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Ophthalmologic findings in patients with nonsyndromic plagiocephaly.

The purpose of this article was to study the prevalence of strabismus and astigmatism in children with nonsyndromic plagiocephaly. Children with deformational plagiocephaly do not have an increased prevalence of strabismus compared with the general population but do have an increased prevalence of astigmatism, whereas children with nonsyndromic craniosynostotic plagiocephaly have an increased prevalence of strabismus and astigmatism. This observational cohort study retrospectively reviewed patients in a multidisciplinary craniofacial clinic between 1993 and 2001. Patients were referred for evaluation and management of plagiocephaly or craniosynostosis. One hundred eleven patients had deformational plagiocephaly, and 45 had synostotic plagiocephaly. Patients with lambdoid craniosynostosis, hemifacial microsomia, any syndromic form of craniosynostosis, or without a formal diagnosis were excluded. All patients underwent detailed ophthalmologic, ocular motility, and craniofacial examinations. Diagnosis was based on clinical and radiographic findings. Patients were evaluated between birth and age 14 years (mean = 13 months; SD = 22 months). Only 1 of 111 (<1%) patients with deformational plagiocephaly had an esodeviation, and none had an exodeviation. Three of 45 patients (7%) with synostotic plagiocephaly had an exodeviation, and none had an esodeviation. Eight of 93 patients (9%) with deformational plagiocephaly had unilateral astigmatism, and 14 (15%) had bilateral astigmatism (mean = 1.82 diopters). Three of 43 patients (7%) with synostotic plagiocephaly had unilateral astigmatism, and 9 (21%) had bilateral astigmatism (mean = 1.83 diopters). Patients with deformational plagiocephaly do not appear to have a higher prevalence of horizontal strabismus than the normal population. Exotropia is more common in patients with nonsyndromic craniosynostotic plagiocephaly than in the general population. The prevalence of astigmatism in patients with nonsyndromic craniosynostotic plagiocephaly appears to be greater than in the general population, but this higher prevalence requires further study.

Adolescent↗

Plagiocephaly: differential diagnosis based on endocranial morphology.

Plagiocephaly is a descriptive term that connotes an asymmetrically oblique or twisted head. Such cranial dysmorphology has a number of etiologies, the most common of which are unicoronal synostosis, unilambdoid synostosis, and plagiocephaly without synostosis. Use of the term plagiocephaly in the literature is often ambiguous in that at times it is used inclusively for all etiologies while at other times it is used exclusively as a synonym for unicoronal synostosis. Although differentiation by physical examination among unicoronal synostosis, unilambdoid synostosis, and plagiocephaly without synostosis usually is possible for an experienced observer, inexperienced observers often have difficulty making an anatomically accurate diagnosis even with the assistance of conventional skull radiographs. High-resolution CT scans, including three-dimensional osseous surface re-formations, have become a standard element in the evaluation of craniofacial anomalies in many centers. We hypothesized that the three major etiologies of plagiocephaly could be unambiguously differentiated by means of endocranial three-dimensional CT osseous surface re-formations. Archival pretreatment CT data on 15 unicoronal synostosis, 4 unilambdoid synostosis, and 15 plagiocephaly without synostosis patients were reviewed to define, qualitatively and quantitatively, the characteristics of the endocranial base morphologies for each group; in addition to visual dysmorphology specific to each group, there was a statistically significant difference in the angle of deviation from the midlines of the anterior and posterior cranial fossae among unicoronal synostosis, unilambdoid synostosis, and plagiocephaly without synostosis. Four radiologists experienced in reading images of craniofacial anomalies were oriented to the group characteristics and then instructed to perform differential diagnosis for each of the 34 patients using only the endocranial three-dimensional CT images. The raters were blind to all other clinical and diagnostic information. The raters correctly diagnosed unicoronal synostosis. Errors were made in differentiation of unilambdoid synostosis and plagiocephaly without synostosis. These errors resulted from the raters' reliance on image inspection rather than quantitation of anteroposterior fossae midline angulation. Such quantitation unambiguously differentiated between unilambdoid synostosis and plagiocephaly without synostosis in the "error" cases. The endocranial base dysmorphology of patients with plagiocephaly is etiology-specific for unicoronal synostosis, unilambdoid synostosis, and plagiocephaly without synostosis. Three-dimensional CT endocranial base images can assist differential diagnosis of plagiocephaly.

Cephalometry↗

Palpatory diagnosis of plagiocephaly.

INTRODUCTION: The term plagiocephaly, from the Greek plagios (oblique) and kephalê (head), means distortion of the head, and refers clinically to cranial asymmetry. Cranial Osteopathy, since it was first proposed, has focussed upon the diagnosis and treatment of birth trauma and cranial asymmetries, and consequently specific therapy for plagiocephalic deformities has been described. Osteopathic manipulation also has been proposed as a treatment for torticollis, a condition associated with plagiocephaly. For these reasons, we decided to look at the mechanics of the occipital bone and the adjacent atlas and bones of the cranial base, in relation to functional plagiocephaly. METHODS: The records of 649 children seen in an osteopathic practice in Lyon, France, were reviewed retrospectively, in compliance with the legal requirements of the Commission Nationale de l'Informatique et des Libertés (CRIL) and the Helsinki accord, for gender, age at presentation, birth history, obstetrical data (breech presentation, vacuum extraction, forceps delivery or Caesarean section), presenting complaint, side of posterior plagiocephaly, side of frontal plagiocephaly, torticollis, motion pattern of the occipital bone upon the atlas, and motion pattern of the spheno-occipital synchondrosis. RESULTS: We found significant correlations between plagiocephaly (right/left) and primipara (P=0.024), use of forceps (P=0.055) and extractor suction (P=0.055). Correlations were also found between flattening of the occiput (right/left) and lateral strain of the spheno-occipital synchondrosis (P=0.002) and between plagiocephaly (right/left) and occipito-atlantal motion (P=0.000). CONCLUSION: We found a significant correlation between the lateral strain pattern of the spheno-occipital synchondrosis and plagiocephaly and between rotational dysfunction of the occiput upon the atlas and the side of posterior plagiocephaly. We suggest that thorough neonatal osteopathic examination can identify individuals predisposed to develop posterior plagiocephaly.

Atlanto-Occipital Joint↗

Plagiocephaly: morphometry of skull base asymmetry.

The aim of this study was to perform a morphometric analysis of the skull base to understand the mechanism of asymmetry between malformational and deformational plagiocephaly. Since 1988, we have carried out 102 CT scans with three-dimensional reconstruction of the endocranial side of the skull base. There were 20 cases of malformational plagiocephaly, consisting of 18 unicoronal synostoses (UCS) and two unilambdoid synostoses (ULS), and 82 cases of plagiocephaly without synostosis, made up of 60 cases of frontal plagiocephaly (FP) and 22 of occipital plagiocephaly (OP). The skull base asymmetry was analyzed in three ways: (1) the hemibases and the cranial fossa angles were compared with each other; (2) the structural asymmetry of the chondrocranium was calculated; and (3) the architectural asymmetry was measured in relation to the medio-labyrinthine plane. The skull base asymmetry of malformational plagiocephaly was located in the anterior and middle cranial fossae for UCS and in the posterior and middle cranial fossae for ULS. The asymmetry was the result of a localized structural anomaly of the chondrocranium. The asymmetry of deformational plagiocephaly corresponded to a distortion in relation to the medio-labyrinthine plane while the chondrocranium remained symmetric. The skull base asymmetry of malformational plagiocephaly is secondary to a localized malformation of the chondrocranium, and that of the deformational plagiocephaly is secondary to an architectural deformation.

Analysis of Variance↗

Occipital plagiocephaly.

The diagnosis of occipital plagiocephaly has remained a complex and controversial issue in the field of craniofacial surgery. Over the past 30 years, numerous studies have been published describing the management and treatment for 'posterior plagiocephaly', 'plagiocephaly without synostosis', 'deformational plagiocephaly' and 'occipital plagiocephaly', with surgical 'correction' being chosen as the primary modality of treatment irrespective of the patency status of the lambdoid sutures. Two hundred and four patients with unilateral occipital plagiocephaly have been seen at the Australian Craniofacial Unit over the past 16 years. Each patient was evaluated by a craniofacial surgeon, paediatric neurosurgeon and paediatric geneticist. All children underwent plain radiographs of the skull to define the sutural anatomy. In those patients where the sutural anatomy was equivocal, 2-D and 3-D CT scans were performed. Only two of the 204 patients (approximately 1%) manifested the clinical, radiographic and pathological features of true unilambdoid synostosis. There was radiographic evidence of sutural fusion on plain films, 2-D and 3-D CT scans. Pathology specimens showed bony sutural fusion. Two hundred and two patients presented with unilateral occipital deformities and patent sutures on radiography. These patients with occipital plagiocephaly in the absence of true synostosis were initially managed conservatively (head positioning, and physiotherapy in those patients with torticollis). Those patients who underwent surgical correction in infancy (21/204) included patients with severe plagiocephaly not responding to conservative therapy (19/204) and the two patients with true unilambdoid synostosis (2/204).One hundred and ninety-one of the total patients (94%) were noted by their parents to have acceptable improvement in their head shape. Thirteen patients were seen within the past year and are too early to assess. Two surgical patients (one fronto-orbital advancement, one occipital craniectomy) and one patient followed conservatively were judged by their parents to be without notable improvement. In our series it is apparent that the majority of cases of occipital plagiocephaly are not secondary to true synostosis and can be managed by conservative positional measures.

Cranial Sutures↗

A classification of plagiocephaly utilizing a three-dimensional computer analysis of cranial base landmarks.

Plagiocephaly is a term commonly used to describe congenital forehead asymmetry. Previous classification systems based on the various etiologies of dysmorphic crania have been used in an effort to categorize the patients into groups and to assist in treatment planning. The system most commonly used today was described by Bruneteau and Mulliken in 1992. The authors separated frontal plagiocephaly into three types: synostotic, compensational, and deformational. The present study was undertaken in order to define a simple system for classifying plagiocephaly based on Bruneteau and Mulliken's system using the patients' preoperative craniofacial computed tomography scans. The involvement of the entire coronal ring in synostotic plagiocephaly led to the choice of 20 skull base landmarks as the basis of the analysis. Nine lateral landmarks (the superior orbital fissure, the optic foramen, the zygomatic arch, the greater palatine foramen, the foramen ovale, the mastoid tip, the hypoglossal canal, the external auditory canal, and the internal auditory canal) and two midline landmarks (the crista galli and the internal occipital protuberance) were used. The changes that occurred in these landmarks were analyzed in 30 patients. The results demonstrated that Bruneteau and Mulliken's classification system underestimated the number of different subtypes of plagiocephaly. As a result, three major types of frontal plagiocephaly and several different subtypes based on the different etiologies were described. Type I plagiocephaly includes plagiocephaly resulting from cranial suture synostosis. Type II includes those with a nonsynostotic etiology. Type III describes patients with craniofacial microsomia-associated plagiocephaly. Statistical analysis was unavailable because of the small number of patients in each subtype. With a larger number of patients, we hope to refine this system for use by the surgeon in preoperative diagnosis and surgical planning. The analysis is unique in its ability to quantitate changes from normal on the x-, y-, and z-coordinates, and therefore allows for identification of both horizontal (frontal bone deviation) and vertical (ear shear) growth disturbances.

Facial Asymmetry↗

Analysis of posterior plagiocephaly: deformational versus synostotic.

Appropriate management of posterior plagiocephaly requires differentiation of occipitoparietal flattening caused by lambdoid synostosis from that caused by deformational forces. In a 2 1/2-year prospective study of 115 infants presenting with unilateral posterior cranial flattening, only one child had synostotic posterior plagiocephaly (lambdoid synostosis), whereas 114 infants had deformational posterior plagiocephaly. Deformational occipitoparietal flattening was more common on the right (61 percent) than on the left (30 percent), and minor contralateral frontal flattening was not unusual (52 percent). The ipsilateral ear was anteriorly displaced in virtually all infants (97 percent). Some infants had ipsilateral torticollis (19 percent); a few had contralateral torticollis (8 percent). Gender ratio was 3:1, male:female. A total of 114 infants with deformational posterior plagiocephaly were treated conservatively either by head positioning in the crib (n = 63) or with a molding helmet (n = 51). Outcome was assessed by pretreatment and posttreatment anthropometry on 53 of these infants, who were either positioned (n = 17) or helmeted (n = 36). Improvement occurred in 52 of 53 patients (mean follow-up 4.6 months), i.e., the difference in length between the long and short transcranial axis diminished in 52 infants (mean 1.2 to 0.7 cm), did not progress in any child, and was unchanged in one infant. At an average age of 10 months, posterior cranial symmetry was better in infants treated with a helmet (mean difference 0.6 cm) than in those managed by positioning (mean difference 1.0 cm) (p < 0.001). Age at initiation of helmet therapy (from 2 to 9 months) was unrelated to rate of improvement. In a 10-year retrospective study, the authors identified 12 infants who had an operation for posterior plagiocephaly. All but one had confirmed premature lambdoid fusion; thus, this condition accounted for 3.4 percent of all primary operations performed for craniosynostosis during this decade (n = 323). In retrospect, the physical findings of synostotic posterior plagiocephaly were not clearly different from those of deformational posterior plagiocephaly. Plain radiography was sometimes used to confirm the clinical diagnosis. Neither sutural narrowing, deep interdigitations, nor perisutural sclerosis indicated lambdoid synostosis. Computed tomography (CT) was necessary if the physical findings were suspicious for lambdoid synostosis or if plain films did not give a definitive diagnosis. Axial CT scans (n = 7) showed a symmetric forehead in all but one patient with lambdoid synostosis. CT studies also demonstrated that auricular position was indeterminate in synostotic posterior plagiocephaly, being anterior, posterior, or symmetric, whereas the ipsilateral ear was virtually always anterior in deformational posterior plagiocephaly.

Craniosynostoses↗

Frontal plagiocephaly: synostotic, compensational, or deformational.

Plagiocephaly is a term commonly used to describe congenital forehead asymmetry. Sixty patients with frontal plagiocephaly were evaluated retrospectively and separated into three types: synostotic (N = 24), compensational (N = 3), and deformational (N = 33). Categorization of frontal plagiocephaly as synostotic or deformational was reliably made by physical examination, focusing on the supraorbital rims, nasal root, ears, and malar eminences. Other anatomic parameters useful in the differential diagnosis included chin point, palpebral fissures, and facial height. This study documented that birth histories were similar for synostotic and deformational frontal plagiocephalic infants. However, other deformational anomalies were more common in deformational frontal plagiocephalic infants, whereas malformations had an equal incidence in deformational and synostotic frontal plagiocephalic infants. Torticollis was an associated finding in 64 percent of infants with deformational frontal plagiocephaly; almost all were ipsilateral. In contrast, head tilt, usually to the contralateral side, was noted in 14 percent of patients with synostotic frontal plagiocephaly. Female preponderance was noted in both synostotic (79 percent) and deformational (76 percent) frontal plagiocephaly. Left-sided involvement was seen in 73 percent of patients with deformational frontal plagiocephaly and in 46 percent of patients with synostotic frontal plagiocephaly. Premature pelvic descent, in the left occipital anterior position, may account for the high incidence of left-sided deformational plagiocephaly and ipsilateral torticollis.

Abnormalities, Multiple↗

Nonsurgical, nonorthotic treatment of occipital plagiocephaly: what is the natural history of the misshapen neonatal head?

Plagiocephaly has recently received increased attention in pediatric neurosurgical circles and its treatment has been controversial. Lack of uniformity persists in the treatment of neonates with occipital plagiocephaly. Plagiocephaly resulting from synostosis is rare. Many patients with nonsynostotic plagiocephaly have been treated surgically. Cranial remodeling orthotic devices (such as headbands and helmets) have also been reported to correct nonsynostotic plagiocephaly. The cost of orthotic treatment has risen and its validity has been contested by many third-party insurance payers. The effectiveness of orthotic treatment has not been adequately compared to the natural history of nonsynostotic plagiocephaly. The first phase of a natural history study was initiated in June 1995 at Phoenix Children's Hospital. All new patients referred with a diagnosis of plagiocephaly were categorized into two groups: those with mild-to-moderate asymmetry and those with moderate-to-severe asymmetry. Categories were determined by cephalic measurements. The patients with moderate-to-severe asymmetry were offered orthotic treatment with a cranial remodeling band. Those patients with mild-to-moderate asymmetry were observed with repeated measurements. Data in patients left untreated and followed for natural history were compared to our data published in 1994 for those treated with external cranial orthosis. Our published data in 47 patients treated with a headband during the period from 1988 to 1993 indicated that cranial vault asymmetry improved from a mean of 8.9 mm to 4 mm over an average treatment period of 4.3 months beginning when the average age of the patient was 5.9 months. Seventy-two neonates treated with neck stretching exercises and repositioning of the head without surgery or orthotic devices showed improvement in cranial vault asymmetry from 10.6 mm to 5.5 mm over an average treatment period of 4.5 months beginning when the average age of the patient was 4.5 months. This study indicates that neonates with mild-to-moderate asymmetry who are less than 6 months of age and who are treated with physiotherapy and repositioning have similar improvement in cranial vault asymmetry as those treated with orthotic devices. The second phase of our study deals with neonates who have moderate-to-severe plagiocephaly.

Journal Article↗

Update on craniofacial surgery: the differential diagnosis of lambdoid synostosis/posterior plagiocephaly.

There has been an evolution of thought on the diagnosis and treatment of posterior plagiocephaly. Synostotic posterior plagiocephaly (lambdoid synostosis) can be diagnosed and differentiated from non-synostotic posterior plagiocephaly (positional molding) based on specific cosmetic and radiologic criteria. The advent of high-resolution three-dimensional CT analysis of cranial morphology combined with meticulous clinical studies of the two major causes of posterior plagiocephaly has added much to our understanding of this skull deformity. Children with lambdoid synostosis have a trapezoid-shaped skull with posterior displacement of their ipsilateral ear, ipsilateral mastoid bossing, contralateral occipital bossing, and a fused lambdoid suture that appears as a ridge. Children with posterior plagiocephaly without lambdoid synostosis have a characteristic parallelogram-shaped skull with anterior displacement of the ipsilateral ear and ipsilateral frontal bossing. This subject is surrounded in controversy because many of the children in the past thought to have lambdoid synostosis probably did not, based in part on the aforementioned specific criteria. This is an important point, as most patients with posterior plagiocephaly without synostosis will improve without surgery. One should thus be appropriately conservative in the selection of patients for surgery. The majority of infants evaluated at craniofacial clinics presumably have posterior plagiocephaly without synostosis and can be successfully treated with frequent head turning, helmet, or band therapy. Patients with clinically and radiologically proven synostotic posterior plagiocephaly and a severe deformity should undergo craniofacial surgery. The technique of biparieto-occipital craniotomy is safe, simple, and delivers a good cosmetic result. Our postoperative photographic evaluations have shown an immediate, aesthetically pleasing change in the contour of the occiput, which tends to improve with time.

Craniofacial Abnormalities↗

Neurodevelopmental delays in children with deformational plagiocephaly.

BACKGROUND: The purpose of this study was to determine whether, in fact, infants with deformational plagiocephaly, or plagiocephaly without synostosis, demonstrated cognitive and psychomotor developmental delays when compared with a standardized population. Through this study, we chose to expand upon our earlier findings from 2001 on patients with deformational plagiocephaly. METHODS: The study population includes a total of 110 consecutive patients, prospectively followed then retrospectively reviewed. Each infant was assessed using the Bayley Scales of Infant Development-II scoring system. The developmental analysis was categorized as either mental or psychomotor using the mental developmental index or the psychomotor developmental index, respectively. These infants were subcategorized into four groups: accelerated, normal, mild, or severely delayed. The groups were then compared with a standardized Bayley's age-matched population, using chi-square test goodness-of-fit tests. RESULTS: Infants with deformational plagiocephaly were found to have significantly different psychomotor development indexes and mental developmental indexes when compared with the standardized population (p < 0.0001; p < 0.0001). With regards to the mental developmental index scores, none of the infants with deformational plagiocephaly were accelerated, 90 percent were normal, 7 percent were mildly delayed, and 3 percent were severely delayed. With regards to the psychomotor development index scores, none of infants were accelerated, 74 percent were normal, 19 percent were mildly delayed, and 7 percent were severely delayed. CONCLUSIONS: This study indicates that before any intervention, infants with deformational plagiocephaly show significant delays in both mental and psychomotor development. Also of particular note is that no child with deformational plagiocephaly showed accelerated development.

Alcohol Drinking↗

Nonsynostotic occipital plagiocephaly: radiographic diagnosis of the "sticky suture".

BACKGROUND: While the clinical differences between nonsynostotic occipital plagiocephaly and lambdoid craniosynostosis have been described, the radiographic differentiation between the two remains obscure. The aim of this study was to characterize morphological differences in the lambdoid suture between nonsynostotic occipital plagiocephaly and lambdoid craniosynostosis. METHODS: Computed tomography scans of children clinically diagnosed with nonsynostotic occipital plagiocephaly (n = 26) were compared with computed tomography scans from children diagnosed with lambdoid craniosynostosis (n = 7). Suture and cranial morphology, ear position, and endocranial base angles were qualitatively and quantitatively compared. RESULTS: Nonsynostotic occipital plagiocephaly sutures demonstrated areas of focal fusion (25 percent), endocranial ridging (78 percent), narrowing (59 percent), sclerosis (19 percent), and changes from overlapping to end-to-end orientation (100 percent). No sutures demonstrated ectocranial ridging. All cases of nonsynostotic occipital plagiocephaly presented with ipsilateral occipital flattening, 85 percent with ipsilateral frontal, and 95 percent with contralateral occipital bossing producing parallelogram morphology. In contrast, a greater frequency of sutures in lambdoid craniosynostosis patients demonstrated nearly complete obliteration (p < 0.001) with ectocranial ridging (p < 0.001); significantly more of these patients presented with ipsilateral occipital flattening with compensatory ipsilateral mastoid (p < 0.001) and contralateral parietal (p < 0.01) bossing, producing a trapezoid morphology. Sutures from nonsynostotic occipital plagiocephaly patients showed endocranial ridging, focal fusions, and narrowing, previously reported as lambdoid craniosynostosis. CONCLUSIONS: In contradiction to previous reports, lambdoid craniosynostosis is not radiographically unique among suture fusions. This work establishes the radiographic diagnosis of nonsynostotic occipital plagiocephaly.

Child, Preschool↗

Multiple-birth infants at higher risk for development of deformational plagiocephaly.

OBJECTIVE: Deformational plagiocephaly refers to the development of an abnormal head shape in infants resulting from externally applied molding forces, which may occur either prenatally or postnatally. We have observed that an unexpectedly high number of multiple-birth infants have presented to our center with this condition. The purposes of this investigation were to: 1) determine the significance of this observation; and 2) examine the risk factors that may make this population more susceptible to the development of plagiocephaly. MATERIALS AND METHODS: A retrospective review of our database was performed to identify those infants who were of multiple-birth origin. The parents of these infants were contacted by phone to complete a survey regarding the prenatal and postnatal history of their child. Similar information was obtained for the state of Arizona from the Office of Vital Statistics. A chi2 analysis was used to compare the incidence of multiple births in Arizona with the incidence of multiple births in our treatment population. RESULTS: Between 1993 and 1996, 69 (8.6%) of the 801 infants treated for deformational plagiocephaly at our Phoenix center were of multiple-birth origin. Four infants who had been treated postoperatively after surgery for craniosynostosis, as well as 5 patients who had been referred from out of state, were excluded from further study. The chi2 analysis of the remaining 60 patients confirmed that a statistically significant number of plural-birth infants had presented with deformational plagiocephaly. Four risk factors were identified as having occurred at high frequency in this population: in utero constraint, supine sleeping position, torticollis, and prematurity. CONCLUSIONS: The current findings of this investigation confirm that a significant number of multiple-birth infants have presented to our clinic with deformational plagiocephaly. Compared with their singleton counterparts, plural infants seem to be at higher risk for the development of deformational plagiocephaly, because they are more likely to be exposed to multiple risk factors.deformational plagiocephaly, multiple birth, plurality.

Female↗

Long-term developmental outcomes in patients with deformational plagiocephaly.

OBJECTIVES: To determine whether there was an increased rate of later developmental delay in school-aged children who presented as infants with deformational plagiocephaly without obvious signs of delay at the time of initial evaluation. METHODS: A retrospective medical record review of 254 patients evaluated at the Craniofacial Center of the Children's Hospital and Regional Medical Center in Seattle, Washington, from 1980 through 1991 was completed. Consenting patient families were interviewed via telephone to determine what, if any, special medical or educational problems had occurred for the children who had had plagiocephaly in infancy or their siblings with normal head shapes. RESULTS: A total of 181 families from the medical record review could be notified about the study and 63 families agreed to participate in a telephone interview. The sample of participants for the telephone interview was random to and representative of the group as a whole. The families reported that 25 of the 63 children (39.7%) with persistent deformational plagiocephaly had received special help in primary school including: special education assistance, physical therapy, occupational therapy, speech therapy generally through an Individual Education Plan. Only 7 of 91 siblings (7.7%), serving as controls, required similar services (chi(2) = 21.24). Delays could not be specifically anticipated at the time of the diagnosis of deformational plagiocephaly from any simple set of factors including treatment with helmet therapy, although effected males with reported uterine constraint were at the highest risk for subsequent school problems. CONCLUSIONS: Infants with deformational plagiocephaly comprise a high-risk group for developmental difficulties presenting as subtle problems of cerebral dysfunction during the school-age years. There is a need for additional research on the long-term developmental problems in infants with deformational plagiocephaly. plagiocephaly, facial asymmetry, torticollis, developmental delay.

Developmental Disabilities↗

[Plagiocephaly in children: etiology, differential diagnosis and helmet treatment].

Plagiocephaly in a head-and-neck irradiated rat model or rhomboid-shaped head, occurs in at least 1 in 300 live births. In most cases such asymmetry is not caused by synostosis of the unilateral coronal or lambdoid sutures, but is rather a deformity produced by intrauterine and/or postnatal deformational forces. Categorization and diagnosis of plagiocephaly as synostotic or deformational is reliably made by physical examination and computerized tomography. Its differential diagnosis is extremely important because prompt surgical correction is usually indicated for the synostotic type. In contrast, infants with deformational frontal or occipital plagiocephaly generally respond to helmet treatment. 10 infants with significant deformational plagiocephaly were treated with individual plastic helmets during the past 2 years and 4 other infants with plagiocephaly are currently being treated. In each instance, cranial asymmetry dramatically improved as the brain grew and the head filled out the helmet. There were no significant complications. Awareness of deformational plagiocephaly allows more accurate diagnosis and appropriate treatment, avoiding unnecessary surgical intervention in patients with positional molding.

Head Protective Devices↗

Association between "plagiocephaly" and hemifacial microsomia.

Fifteen of 155 patients with hemifacial microsomia were noted to have frontal plagiocephaly. These patients were examined to determine whether the frontal flattening was either secondary to deformation, the result of unilateral coronal synostosis, or part of the spectrum of hemifacial microsomia. The patients were categorized as having deformational versus synostotic frontal plagiocephaly by documenting position of the supraorbital rims, nasal root, ears, malar eminences, chin point, and the palpebral fissure height. Other extracraniofacial anomalies were also noted. Fourteen of 15 (93%) patients had characteristic deformational abnormalities. Only 1/15 (7%) had an elevated orbit, suggestive of unilateral coronal synostosis, but this diagnosis was not radiographically confirmed. Frontal deformational plagiocephaly was ipsilateral to the side predominantly affected by hemifacial microsomia in all but one patient. Patients with hemifacial microsomia-deformational frontal plagiocephaly often had ipsilateral torticollis, cervical spine abnormalities, and anomalies outside the craniofacial region. This was in contrast to patients with deformational frontal plagiocephaly, in the absence of hemifacial microsomia, who frequently had ipsilateral torticollis but no other anomalies. This study also underscores possible confusion in differentiating hemifacial microsomia from deformational hemifacial hypoplasia on physical examination. The association of deformational frontal plagiocephaly and hemifacial microsomia belies a rigid etiologic label of deformational versus malformative anomaly.

Adult↗

The differential diagnosis of posterior plagiocephaly: true lambdoid synostosis versus positional molding.

The diagnosis and treatment of posterior plagiocephaly is one of the most controversial aspects of craniofacial surgery. The features of true lambdoid synostosis versus those of deformational plagiocephaly secondary to positional molding are inadequately described in the literature and poorly understood. This has resulted in many infants in several craniofacial centers across the United States undergoing major intracranial procedures for non-synostotic plagiocephaly. The purpose of this study was to describe the detailed clinical, imaging, and operative features of true lambdoid synostosis and contrast them with the features of positional plagiocephaly. During a 4-year period from 1991 to 1994, 102 patients with posterior plagiocephaly were assessed in a large multidisciplinary craniofacial program. During the same period, 130 patients with craniosynostosis received surgical treatment. All patients were examined by a pediatric dysmorphologist, craniofacial surgeon, and pediatric neurosurgeon. Diagnostic imaging was performed where indicated. Patients diagnosed with lambdoid synostosis and severe and progressive positional molding underwent surgical correction using standard craniofacial techniques. Only 4 patients manifested the clinical, imaging, and operative features of unilambdoid synostosis, giving an incidence among all cases of craniosynostosis of 3.1 percent. Only 3 among the 98 patients with positional molding required surgical intervention. All the patients with unilambdoid synostosis had a thick ridge over the fused suture, identical to that found in other forms of craniosynostosis, with compensatory contralateral parietal and frontal bossing and an ipsilateral occipitomastoid bulge. The skull base had an ipsilateral inferior tilt, with a corresponding inferior and posterior displacement of the ipsilateral ear. These characteristics were completely opposite to the findings in the 98 patients who had positional molding with open lambdoid sutures and prove conclusively that true unilambdoid synostosis exists as a specific but rare entity. Awareness of the features of unilambdoid synostosis will allow more accurate diagnosis and appropriate treatment of posterior plagiocephaly in general and in particular will avoid unnecessary surgical intervention in patients with positional molding.

Cranial Sutures↗

Neurodevelopment in children with single-suture craniosynostosis and plagiocephaly without synostosis.

The objective of this study was to determine whether children with nonsyndromic craniosynostosis and plagiocephaly without synostosis demonstrated cognitive and psychomotor delays when compared with a standardized population sample. This was the initial assessment of a larger prospective study, which involved 21 subjects with nonsyndromic craniosynostosis (mean age, 10.9 months) and 42 subjects with plagiocephaly without synostosis (mean age, 8.4 months). Each child was assessed using the Bayley Scales of Infant Development-II (BSID-II) for cognitive and psychomotor development before therapeutic intervention (surgery for craniosynostosis and molding-helmet therapy for plagiocephaly without synostosis). The distribution of the scores was divided into four groups: accelerated, normal, mild delay, and significant delay. The distributions of the mental developmental index (MDI) and the psychomotor developmental index (PDI) were then compared with a standardized Bayley's age-matched population, using Fisher's exact chi-square test. Within the craniosynostosis group, the PDI scores were significantly different from the standardized distribution (p < 0.001). With regard to the PDI scores, 0 percent of the subjects in the craniosynostosis group were accelerated, 43 percent were normal, 48 percent had mild delay, and 9 percent had significant delay. In contrast, the MDI scores were not statistically different (p = 0.08). Within the group with plagiocephaly without synostosis, both the PDI and MDI scores were significantly different from the normal curve distribution (p < 0.001). With regard to the PDI scores, 0 percent of the subjects in the group with plagiocephaly without synostosis were accelerated, 67 percent were normal, 20 percent had mild delay, and 13 percent had significant delay. With regard to the MDI scores, 0 percent of the subjects in this group were accelerated, 83 percent were normal, 8 percent had mild delay and 9 percent had significant delay. This study indicates that before any intervention, subjects with single-suture syndromic craniosynostosis and plagiocephaly without synostosis demonstrate delays in cognitive and psychomotor development. Continued postintervention assessments are needed to determine whether these developmental delays can be ameliorated with treatment.

Child Development↗