Release: May 12, 2000
Contact: Kenneth Satterfield, 407-238-4161 (as of 5/12/2000)
MAYO CLINIC RESEARCHERS CHART TREATMENT COURSE FOR CONGENITAL CONDITION AFFECTING NECK MOVEMENT
Pseudotumor of infancy and congenital muscular torticollis are rare and benign conditions. A new research study highlights differences in diagnosis and treatment for the two medical disorders
Orlando, FL -- Pseudotumor of infancy (POI), also known as sternocleidomastoid tumor of infancy or fibromatosis colli, is reported to occur in 0.4 percent of all newborns. These children typically display a palpable, firm, fibrous, nontender mass before six weeks of age. This mass may increase in size, but the majority of patients experience complete resolution without intervention and after physical therapy.
Congenital muscular torticollis (CMT) is the third most common congenital musculoskeletal anomaly following dislocation of the hip and clubfoot. The majority of young patients with this disorder also experience complete resolution after conservative therapy. However, those with persistent head tilt who are not treated may incur long-term consequences resulting in facial asymmetry and plagiocephaly (skull asymmetry secondardary to asymmetrical closure of the cranial sutures). This can cause not only cosmetic but possibly functional abnormalities.
Both of these conditions are most often first noted by a pediatrician or family physician. The otolaryngologist may evaluate the infant or child for neck mass, unilateral contracture of the sternocleidomastoid muscle (SCM) muscle, or both. Caused by, or as a result of, fibrotic change of the SCM muscle, the unilateral contracture may subsequently result in plagiocephaly or facial hemi-hypoplasia. Torticollis, or 'wryneck," has been recognized for centuries, with the term derived from the Latin words "tortus," meaning twisted, and "collum," meaning neck. Contracture of the SCM causes the head to be turned towards the affected side and the chin to be rotated and pointed to the opposite side.
Surprisingly , there is no consensus in the medical community for labeling a patient with POI versus CMT. It is unknown whether POI and CMT are two distinct pathologic entities or if they compose a spectrum of the same muscular abnormality. Speculations on the pathogenesis for both include intrauterine mechanical factors, birth trauma, and anoxic injury to the SCM muscle, all which can lead to subsequent muscle fibrosis.
This lack of differentiation in diagnosing these two disorders brought together a medical research team from Rochester, MN, to review the clinical presentation, management, and long-term follow-up of a large series of patients with POI or CMT, and recommend evaluation and follow-up for both conditions.
The authors of the study, "Pseudotumor of Infancy and Congenital Muscular Torticollis : A Review of 170 Cases Evaluation, Management, and Long-Term Follow-up," are Julie L. Wei, MD, and Laura J. Orvidas, MD, Department of Otorhinolaryngology, and Amy L. Weaver, M.S., Department of Biostatistics, all at the Mayo Clinic; and Kara M. Schwartz, B.S., Mayo Medical School, Rochester, MN. Their findings were presented before the annual meeting of the American Society of Pediatric Otolaryngologists, being held in May 17-18, in Orlando, FL.
Methodology: The Mayo-Rochester Surgical Information Recording System identified 201 patients diagnosed with pseudotumor of infancy (POI) and ccongenital muscular torticollis (CMT) between January 1, 1962 and February, 1998. Only patients diagnosed prior to age 24 months were included in the study, to better define the condition as "congenital."
Children diagnosed at six weeks of age or less were considered to have POI, and those diagnosed between six weeks and 24 months, CMT. Retrospective chart reviews were performed on all 170 patients, with phone surveys performed on patients without evidence of disease resolution. Study data included age at diagnosis, gender, presenting symptoms, method of evaluation, imaging studies, specialty referral(s), treatment method, subsequent visit information, surgical treatment(s), and final outcome (total resolution, subtotal resolution, or long-term cosmetic or functional deformity). Phone survey included data specific to final outcome. Only 11 patients (six percent) were lost to follow-up.
Results: A total of 170 patients, 81 males and 89 females, were included. Thirty-eight patients were diagnosed prior to six weeks of age and considered to have POI, while the remaining 132 (78%) were diagnosed after six weeks of age and considered CMT patients. The mean age of diagnosis was four months.
Key findings included:
In 54.1 percent of the patients, the left side of neck was affected. Over 90 percent of patients in both groups presented with head tilt, with plagiocephaly present in 64 percent of CMT patients and 40 percent of POI patients.
Sixty-three percent of patients with POI and 18 percent of patients with CMT presented with a neck mass. Facial asymmetry was present in 7.9 percent of POI patients and 10.6 percent of CMT patients, and only 2.4 percent of the entire group experienced feeding difficulty as a result of the diagnosis. The most common associated anomoly was congenital hip displasia found in ten percent of the subjects.
Muscle release was the most common surgical treatment when patients failed conservative therapy (8.8 percent), with 0.6 percent of patients requiring a second surgery. Follow-up data was available on 159 patients; 85.5 percent experienced total resolution and 14.5 percent experienced subtotal resolution or definite long-term abnormality.
Conclusions: Newborns and infants diagnosed with pseudotumor of infancy (POI) or congenital muscular torticollis (CMT) should be treated and followed for at least 12 months or until resolution of symptoms. Patients with symptoms persisting one year after diagnosis despite conservative therapy should be considered for surgical treatment. Surgical treatment should not be considered unless the child is 12-18 months.The majority of children with either POI or CMT experience total resolution of symptoms. The long-term consequences rarely result in functional problems, but facial or skull asymmetry may persist from the time of diagnosis.
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