In infants and children, cervical masses originate from a variety of pathologic, congenital and developmental conditions, and it is important to distinguish benign from serious lesions. Fibromatosis colli is a rare cervical lesion that typically presents in the neonatal period. It is the most common cause of neonatal torticollis and should be recognized by physicians caring for neonates and infants.
The most common cause of pediatric torticollis is fibromatosis colli, which is the result of fibrous proliferation and replacement of the sternocleidomastoid muscle. The condition occurs in 0.4 percent of all infants and generally appears between two and four weeks of age. The right and left sides are involved equally, and there is no gender predilection.[3,4] Recognized risk factors for the development of fibromatosis colli are breech delivery, primiparous birth and instrumented extraction.
A healthy male infant was referred to the otolaryngology-head and neck surgery clinic at 22 days of age for evaluation of a left-sided neck mass. He was the product of an uncomplicated, term, vertex delivery by a healthy 23-year-old primiparous woman. Although labor was prolonged, no induction agents were used and instrumented extraction was not necessary. Physical findings were normal, and the infant was discharged from the newborn nursery on his second day of life.
When the infant was 18 days of age, his parents noted the development of a nontender, left-sided neck mass (Figure 1). Mild right-sided torticollis and head tilt appeared simultaneously. The infant was seen by his primary physician and subsequently referred to the clinic for evaluation and management.
Physical examination revealed a firm, spindle-shaped mass within the left sternocleidomastoid muscle. The mass measured 2.5 cm in greatest diameter and extended the entire length of the muscle. It was most prominent with active rotation of the head to the right side, and it was completely nontender to palpation and manipulation. The remainder of the physical examination was unremarkable; no adenopathy, airway obstruction or additional neck masses were found. The condition was diagnosed as fibromatosis colli, and no additional studies were obtained.
The infant was referred to the physical therapy department, and passive stretching exercises were started immediately. The parents were advised about the condition and the necessity for intensive physical therapy as the initial management. They were cautioned to avoid prolonged supine positioning and were instructed to alternate the infant's sleeping position often.
The infant was observed regularly and showed complete resolution of the torticollis after three weeks of gentle stretching exercises. The sternocleidomastoid mass slowly resolved over the subsequent six months. By seven months of age, the infant had no residual torticollis, neck mass or craniofacial asymmetry.
The pathogenesis of fibromatosis colli is unclear. The condition was once attributed to trauma at parturition, but this theory has been abandoned. The fibrous tissue found in neonates with fibromatosis colli is remarkably mature, which suggests that the process begins in utero. In light of this, most authorities believe the condition is likely to be the cause, rather than the result, of breech presentation and difficult delivery.
As in the illustrative case, fibromatosis colli generally presents as a firm, nontender, fusiform enlargement within the sternocleidomastoid muscle. It usually appears between two and four weeks of age. Most often, the fibrous process extends throughout the mass of the muscle from mastoid to clavicle. The tumor generally increases in size for two to four weeks after presentation, remains constant for several months and then begins to slowly resolve. At least 50 percent of patients have complete resolution of the mass within six to eight months of onset. An additional 30 percent have slower resolution or insignificant residual enlargement of the involved muscle. Less than 20 percent of patients require surgical intervention.[4,9,l0] In contrast, nonoperative management after the age of one year is rarely successful.
Diagnosis is made by a thorough history and physical examination. It is essential to localize the mass within the sternocleidomastoid muscle and to be certain that it is not separate from or anterior to this structure. The differential diagnosis includes branchial cleft cyst, cervical lymphadenopathy, lymphosarcoma, rhabdomyosarcoma and fibrosarcoma. The diagnosis of fibromatosis colli can usually be made without fine-needle aspiration, biopsy or imaging studies, although these measures may be necessary in atypical or questionable cases.
Care must be taken to avoid gravitational distortion of the moldable infant cranium. If the child is allowed to maintain a supine position with the head rotated, gravitational forces may lead to acranial distortion known as plagiocephaly. In this type of craniofacial asymmetry, the affected side of the face is flattened and the ipsilateral ear situated posteriorly. This causes widening of the ipsilateral side of the face and contralateral facial hemihypoplasia.
The initial treatment is close observation and gentle passive stretching of the affected muscle. A physical therapist, especially a pediatric therapist, may offer valuable assistance in initiating these activities and educating the parents in proper stretching techniques. To avoid plagiocephaly, the child must sleep on each side alternately, instead of supine or prone. Surgical intervention becomes necessary in refractory cases with the development of facial hemihypoplasia, plagiocephaly or airway obstruction, or in the child greater than one year of age.
As in the illustrative case, the condition is generally self-limited. More than c,O percent of cases respond to conservative management. Initial surgical intervention in refractory cases involves open tenotomy and lengthening of the affected muscle. If this measure fails, excision of the entire musculofibrous mass may be required. Studies have shown excellent resolution of both the torticollis and the craniofacial abnormalities after surgical correction of the affected sternocleidomastoid muscle.
[1.] Bredenkamp JK, Hoover LA, Berke GS, Shaw A. Congenital muscular torticollis. A spectrum of disease. Arch Otolaryngol Head Neck Surg 1990; 116: 212-6. [2.] Coventry MB, Harris LE. Congenital muscular torticollis in infancy. J Bone Joint Surg Am 1959; 41: 815. [3.] Morrison DL, MacEwen GD. Congenital muscular torticollis: observations regarding clinical findings, associated conditions, and results of treatment. J Pediatr Orthop 1982; 2: 500-5. [4.] Binder H, Eng GD, Gaiser JF, Koch B. Congenital muscular torticollis: results of conservative management with long-term follow-up in 85 cases. Arch Phys Med Rehabil 1987; 68: 222-5. [5.] Ling CM, Low YS. Sternomastoid tumor and muscular torticollis. J Bone Joint Surg Br 1973; 55: 236. [6.] Ling CM, Low YS. Sternomastoid tumor and muscular torticollis. Clin Orthop 1972;86:144-50. [7.] Dunn PM. Congenital sternomastoid torticollis: an intrauterine postural deformity. J Bone Joint Surg Br 1973; 55: 877. [8.] Tom LW, Rossiter JL, Sutton LN, Davidson RS, Potsic WP. Torticollis in children. Otolaryngol Head Neck Surg 1991; 105: 1-5. [9.] Jones PG. Torticollis in infancy and childhood. Springfield, Ill.: Charles C. Thomas, 1968. [10.] May M. Neck masses in children: diagnosis and treatment. Ear Nose Throat J 1978;57:136-58. [11.] Porter SB, Blout BW. Pseudotumor of infancy and congenital muscular torticollis. Am Fam Physician 1995; 52: 1731-6.
WILLIAM R. BLYTHE M.D. is a resident in the Division of Otolaryngology-Head and Neck Surgery at the University of North Carolina School of Medicine, Chapel Hill. He received his medical degree from the University of Alabama School of Medicine, Birmingham.
THOMAS C. LOGAN, M.D. is a resident in the Division of Otolaryngology-Head and Neck Surgery at the University of North Carolina School of Medicine, where he also earned his medical degree.
DOUGLAS K. HOLMES, M.D. is an assistant professor of surgery/otolaryngology at the University of North Carolina School of Medicine and chief of the Division of Otolaryngology at Wake AHEC Medical Education Institute, Raleigh, N.C. He is a graduate of the University of North Carolina School of Medicine.
AMELIA E DRAKE, M.D. is an associate professor of surgery/otolaryngology, associate professor of pediatrics and chief of the pediatric otolaryngology section at the University of North Carolina School of Medicine, where she also received her medical degree.
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