Laryngeal Palsy in Association with Myelomeningocele, Hydrocephalus, and the Arnold-Chiari Malformation* W. M. KIRSCH, M.D., B. R. DUNCAN, M.D., F. O. BLACK, M.D., AND J. C. STEARS,M.D. Divisions of Neurosurgery and Otolaryngology, and Departments of Pediatrics and Radiology, University of Colorado Medical Center, Denver, Colorado THOUGH disturbances of laryngeal motor teristic of the Arnold-Chiari malformation function in infants and children with (Fig. 1). When the child was 2 weeks old, a congenital hindbrain malformations ventriculogram (Fig. 1) showed significant have been described and recognized in both supratentorial hydrocephalus with the medul- the otolaryngological and pediatric litera- la, cerebellar tonsils, and caudal fourth ture, 4,~,22,25,26 these problems have received ventricle herniated caudally to the C-2 arch. comparatively scant neurosurgical attention. Functional assessment of the aqueduct and of In this report, we present five cases in which the cisterns was not made. mild to severe laryngeal motor deficits oc- Operation. A ventriculoatrial shunt (Pu- curred in association with the Arnold-Chiari denz) was inserted. Immediately after this malformation, myelomeningocele, and aque- operation stridor disappeared, but 8 days ductal stenosis in varying combinations. We later the shunt was not functioning and the have reviewed the cases from the standpoint fontanelle again became tense. Severe stridor of the pathological physiology of abductor returned and laryngoscopy demonstrated bi- vocal cord paralysis, as well as the clinical lateral abductor paralysis of the vocal cords. aspects pertinent to the management of this The shunt was revised and a clot found in the perplexing, and at times, life-threatening de- cranial limb of the Pudenz valve. After the rangement. shunt revision, stridor subsided, and the my- elomeningocele was closed when the child was Case Reports 6 weeks old. Case 1. A 2-day-old baby, the product of a The child's subsequent course has been free full-term uncomplicated pregnancy and de- of laryngeal symptomatology, and the vocal livery, was transferred to the University of cords have been observed to move normally. Colorado Medical Center because of a leaking At age 20 months, the shunt was again revised myelomeningocele in the lumbosacral area. because of an abnormal progression of head Examination. On admission the child was circumference, with a subsequent regression alert, had spontaneous movements at the to a normal rate of head enlargement. At age hips, but showed a sensory and motor loss be- 30 months, the valve is functioning well, but low the L-3 spinal segmental level, as well as intellectual performance indicates mild re- urinary and fecal incontinence. The myelo- tardation in both language development and meningocele sac intermittently drained clear motor skills. cerebrospinal fluid. During the initial 12 days of hospitalization Case 2. A 2-day-old boy, born after a full- the head circumference increased 3 cm, the term pregnancy complicated by persistent anterior fontanelle became tense, and marked vomiting and severe peripheral edema, was stridor with suprasternal retraction super- transferred to the University of Colorado vened. Radiological examination demon- Medical Center because of a large lumbosaeral strated a lacunar skull, and a caudally coned myelomeningocele measuring 8 • 10 cm, as foramen magnum which may be a charac- well as bilateral club feet. Examination. The infant was noted to have a superficially infected myelomeningocele sac Received for publication July 6, 1967. (nonhemolytic staphylococcus), flaccid paral- * Research supported from grants for the U.S. Pub- lic Health Service, Otolaryngology Training Grant ysis of both legs, absent sensation below the 1TO1NB5504-01, and the National Foundation Birth T-12 dermatomal level, urinary and fecal in- Defects Treatment Center. continence, bilateral club feet, and dislocated 207 208 W.M. Kirsch, B. R. Duncan, F. O. Black and J. C. Stears Fie. 1. Case 1. Transcoronal pneumoencephalogram (ventriculogram), demonstrating the transforaminal fourth ventricle and the inferiorly coned lateral margins of the foramen magnum, features of the Arnold-Chiari malformation. Also present is a functional semi-obstruction of the aqueduct. hips. The myelomeningocele sac was treated Case 3. At the age of 13, an otherwise nor- with local application of ]% silver nitrate mally developing girl began to complain of dressings, and cultures from the surface of the suboccipital headache and paresthesias in her sac were sterile within 24 hours. finger tips. These symptoms became progres- The head circumference increased abnor- sively more severe over a 2-year period, and at mally during the first 3 weeks of life. A ventric- the age of 15 she developed truncal ataxia, ulogram at 3 weeks demonstrated marked dysphagia, and hoarseness. supratentorial hydrocephalus, total occlusion Examination. The patient was first seen at of an undeformed and midline rostral aque- the University of Colorado Medical Center at duct, and foramen magnum caudal coning the age of 15 years. On physical examination suggesting the presence of an Arnold-Chiari she was alert and cooperative, but had a nasal, malformation. No soft tissue evaluation of hoarse voice, difficulty in clearing pharyngeal the posterior fossa or cisterns could be made. secretions, decreased palatal movements, and Operation. A ventriculoatrial shunt (Holter, a markedly reduced gag reflex. Unfortunately, "normal pressure") was inserted. On the 20th direct laryngoscopy was not performed in this postoperative day, marked inspiratory stridor case. The fundi were normal except for healed developed in association with an abnormal chorioretinitis in the left eye, There was gen- increase in head circumference (2.5 cm in 10 eralized weakness in the arms as well as bi- days). Direct laryngoscopy demonstrated ab- lateral extensor plantar responses and marked ductor paralysis of the vocal cords. The re- disequilibration on attempted tandem gait. A spiratory distress became so severe that tra- cine-esophagram demonstrated a delayed cheostomy was necessary. The ventriculoatrial initiated swallowing, feeble disorganized shunt was revised and the Holter valve was pharyngo-esophageal peristalsis, and laryngo- found to operate at a pressure of 120 mm of tracheal aspiration with no cough reflex. A water instead of an expected pressure of ap- Tensilon test was normal. X-rays demon- proximately 70 mm of water. After the re- strated skeletal and soft tissue craniovertebral vision the head circumference decreased and anomalies with mild dorso-rostral basilar in- at the age of 4 months the myelomeningocele vagination, and minimal symmetrical supra- was closed. At the age of 5 months, laryngos- tentorial hydrocephalus; the left cerebellar copy revealed normal movement of the vocal tonsil extended down to the level of C-2. The cords, and the tracheostomy was gradually cisterns were patent, and the functional level closed over a 9-day period. At the age of 12 of the obstruction in the CSF pathway was not months, the patient is free of stridor. proven. .