of birth brachial plexus palsy. Childs Nerv Syst. 2006;22(2):103-12. doi:10.1007/s00381-005-1261-y. The classical finding is the pseudomeningocele, but the correlation of this marker with root avulsion is not perfect³⁰30 . Chow BC, Blaser S, Clarke HM. Predictive value of computed tomographic myelography in obstetrical brachial plexus palsy. Plast Reconstr Surg. 2000;106(5):971-7. doi:10.1097/00006534-200010000-00001. Modern image studies can detect intraspinal nerve root continuity³¹31 . Smith AB, Gupta N, Strober J, Chin C. Magnetic resonance neurography in children with birth-related brachial plexus injury. Pediatr Radiol. 2008;38(2):159-63. doi:10.1007/s00247-007-0665-0. The ideal method for evaluation is still a matter of controversy. Some prefer computed tomography myelogram due to a higher resolution. On the other hand, magnetic resonance image (MRI) is less invasive, allows multiplanar reconstructions, and can evaluate extraspinal lesions. Some authors report similar resolution between these methods. MRI is currently the method of choice in pediatric patients³²32 . Medina LS, Yaylali Y, Zurakowski D, Ruiz J, Altman NR, Grossman JA. Diagnostic performance of MRI and MR myelography in infants with a brachial plexus birth injury. Pediatr Radiol. 2006;36(12):1295-9. doi:10.1007/s00247-006-0321-0.

PROGNOSIS AND SURGICAL INDICATION

Data about NBPP prognosis is surprisingly confuse. The proportion of patients with complete recovery varies among different studies from 7% to 97%³³33 . Adler B, Patterson RL. Erb’s palsy. Long-term results of eighty-eight cases. J Bone Joint Surg A. 1967;49(6):1052-64.,³⁴34 . Nocon JJ, McKenzie DK, Thomas LJ, Hansell RS. Shoulder dystocia: an analysis of risks and obstetric maneuvers. Am J Obstet Gynecol. 1993;168(6 Pt 1):1732-7. doi:10.1016/0002-9378(93)90684-B. Ancient publications had a grim perspective, which were followed by an overly optimistic view³⁵35 . Gordon M, Rich H, Deutschberger J, Green M. The immediate and long term outcome of obstetric trauma. Am J Obstet Gynecol. 1973;117(1):51-6.. Unfortunately, there is no perfect study to address this issue. The ideal design would be a population based on a prospective study, with patients enrolled soon after birth, followed for at least three years with no surgical intervention and with less than 10% of losses, and with a complete and reproducible final evaluation³⁶36 . Pondaag W, Malessy MJ, van Dijk JG, Thomeer RT. Natural history of obstetric brachial plexus palsy: a systematic review. Dev Med Child Neurol. 2004;46(2):138-44. doi:10.1111/j.1469-8749.2004.tb00463.x. Recent studies indicate a more balanced perspective: about 50% of the patients will be completely recovered²2 . Terzis JK, Kokkalis ZT. Pediatric brachial plexus reconstruction. Plast Reconstr Surg. 2009;124:370e-85e. doi:10.1097/PRS.0b013e3181bcf01f,⁵5 . Evans-Jones G, Kay SP, Weindling AM, Cranny G, Ward A, Bradshaw A et al. Congenital brachial palsy: incidence, causes, and outcome in the United Kingdom and Republic of Ireland. Arch Dis Child Fetal Neonatal Ed. 2003;88:f185-9. doi:10.1136/fn.88.3.F185, while about 15% will be severely handicapped. These would be the ideal candidates for surgical intervention. The remaining 35% of the patients will have a satisfactory outcome, but with some shoulder functional limitation³⁷37 . Hale HB, Bae DS, Waters PM. Current concepts in the management of brachial plexus birth palsy. J Hand Surg Am. 2010;35(2):322-31. doi:10.1016/j.jhsa.2009.11.026. External rotation is usually the main problem, and these patients show excessive shoulder abduction (trumpet sign) while attempting to put hand to mouth.

Early surgery provides a larger time window for nerve regeneration and theoretically would have a better outcome. On the other hand, since the rate of spontaneous recovery is high, many children would be submitted to an unnecessary procedure. There is no agreement of which infants should be operated and when it should be done. The most popular criterion was introduced by Gilbert, based on the prognostic studies conducted earlier by Tassin: infants without biceps function at three months of age should be operated⁴4 . Gilbert A, Razaboni R, Amar-Khodja S. Indications and results of brachial plexus surgery in obstetrical palsy. Orthop Clin North Am. 1988;19(1):91-105.. This view has been endorsed by many other nerve surgeons, although some studies have criticized this approach due to a low specificity³⁸38 . Smith NC, Rowan P, Benson LJ, Ezaki M, Carter PR. Neonatal brachial plexus palsy: Outcome of absent biceps function at three months of age. J Bone Joint Surg Am. 2004;86(10):2163-70.. Note that “biceps function” was originally related to biceps palpable contraction, but others use elbow flexion²⁴24 . Clarke HM, Curtis CG. An approach to obstetrical brachial plexus injuries. Hand Clin. 1995;11(4):563-80.,²⁶26 . Heise CO, Siqueira MG, Martins RS, Gherpelli JL. Motor nerve-conduction studies in obstetric brachial plexopathy for a selection of patients with a poor outcome. J Bone Joint Surg Am. 2009;91(7):1729-37.. Electric muscle activity detected by needle electromyography is not considered biceps function.

For patients with total plexus lesions, there is little controversy about the indication of early surgery³⁷37 . Hale HB, Bae DS, Waters PM. Current concepts in the management of brachial plexus birth palsy. J Hand Surg Am. 2010;35(2):322-31. doi:10.1016/j.jhsa.2009.11.026. Some actually prefer to operate earlier than three months, while most wait up to this age due to anesthetic safety²2 . Terzis JK, Kokkalis ZT. Pediatric brachial plexus reconstruction. Plast Reconstr Surg. 2009;124:370e-85e. doi:10.1097/PRS.0b013e3181bcf01f. For patients with C5-C6 or C5-C7 lesions, some surgeons prefer to wait a little longer²⁹29 . O’Brien DF, Park TS, Noetzel MJ, Weatherly T. Management of birth brachial plexus palsy. Childs Nerv Syst. 2006;22(2):103-12. doi:10.1007/s00381-005-1261-y, up to six months of age, which is probably a more cost-effective approach³⁹39 . Brauer CA, Waters PM. An economic analysis of timing of microsurgical reconstruction in brachial plexus birth palsy. J Bone Joint Surg Am. 2007;89(5):970-8. doi:10.2106/JBJS.E.00657. Clinical evaluation should be not only based on elbow flexion, but also include shoulder abduction, elbow extension and wrist and finger extension²⁴24 . Clarke HM, Curtis CG. An approach to obstetrical brachial plexus injuries. Hand Clin. 1995;11(4):563-80.. Surgery after twelve months of age is usually not very effective, although some late selective distal nerve transfers can still offer good results⁴⁰40 . Ruchelsman DE, Pettrone S, Price AE, Grossman JA. Brachial plexus birth palsy: an overview of early treatment considerations. Bull NYU Hosp Jt Dis. 2009;67(1):83-9..

SURGERY

The supraclavicular approach usually provides adequate field for exploration and reconstruction of the brachial plexus structures⁴¹41 . Bahm J, Ocampo-Pavez C, Noaman H. Microsurgical technique in obstetric brachial plexus repair: a personal experience in 200 cases over 10 years. J Brachial Plex Peripher Nerve Inj. 2007;2(1):1. doi:10.1186/1749-7221-2-1. Combined infraclavicular approach through a deltopeitoral incision is rarely necessary in cases of lower trunk lesions, but section of the clavicle is usually not performed. Intraoperative nerve stimulation is crucial for the identification of viable neural structures, but recording of nerve action potentials across the sites of lesions has not been proved to be advantageous in this particular situation¹²12 . Malessy MJ, Pondaag W. Obstetric brachial plexus injuries. Neurosurg Clin N Am. 2009;20(1):1-14. doi:10.1016/j.nec.2008.07.024,⁴⁰40 . Ruchelsman DE, Pettrone S, Price AE, Grossman JA. Brachial plexus birth palsy: an overview of early treatment considerations. Bull NYU Hosp Jt Dis. 2009;67(1):83-9.,⁴²42 . Lin JC, Schwntker-Colizza A, Curtis CG, Clarke HM. Final results of grafting versus neurolysis in obstetrical brachial plexus palsy. Plast Reconstr Surg. 2009;123(3):939-48. doi:10.1097/PRS.0b013e318199f4eb.

The typical lesion found is the neuroma in continuity (Figure 3), in which there is an internal rupture of axons and disorganization of the supporting connective tissue, corresponding to lesions type 3 or 4 in the Sunderland classification⁴³43 . Laurent JP, Lee RT. Birth-related upper brachial plexus injuries in infants: operative and nonoperative approaches. J Child Neurol. 1994;9(2):111-7. doi:10.1177/088307389400900202. This leads to a local proliferation of axonal sprouts and fibroblasts, but very few axons are able to effectively cross the lesion site¹²12 . Malessy MJ, Pondaag W. Obstetric brachial plexus injuries. Neurosurg Clin N Am. 2009;20(1):1-14. doi:10.1016/j.nec.2008.07.024. There are three possible surgical approaches in this situation: external neurolysis, nerve grafting, and nerve transfers²2 . Terzis JK, Kokkalis ZT. Pediatric brachial plexus reconstruction. Plast Reconstr Surg. 2009;124:370e-85e. doi:10.1097/PRS.0b013e3181bcf01f. External neurolysis consists in removing scaring around the nerve. It has not been proved to be an effective isolated procedure⁴²42 . Lin JC, Schwntker-Colizza A, Curtis CG, Clarke HM. Final results of grafting versus neurolysis in obstetrical brachial plexus palsy. Plast Reconstr Surg. 2009;123(3):939-48. doi:10.1097/PRS.0b013e318199f4eb, but it is a necessary step for other reconstructive strategies.

Nerve grafts are used to connect nerve stumps after the removal of the neuroma in continuity⁴²42 . Lin JC, Schwntker-Colizza A, Curtis CG, Clarke HM. Final results of grafting versus neurolysis in obstetrical brachial plexus palsy. Plast Reconstr Surg. 2009;123(3):939-48. doi:10.1097/PRS.0b013e318199f4eb. The sural nerve is usually harvested for this purpose, but other nerves can be used as well⁴⁴44 . Spinner RJ, Kline DG. Surgery for peripheral nerve and brachial plexus injuries or other nerve lesions. Muscle Nerve. 2000;23(5):680-95. doi:10.1002/(SICI)1097-4598(200005)23:5<680::AID-MUS4>3.0.CO;2-H
https://doi.org/10.1002/(SICI)1097-4598(... . The grafts provide a path for nerve regeneration, but clinical results will take many months to appear, since the axonal sprouts will have to grow from the lesion site to the target muscle. After crossing the cooptation site, the axon grows at a rate of 1 to 5 mm/day⁴⁵45 . Robinson LR. Traumatic injury to peripheral nerves. Muscle Nerve. 2000;23(6):863-73. doi:10.1002/(SICI)1097-4598(200006)23:6<863::AID-MUS4>3.0.CO;2-0
https://doi.org/10.1002/(SICI)1097-4598(... .

Nerve transfers were originally developed for nerve repair when a viable proximal nerve stump was not available, such as in cases of root avulsions³⁷37 . Hale HB, Bae DS, Waters PM. Current concepts in the management of brachial plexus birth palsy. J Hand Surg Am. 2010;35(2):322-31. doi:10.1016/j.jhsa.2009.11.026. The donor nerve may be part of the brachial plexus itself (intraplexual transfer) or a nearby nerve outside the plexus (extraplexual transfer)²2 . Terzis JK, Kokkalis ZT. Pediatric brachial plexus reconstruction. Plast Reconstr Surg. 2009;124:370e-85e. doi:10.1097/PRS.0b013e3181bcf01f. Examples of intraplexual transfers include the use of the medial pectoral nerve, transfer from a triceps motor branch to the axillary nerve, or from a fascicle of the ulnar nerve to the biceps motor branch (Oberlin procedure)⁴⁶46 . Siqueira MG, Socolovsky M, Heise CO, Martins RS, Di Masi G. Efficacy and safety of Oberlin’s procedure in the treatment of brachial plexus birth palsy. Neurosurgery. 2012;71(6):1156-60. doi:10.1227/NEU.0b013e318271ee4a. Examples of extraplexual transfers include from the accessory nerve to the suprascapular nerve or from the intercostal nerves to the musculocutaneous nerve (Figure 4)¹²12 . Malessy MJ, Pondaag W. Obstetric brachial plexus injuries. Neurosurg Clin N Am. 2009;20(1):1-14. doi:10.1016/j.nec.2008.07.024,³⁷37 . Hale HB, Bae DS, Waters PM. Current concepts in the management of brachial plexus birth palsy. J Hand Surg Am. 2010;35(2):322-31. doi:10.1016/j.jhsa.2009.11.026. The phrenic nerve is also a possible donor in adults, but is not used in infants. Nerve transfers provide a more distal source of motor axons with a single cooptation site³⁷37 . Hale HB, Bae DS, Waters PM. Current concepts in the management of brachial plexus birth palsy. J Hand Surg Am. 2010;35(2):322-31. doi:10.1016/j.jhsa.2009.11.026. This means that recovery is usually faster and that late procedures can still be effective.

REHABILITATION

Limb immobilization has been associated with shoulder deformities and is not recommended⁴⁷47 . Peixinho M, Serdeira A. Luxação ântero-inferior da articulação do ombro na paralisia obstétrica secundária à imobilização contínua. Rev Hosp Clin Fac Med S Paulo. 1971;26(2):49-54., except if bone fractures are also present. Some advocate that immobilization may be useful for pain treatment during the first week⁴⁸48 . Eng GD, Binder H, Getson P, O’Donnell R. Obstetrical brachial plexus palsy outcome with conservative management. Muscle Nerve1996;19(7):884-91. doi:10.1002/(SICI)1097-4598(199607)19:7<884::AID-MUS11>3.0.CO;2-J
https://doi.org/10.1002/(SICI)1097-4598(... , but it is difficult to evaluate pain in these patients. It seems that NBPP is not painful, at least in older patients⁴⁹49 . Anand P, Birch R. Restoration of sensory function and lack of long-term chronic pain syndromes after brachial plexus injury in human neonates. Brain. 2002;125(1):113-22. doi:10.1093/brain/awf017. This picture is very different from that seen in adults with brachial plexus lesions, who usually show severe neuropathic pain, especially after root avulsions.

Physical therapy and occupational hand therapy are important, but it is essential to involve the parents in the rehabilitation program. Passive range-of-motion exercises are critical to avoid muscle contractures and should be done several times on a daily basis⁵⁰50 . Yang LJ. Neonatal brachial plexus palsy: management and prognostic factors. Semin Perinatol. 2014;38(4):222-34. doi:10.1053/j.semperi.2014.04.009. It is a good idea to include it in other routine activity such as changing dippers. As soon as the child shows intentional voluntary control, it is important to stimulate the affected limb to avoid developmental apraxia¹⁹19 . Dijk JG, Pondaag W, Malessy MJ. Obstetric lesions of brachial plexus. Muscle Nerve. 2001;24(11):1451-61. doi:10.1002/mus.1168,⁴⁸48 . Eng GD, Binder H, Getson P, O’Donnell R. Obstetrical brachial plexus palsy outcome with conservative management. Muscle Nerve1996;19(7):884-91. doi:10.1002/(SICI)1097-4598(199607)19:7<884::AID-MUS11>3.0.CO;2-J
https://doi.org/10.1002/(SICI)1097-4598(... . Encouraging bimanual activities is an interesting strategy for that⁵¹51 . Dumont CE, Forin V, Asfazadourian H, Romana C. Function of the upper limb after surgery for obstetric brachial plexus palsy. J Bone Joint Surg. 2001;83(6):894-900. doi:10.1302/0301-620X.83B6.11389. Wrist splinting can help to enhance hand function in cases of wrist drop⁴⁸48 . Eng GD, Binder H, Getson P, O’Donnell R. Obstetrical brachial plexus palsy outcome with conservative management. Muscle Nerve1996;19(7):884-91. doi:10.1002/(SICI)1097-4598(199607)19:7<884::AID-MUS11>3.0.CO;2-J
https://doi.org/10.1002/(SICI)1097-4598(... , as long as it does not prevent limb use during daytime. Aberrant reinnervation can result in biceps-triceps cocontraction¹⁹19 . Dijk JG, Pondaag W, Malessy MJ. Obstetric lesions of brachial plexus. Muscle Nerve. 2001;24(11):1451-61. doi:10.1002/mus.1168, which can be treated with botulinum toxin⁵²52 . Rollnick JD, Hierner R, Schubert M, Shen ZL, Johannes S, Tröger M et al. Botulinum toxin treatment of cocontractions after birth-related brachial plexus lesions. Neurology. 2000;55(1):112-4. doi:10.1212/WNL.55.1.112. This can also be used to prevent muscle contracture of the shoulder internal rotators⁴⁰40 . Ruchelsman DE, Pettrone S, Price AE, Grossman JA. Brachial plexus birth palsy: an overview of early treatment considerations. Bull NYU Hosp Jt Dis. 2009;67(1):83-9..

LONG TERM COMPLICATIONS

Internal rotation contractures and posterior humeral subluxation are by far the most common long term complication in NBPP²2 . Terzis JK, Kokkalis ZT. Pediatric brachial plexus reconstruction. Plast Reconstr Surg. 2009;124:370e-85e. doi:10.1097/PRS.0b013e3181bcf01f,³⁷37 . Hale HB, Bae DS, Waters PM. Current concepts in the management of brachial plexus birth palsy. J Hand Surg Am. 2010;35(2):322-31. doi:10.1016/j.jhsa.2009.11.026. It is related to muscular imbalance due to poor active external rotation³⁷37 . Hale HB, Bae DS, Waters PM. Current concepts in the management of brachial plexus birth palsy. J Hand Surg Am. 2010;35(2):322-31. doi:10.1016/j.jhsa.2009.11.026,⁴⁰40 . Ruchelsman DE, Pettrone S, Price AE, Grossman JA. Brachial plexus birth palsy: an overview of early treatment considerations. Bull NYU Hosp Jt Dis. 2009;67(1):83-9.. It leads to a progressive shoulder deformity according to the Waters classification, ranging from mild glenoid retroversion (Waters grade II) to a complete posterior luxation with false glenoid and proximal humeral deformity (Waters grade VII)⁵³53 . Waters PM, Smith GR, Jaramillo D. Glenohumeral deformity secondary to brachial plexus birth palsy. J Bone Joint Surg Am. 1998;80(5):668-77.. Early referral to an orthopedic surgeon is important to avoid glenoid dysplasia and possibly shoulder pain³⁷37 . Hale HB, Bae DS, Waters PM. Current concepts in the management of brachial plexus birth palsy. J Hand Surg Am. 2010;35(2):322-31. doi:10.1016/j.jhsa.2009.11.026. Other orthopedic deformities can also be seen, such as scapular winging, elbow flexion contracture, radial head luxation, fixed pronation or supination posture, and claw hand deformity²2 . Terzis JK, Kokkalis ZT. Pediatric brachial plexus reconstruction. Plast Reconstr Surg. 2009;124:370e-85e. doi:10.1097/PRS.0b013e3181bcf01f. Growth imbalance between the upper limbs is common in severe cases of NBPP²2 . Terzis JK, Kokkalis ZT. Pediatric brachial plexus reconstruction. Plast Reconstr Surg. 2009;124:370e-85e. doi:10.1097/PRS.0b013e3181bcf01f.

Little attention has been devoted to sensory disturbances, since the prognosis of sensory deficits is usually good⁴⁹49 . Anand P, Birch R. Restoration of sensory function and lack of long-term chronic pain syndromes after brachial plexus injury in human neonates. Brain. 2002;125(1):113-22. doi:10.1093/brain/awf017. However, some children can develop a self-mutilating biting behavior⁵⁴54 . Al-Qattan MM. Self-mutilation in children with obstetric brachial plexus palsy. J Bone Joint Surg Eur Vol. 1999;24(5):547-9. doi:10.1054/jhsb.1999.0222. This is more common after brachial plexus surgery and is probably related to some kind of uncomfortable paresthesia⁵⁵55 . McCann ME, Waters P, Goumnerova LC, Berde C. Self-mutilation in young children following brachial plexus birth injury. Pain. 2004;110(1-2):123-9. doi:10.1016/j.pain.2004.03.020. This is only temporary and it is crucial to prevent the child from eating off their own fingers and assure the parents that it will pass after a few months.

CONCLUSION

NBPP is a common situation and there is no perspective of adequate prevention in the near future. Most affected newborns will recover spontaneously, but some might be severely handicapped without appropriated care. Early referral to specialized centers with multidisciplinary approach should be provided to all patients that do not recover after a couple of weeks.

References

Publication Dates

History