Baeteman C, Denis D, Loudot C, Toesca E, Bronsard J, Benso C, Fogliarini C, Hadjadj E, Antoniotti S, Chabrol B, Mancini J, Girard N.
J Fr Ophtalmol. 2008 Mar;31(3):287-94.
INTRODUCTION: Primary exotropia is a divergent strabismus that appears from the first day of life to the second year. It can be isolated or associated with a pathological context requiring cerebral imaging to determine diagnosis and prognosis. The objective of this study was to report the sensorimotor state and the result of MRI in infantile exotropia. PATIENTS AND METHOD: Forty-seven children with primary exotropia had a complete ophthalmologic assessment (visual acuity, binocular vision, refraction with cycloplegia, eye fundus) and cerebral MRI (average age, 16 months). Deviation was classified into four categories<4 degrees, > or =4<10 degrees, > or =10<20 degrees, > or =20 degrees. MRI results were classified into seven categories: white matter abnormalities (gliosis, delay of maturation, periventricular leukomalacia, aspecific hyperintense signal, and necroses); Virchow-Robin enlargement space and enlarged subarachnoid space; gray matter abnormalities (necroses, cerebral atrophy, occipital cortex, basal ganglia); ventriculomegaly; thin corpus callosum; cerebellar injury; and tumor. RESULTS: There was a statistically significant increase in the rate of pathological MRI as the angle deviation increased: 76.6% of patients had a pathological ophthalmologic exam (amblyopia, ptosis, head posture, Duane's syndrome, cataract, albinism, or pigmentary retinopathy). We found 38% nystagmus and 38% optic nerve hypoplasia. There was a real pathological context in 61.7% of the exotropia cases: 27.7% prematurity, 31.9% fetal distress, 21.3% facial dysmorphy (plagiocephaly), 12.8% psychomotor delay, and 14.9% epilepsy. Only three children had isolated exotropia. Thirty-four cerebral MRI (72.3%) were not normal. In pathological MRI, there was 61.8% white matter injury, 41.2% gray matter injury, and 17.6% thin corpus callosum. CONCLUSION: This study demonstrates the fundamental contribution of cerebral MRI in infantile exotropia. The greater the deviation, the more abnormal the MRI results are. The pathological context and ophthalmological abnormalities are important in infantile exotropia.
Torticollis, facial asymmetry and plagiocephaly in normal newborns.
Stellwagen L, Hubbard E, Chambers C, Jones KL.
Arch Dis Child. 2008 Apr 1 [Epub ahead of print]
OBJECTIVE: To evaluate the incidence and characteristics of torticollis, plagiocephaly, and facial asymmetry in normal newborn infants. DESIGN: One hundred and two healthy newborn infants were examined prospectively during their birth hospitalization for torticollis with neck range of motion (ROM) assessment, and for facial, mandibular, and cranial asymmetry by photographic analysis. RESULTS: Seventy-three percent of newborns had one or more asymmetry: torticollis (16%), asymmetry of the mandible (13%), facial asymmetry (42%), and asymmetry of the head (61%). Torticollis was associated with maternal report of having been "stuck" in one intrauterine position for more than 6 weeks prior to delivery. Moderate facial asymmetry was associated with a longer second stage of labor, forceps delivery, a bigger baby, and birth trauma. Moderate cranial and mandibular asymmetries were associated with birth trauma. Ten percent of newborns had more than one significant asymmetry. CONCLUSIONS: Asymmetries of the head and neck are very common in normal newborns, and sixteen (16%) of 102 study newborns were found to have torticollis. Such newborns, especially if they sleep supine, are thought to be at risk of developing deformational posterior plagiocephaly. Identification of affected infants may allow early implementation of positioning recommendations or physical therapy to prevent the secondary craniofacial deformations that are part of an increasingly common phenomenon.
MOC-PS(SM) CME article: management considerations in the treatment of craniosynostosis.
Persing JA.
Plast Reconstr Surg. 2008 Apr;121(4 Suppl):1-11.
LEARNING OBJECTIVES: After studying this article, the participant should be able to: 1. Recognize the anatomical characteristics of the individual forms of craniosynostosis. 2. Differentiate deformational plagiocephaly from craniosynostosis. 3. Identify the pros and cons of individual types of craniosynostosis operations (i.e., endoscopic craniectomy and open cranioplasty procedures). 4. Understand risks of operative procedure versus the natural history of untreated craniosynostosis. SUMMARY: The term "craniosynostosis" refers to the premature fusion of one or more cranial vault sutures and its associated skull deformities. The pattern of skull deformities for each of the sutures (metopic, coronal, sagittal, and lambdoid) is characteristic from patient to patient, although variable in the degree of severity. The skull pattern is predictable in that restriction of growth occurs in a plane perpendicular to the plane of the fused vault suture, and compensatory changes occur frequently, parallel to it. When skull deformities are recognized with midfacial and extremity deformities, often the craniosynostosis is referred to as syndromic, implying a genetic basis for the skeletal maldevelopment. Indications for surgery relate to benefits in changing the shape of the skull toward normal and potentially avoiding brain maldevelopment. Brain injury is presumed to be related to local or regional increases in intracranial pressure. A broad range of surgical options to treat craniosynostosis exist, from strip craniectomy to comprehensive, or whole vault, cranioplasty. The optimal surgical timing for these approaches must balance both the desire for early intervention to reduce the effects of bone restriction on brain growth and the ability of a child to withstand the rigors of surgery. Complications of surgery include blood loss, bone defects, and scalp scarring. The most common, significant, postoperative complication, however, is incomplete correction of the skull deformity. Infection in nonsyndromic synostosis surgery is rare. Team management and longitudinal follow-through will aid in determining efficacy and safety of the available surgical approaches.
Head shape measurement standards and cranial orthoses in the treatment of infants with deformational plagiocephaly: a systematic review.
McGarry A, Dixon MT, Greig RJ, Hamilton DR, Sexton S, Smart H.
Dev Med Child Neurol. 2008 Mar 18 [Epub ahead of print]
This review aims to determine how head shape is measured and describes the use of orthoses in the management of deformational plagiocephaly. A systematic review was conducted and papers published in English up to and including 2006 were sourced from nine databases. After initial screening, 20 papers were included; three literature reviews and 17 original papers. Of the original papers, eight concerned the method of head shape measurement. Measurements are important in determining clinical classification and treatment modality of deformational plagiocephaly. All studies were appraised and assigned a level of evidence according to the Scottish Intercollegiate Guidelines Network. Methodological quality was inadequate. Publications involving the use of cranial orthoses used convenience samples, were not blinded, and used different measurement techniques. No comparison groups were included and participants were not randomized. Evidence suggests that conservative treatments might reduce skull deformity although the quality is poor. Clinical studies investigating the use of cranial orthoses reported beneficial effects. Further research is required to identify the efficacy of cranial orthoses in the treatment of deformational plagiocephaly based on a standardized measurement technique to facilitate classification of this condition.
Neurologic Findings in Infants With Deformational Plagiocephaly.
Fowler EA, Becker DB, Pilgram TK, Noetzel M, Epstein J, Kane AA.
J Child Neurol. 2008 Mar 19 [Epub ahead of print]
This study evaluates the neurologic profiles of infants with deformational plagiocephaly. Forty-nine infants with deformational plagiocephaly between the ages of 4 and 13 months (mean age, 8.1 months) are evaluated, along with 50 age-matched control subjects (mean age, 8.1 months). A modified version of the Hammersmith infant neurologic assessment was performed on each infant. A caregiver completed a questionnaire regarding the infant's prematurity, development, and health to date. Results are analyzed using t test. There is a statistically significant difference in overall neurologic assessment scores of infants with deformational plagiocephaly vs their healthy peers (P = .002). This difference is predominately in tone, whereby infants with deformational plagiocephaly have significantly more abnormal tone than nonplagiocephalic infants (P = .003). This abnormality is not one of decreased tone but one of variable tone, deflecting abnormally high and low tone. Infants with deformational plagiocephaly are more likely to have altered tone but not exclusively decreased tone.
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