Develop. Med. Child Neurol. 1982, 24. 202-218 Review Article Robert N. Hensinger Eric T. Jones Developmental Orthopaedics. 11: The Spine, Trauma and Infection Torticollis Congenital muscular torticollis is Torticollis, or wryneck, is a common believed to result from local trauma to the clinical sign in a wide variety of childhood soft tissues of the neck during delivery. illnesses. When recognized at or soon after Birth records of these children birth, the usual cause is congenital demonstrate a preponderance of breech or muscular torticollis. However, difficult forceps deliveries, or primiparous roentgenograms of the cervical spine births', '. A common misconception is that should be obtained to exclude other less the neck is contused during delivery and common congenital conditions, such as the the resultant hematoma leads to fibrosis fixed or bony torticollis associated with and contracture. However, experimental Klippel-Feil syndrome and/or anomalies of the atlanto-axial articulation (Table I). TABLE I Congenital muscular torticollis is Differential diagnosis of torticollis usually discovered in the first six to eight weeks of life. If the infant is examined Congenital Congenital muscular torticollis within the first month of life, commonly a Klippel-Feil syndrome mass or 'tumor' is palpable in the neck' Basilar impressions Atlanto-occipital fusion (Fig. 1). Generally there is a non-tender, Pterygium colli (skin webs) soft enlargement which is mobile beneath Odontoid anomalies the skin and attached to or located within Neurological the body of the sternocleidomastoid Ocular dysfunction muscle. The mass obtains maximum size Syringomyelia Spinal-cord or cerebellar tumors (posterior within the first month and then gradually fossa) regresses. If the child is examined at about Bulbar palsies four to six months of age the tumor Inflammatoi:,. usually will have disappeared and the only Lymphadenitis of the neck clinical findings are the contracture of the Spontaneous hyperemic atlanto-axial rotary subluxation sternocleidomastoid muscle and torticollis posture-head titled towards the involved Trauma Fractures. subluxation, dislocations of the side and chin rotated towards the opposite cervical spine (particularly CI-2) shoulder2* (Fig. 2). Correspondence to Robert N. Hensinger, M.D.. Associate Professor OfOrthopaedicSurgery, University of Michigan, University Hospital, Ann Arbor, Michigan 48109. 20 2 REVIEW ARTICLE work suggests that the fibrosis within the particularly asymmetrical findings such as muscle is due to venous occlusion from atrophy and hemihypertrophy, should be pressure on the neck in the birth canal” ’. suspected of having an underlying The clinical deformity is probably related vertebral deformity. to the ratio of fibrosis to remaining The young child with congenital functional muscle after the initial insult2.If there is sufficient normal muscle it will stretch with growth and the child will not develop the torticollis posture. The fibrotic area, however, has little elastic potential. Further support for intra-uterine molding is provided by additional factors: in three out of four children the lesions are on the right side,’. and 20 per cent of affected children have congenital dysplasia of the hip6. All children with torticollis should be evaluated with roentgenograms to exclude bony abnormality or fracture. Congenital muscular torticollis is painless and is associated with a contracted or shortened sternocleidomastoid muscle, and it is not accompanied by bony abnormalities or neurological deficit. Fig. 1. Six-week-old infant with swelling in region of sternocleidomastoid muscle. Congenital scoliosis Although congenital scoliosis seldom poses a serious problem for the neonate, early recognition of a vertebral anomaly is important. If not controlled, it can quickly lead to significant deformity, both cosmetic and life-threatening’ (Fig. 3a). In the neonate, there may be no visible curvature to suggest a vertebral anomaly, but there are other clinical and roentgenographic findings which should alert the examiner to such a possibility. Children with cutaneous abnormalities Tn the region of the back-dermal sinuses, hairy patches, dimples and hemangioma- should be carefully evaluated for the presence of associated anomalies of the cervical spine (Klippel-Feil), bony torticollis, congenital heart-disease ( 15 per Fig. 2. Six-month-old infant with right-sided cent), and Sprengel’s deformity8. Children congenital muscular torticollis, Note rotation of skull, asymmetry and flattening of face, with ‘Ongenital problems Of the lower depression adjacent to left eye (on side of extremities, foot deformities, and contracted sternocleidomastoid muscle). 203 DEVELOPMENTAL ORTHOPAEDICS. 11: THE SPINE, TRAUMA AND INFECTION scoliosis will seldom have a neurological It is presumed that the clinical problem, un1es.s there is associated consequences are due to the ‘tethering’ diastematomyelia, myelodysplasia or effect of the diastematomyelia on the sacral agenesis. Affected children have a 20 normal ascent of the spinal cord. The per cent incidence of associated anomalies diastematomyelia may act as a ‘check-rein’ of the kidney and bladder, which may only on the upward migration of the neural be discovered on intravenous elements, with progressive neurological pyelography’. This study should be routine signs in the lower extremities. It should be for all children who have vertebral emphasized that the greatest change in the anomalies (Fig. 3b). vertebral architecture occurs in the last six months of intra-uterine skeletal growth: Diastematomyelia differential growth during childhood is at a Diastematomyelia is an uncommon slower pace, with more time for adaptive congenital disturbance of vertebral changes in the spinal cord and nerve architecture in which the neural elements, roots’” 14. the spinal cord or intraspinal nerve-roots The majority of patients have a are split into two columns by a midline cutaneous abnormality in the midline of mass in the spinal canal, fixed anteriorly to the back’’, I5-l7 which can be an important the vertebral body. The mass may be an clue to the diagnosis. The form of the osseous or cartilaginous spicule of a cutaneous defect is extremely varied: a fibrous septum partially or completely patch of hair, a discreet dimple, dividing the neural canal. Diastema- subcutaneous fatty tumor, pigmented nevi, tomyelia may be localized at one vertebral and hemangiomas have all been level or extended over several segments, reportedl6-I9. Although infrequent, the and is commonly found in the lumbar association of a dermal sinus is of Fig. 3a. Newborn with multiple congenital Fig. 3b. Same infant as in Fig. 3a. Intravenous anomalies of the spine. Routine roentgenography pyelogram demonstrates absent right kidney. should include (left) antero-posterior and (rixht) lateral views of spine. 204 REVIEW ARTICLE particular concern and must be carefully erythematous, and the child may have low- searched for, as it may represent a portal grade fever22. The clinical and for entry of bacteria into the spinal canal2’. radiographic appearance is similar to bone The orifice may be quite small- and or joint infection, and the two problems innocuous in appearance, yet lead to are often confused, particularly in the child abscess formation and meningitis, or a with an epiphyseal separation. frank neural cutaneous fistula2’. Similarly, the incidental discovery of such a defect Cervical spine injuries in neonates should make one suspect diastema- The most commonly reported spine tornyelial6. injuries in the neonatal period are those that involve the cervical spinal cord. Fractures in neonates Cervical injury appears to be uncommon, The majority of fractures occur at the or at least not commonly recognized, and -time of delivery and $re either epiphyseal occurs principally in two areas- separations of the humerus or femur, or proximally near the occiput (the C1-2 midshaft fractures of the long bones, most articulation) and distally neal: the thorax commonly the clavicle2’322. The few studies (C6-7 or C7-T1)22. However, there is reported in recent times suggest that the seldom roentgenographic evidence of a over-all incidence of fracture is bony injury26*27.The neonatal spine is uncommon, occurring in less than 1 per more elastic than the spinal cord itself: cent of newborn infants2’. The vast Towbin noted that traction could lengthen majority are clavicular fractures, of which the newborn spine by two inches, but the only a small percentage are recognized2’. spinal cord by only one-quarter inch2’. Similarly, many epiphyseal separations are Thus, stretching forces can cause a only slightly displaced and may be complete transection of the spinal cord undetected22. In contrast, midshaft without roentgenographic evidence of fractures of the long bones generally are bony disruption. recognized immediately, often by the Studies of stillborn infants demonstrate obstetrician at the precise moment they that the maximum angulation of the occur23*24. vertebral column occurs at the As might be expected, injury to a bone or cervicothoracic junction, but without joint occurs more often with complicated vertebral disruption. Consequently, if a obstetrical procedures or breech deliveries dislocation or fracture of the cervical (6 per cent), and is rare in spontaneous vertebrae is discovered, it suggests a severe Injury is more common among transection-type injury to the spinal cord22. first-born infants, but with the exception of C1-2 injuries