Transient congenital Horner syndrome and multiple peripheral nerve injury: a scarcely reported combination in birth trauma

Marina Ramos Pérez; Pedro Cabrera Vega; Lourdes Urquía Martí; Fermín García-Muñoz Rodrigo

doi:10.1515/crpm-2021-0025

Article Publicly Available

Transient congenital Horner syndrome and multiple peripheral nerve injury: a scarcely reported combination in birth trauma

Marina Ramos Pérez , Pedro Cabrera Vega , Lourdes Urquía Martí and Fermín García-Muñoz Rodrigo

Published/Copyright: September 21, 2021

Published by

Become an author with De Gruyter Brill

Submit Manuscript Author Information Explore this Subject

From the journal Case Reports in Perinatal Medicine Volume 10 Issue 1

Abstract

Objectives

To describe an infrequent association of multiple injuries in relation to perinatal trauma and the challenge of clinical examination for proper diagnosis.

Case presentation

A male newborn was born at 41 weeks gestational age to a multipara diabetic mother, by forceps. Apgar 8/9. Upon admission, he exhibited axial hypotonia, right brachial paresis, a large left parietal cephalohematoma, but no skull fractures or orbital injuries. He also showed ocular misalignment with marked esotropia of the right eye, ptosis and mild anisocoria, suggesting right Horner syndrome, and left facial palsy. During the oculo-cephalic reflex examination he exhibited a normal adduction and impaired abduction of the right eye. The cranial MRI showed an extensive left parietal cephalohematoma, with internal foci of recent bleeding, and supra and infratentorial laminar subdural hematomas. No lesions were evidenced in the cavernous sinuses. The recovery of extra and intraocular motility at one month of life in our patient highlighted the transitory nature of the lesions and was reassuring for the parents.

Conclusions

This case highlights the importance of a detailed clinical examination in the initial evaluation of a newborn at neurological risk after birth trauma. The association of sixth cranial nerve palsy, Horner syndrome, facial palsy, and brachial plexus injury have been scarcely referred in this context and, if not adequately interpreted, might suggest erroneous diagnoses with very different prognoses.

Keywords: Abducens palsy; birth trauma; Erb palsy; facial palsy; Horner syndrome; newborn

Introduction

Perinatal trauma refers to those injuries produced to the fetus or newborn as a consequence of the mechanical forces of childbirth. They are not always avoidable and, although its incidence is highly variable, among the most common lesions are extra and intracranial hemorrhages, bone lesions, and injuries to the peripheral nervous system, such as facial palsy and brachial plexus injury [1]. Transient sixth nerve palsy in obstetric trauma has been scarcely referred in the literature as an isolated clinical finding [2], or in relation to delivery by forceps [3]. Likewise, in the neonatal period, Horner’s syndrome can be congenital or acquired at birth, in which case it is usually related to brachial plexus injury due to obstetric trauma [4]. The combination of all these injuries, as far as we know, has not been previously reported.

Case presentation

A 41⁺¹ weeks of gestational age male newborn was born to a multipara diabetic mother, after an uneventful pregnancy, by forceps because of prolonged second stage of childbirth. The mother received intrapartum antibiotic prophylaxis due to a positive vaginal exudate for Group B Streptococcus. Time since rupture of membranes was 16 h. The Apgar scores at one and 5 min were eight and nine. The patient was admitted to the Neonatal Unit on the second day of life due to the presence of infectious risk factors and an elevation of acute phase reactants. The initial examination revealed axial hypotonia, no craniofacial dysmorphia or external malformations, a right brachial paresis, and a large left parietal cephalohematoma, but no skull fractures or orbital injuries. He also exhibited ocular misalignment with marked esotropia of the right eye, a decreased right palpebral fissure and mild anisocoria (Figure 1A); facial asymmetry with crying, with less marked left nasolabial fold and incomplete closure of the eye on the same side (Figure 1B), and weak suction during feeds. During the oculo-cephalic reflex examination (Supplementary Video) he exhibited a normal adduction of the right eye along with apparent narrowing of the palpebral fissure (Figure 1C), and impaired abduction with widening of the palpebral fissure during the left rotation of the head (Figure 1D). The intrinsic and extrinsic movement of the left eye, as well as the oculocephalic and pupillary reflexes were normal.

Supplementary Video

Figure 1:

Facial and ocular findings during physical examination. (A) Marked esotropia of the right eye with decreased palpebral fissure. (B) Facial asymmetry with crying. Less marked left nasolabial fold together with incomplete closure of the eyelids on the same side. (C) Oculo-cephalic reflex: Complete adduction of the right eye when turning the head to the right. (D) Absence of abduction of the right eye when turning the head to the left. Informed consent signed by the parents was obtained to publish the non-anonymous photo of their baby.

Work-up included C-reactive protein of 5.5 mg/dL, a lumbar puncture with bloody cerebro-spinal fluid, with 3,280 cells/mm³, 182 leukocytes (75% mononuclear and 25% polynuclear). The cranial ultrasound and Magnetic Resonance Imaging showed an extensive left parietal cephalohematoma (14 mm in transverse diameter), with internal foci of recent bleeding and supra and infratentorial laminar subdural hematomas (<2 mm). No lesions in the cavernous sinuses were identified. He received antibiotic therapy for 5 days until negative bacteriological results were confirmed, and the acute phase reactants normalized. He was discharged on the ninth day of life, having partially recovered the facial paresis, but the paralysis of the right sixth cranial nerve persisted. In the outpatient follow-up visit at one month of age, there was still a slight facial asymmetry with a smaller right palpebral fissure and a slight anisocoria, but normal ocular alignment and oculo-cephalic reflex. The ophthalmological examination at two months showed normal fundus and normal intrinsic and extrinsic ocular motility.

Discussion

The findings in this patient suggested, initially, a differential diagnosis between isolated right sixth cranial nerve palsy and type I Duane syndrome. Congenital sixth nerve palsy has been reported both after spontaneous and after forceps delivery [2, 3]. Most cases resolve spontaneously in a few weeks. In our case, the apparent retraction of the eye globe during adduction led us to suspect a type I Duane syndrome, which is due to the congenital absence of the sixth cranial nerve, together with paradoxical innervation of the lateral rectus muscle by third nerve fibers. This entity frequently associates other anomalies and syndromes, being a permanent alteration [5]. The recovery of ocular motility at one month of life in our patient finally ruled out this diagnosis. On the other hand, the evident reduction of the palpebral fissure on initial examination, suggested the possibility of a diagnosis of Horner syndrome. Congenital Horner syndrome is usually secondary to perinatal trauma, after forceps delivery, vacuum extraction, shoulder dystocia, fetal rotation and/or manipulation of the extremities and is usually associated with brachial plexus injury [4]. Attenuated light examination may be a determinant factor to detect anisocoria secondary to the paresis of the iris dilator muscle since, due to the integrity of the parasympathetic innervation, the reaction to light and accommodation are not altered [6]. Apart from ptosis and miosis, the third classic sign of Horner’s triad is ipsilateral anhidrosis. This sign is present when the involvement of the sympathetic pathway is preganglionic but does not appear if the involvement is postganglionic.

In conclusion, this case highlights the importance of a detailed clinical examination in the initial evaluation of the newborn with neurological risk. Some of the findings presented, or their combination, have been scarcely reported in the context of obstetric trauma [7] and, if not adequately interpreted, they may suggest erroneous diagnoses, with very different prognoses. In addition to the clinical examination, the appropriate choice of complementary tests and their timing are essential for quality clinical care.

Corresponding author: Fermín García-Muñoz Rodrigo, Neonatal Unit, Hospital Universitario Materno Infantil de Canarias, Avenida Marítima del Sur S/N, 35016, Las Palmas de Gran Canaria, Las Palmas, Spain, Phone: +34680406241, Fax: +34928444694, E-mail: fgarciamu@gmail.com

Research funding: None declared.
Author contributions: All authors have accepted responsibility for the entire content of this manuscript and approved its submission.
Competing interests: Authors state no conflict of interest.
Informed consent: Informed consent was obtained from the parents of the patient. Informed consent signed by the parents was obtained to publish the non-anonymous photo of their baby.
Ethical approval: The local Institutional Review Board deemed the study exempt from review.

References

1. Volpe, JJ. Injuries of extracranial, cranial, intracranial, spinal cord, and peripheral nervous system structures. In: Volpe JJ, Inder TE, Darras BT, de Vries LS, du Plessis AJ, Neil JJ, Perlman JM, editors Volpe’s Neurology of the Newborn, 6th ed. Elsevier; 2018. 1093–123 pp. https://doi.org/10.1016/b978-0-323-42876-7.00036-3.Search in Google Scholar

2. Reisner, SH, Perlman, M, Ben-Tovim, N, Dubrawski, C. Transient lateral rectus muscle paresis in the newborn infant. J Pediatr 1971;78:461–5. https://doi.org/10.1016/s0022-3476(71)80228-7.Search in Google Scholar PubMed

3. de Grauw, AJ, Rotteveel, JJ, Cruysberg, JR. Transient sixth cranial nerve paralysis in the newborn infant. Neuropediatrics 1983;14:164–5. https://doi.org/10.1055/s-2008-1059571.Search in Google Scholar PubMed

4. Alfonso, I, Alfonso, DT, Papazian, O. Focal upper extremity neuropathy in neonates. Semin Pediatr Neurol 2000;7:4–14. https://doi.org/10.1016/s1071-9091(00)80005-4.Search in Google Scholar PubMed

5. Kekunnaya, R, Negalur, M. Duane retraction syndrome: causes, effects and management strategies. Clin Ophthalmol 2017;11:1917–30. https://doi.org/10.2147/opth.s127481.Search in Google Scholar

6. Maloney, WF, Younge, BR, Moyer, NJ. Evaluation of the causes and accuracy of pharmacologic localisation in Horner’s syndrome. Am J Ophthalmol 1980;90:394–402. https://doi.org/10.1016/s0002-9394(14)74924-4.Search in Google Scholar PubMed

7. Sauber-Schatz, EK, Markovic, N, Weiss, HB, Bodnar, LM, Wilson, JW, Pearlman, MD. Descriptive epidemiology of birth trauma in the United States in 2003. Paediatr Perinat Epidemiol 2010;24:116–24. https://doi.org/10.1111/j.1365-3016.2009.01077.x.Search in Google Scholar PubMed

Supplementary Material

The online version of this article offers supplementary material (https://doi.org/10.1515/crpm-2021-0025).

Received: 2021-03-22

Accepted: 2021-09-01

Published Online: 2021-09-21

Articles in the same Issue

https://doi.org/10.1515/crpm-2021-0025

Keywords for this article

Abducens palsy; birth trauma; Erb palsy; facial palsy; Horner syndrome; newborn