Posterior Plagiocephaly Research Articles

Periocular Asymmetry in Infants with Deformational Posterior Plagiocephaly

ABSTRACTPurpose: To analyze the clinical significance of the periorbital features associated with the facial asymmetry that is common in deformational posterior plagiocephaly (DPP).Patients and methods: We identified 32 patients with DPP, photographed their faces and tops of their head, and performed a complete eye examination. Four examiners analyzed the patient’s periorbital features on the photographs.Results: Median age was 6.5 months (range 3–12 months). Pseudoptosis was identified in 30 patients and pseudo-brow ptosis in 19. Pseudoptosis was marked in 17 patients. Five patients were misdiagnosed with congenital blepharoptosis and received regular follow-ups for amblyopia checks until the diagnosis of pseudoptosis was established. All patients had normal levator function and symmetric eyelid crease. One patient with pseudoptosis and physiologic anisocoria was diagnosed with pseudo-Horner syndrome after a negative 10% cocaine test. None of the patients developed meridional or occlusion amblyopia.Conclusion: DPP is the most frequent form of skull deformation in infants. Its main features are occipital flatness and facial asymmetry. Infants with DPP may present with pseudoptosis and pseudo-brow ptosis on the contralateral side of the occipital flatness. The pseudoptosis in DPP is non-amblyogenic, therefore, ophthalmologic intervention and regular follow-ups are not necessary unless other abnormalities co-exist.

Use of black-bone MRI in the diagnosis of the patients with posterior plagiocephaly.

Ionising radiation exposure is especially harmful to brain development. The purpose of this study was to evaluate whether black-bone (BB) magnetic resonance imaging (MRI), a non-ionising imaging method, offers an alternative to ionising imaging methods such as computed tomography (CT) in the examination of cranial deformities. From 2012 to 2014, a total of 408 children were referred to the Craniofacial Centre at the Helsinki University Hospital for further examination due to flatness of the posterior skull. Fifteen of these patients required further diagnostic imaging. To avoid ionising radiation, we used an MRI protocol that included sequences for evaluation of both brain anatomy and skull bone and sutures by BB-MRI. A semi-automatic skull segmentation algorithm was developed to facilitate the visualisation. Two patients with scaphocephaly were included in the study to confirm the ability to differentiate synostosis with BB-MRI. We obtained informative 3D images using BB-MRI. Seven patients (7/15, 46.7%) had plagiocephaly on the right side and seven on the left side (7/15, 46.7%). One patient (1/15, 6.7%) had symmetric posterior flatness affecting both sides. Neither structural nor signal-intensity alterations of the brain were detected in visual analysis. BB-MRI provides an alternative to CT when imaging craniofacial deformities. BB-MRI provides not only high-quality 3D-reconstructed imaging of the bony structures and sutures but also information on brain structure in one imaging session. With further development, this method could replace ionising radiation-based methods in analysing deformities of the skull.

Lambdoid Synostosis Versus Positional Posterior Plagiocephaly, a Comparison of Skull Base and Shape of Calvarium Using Computed Tomography Imaging

The differential diagnostics between the common positional posterior plagiocephaly and relatively rare lambdoid synostosis is important due to the differences in their treatment plan and clinical management. However, the clinical criteria for the diagnosis of lambdoid synostosis are not clear since there is a considerable overlap in the features of positional posterior plagiocephaly and unilateral lambdoid synostosis. To systematically evaluate the clinical findings in these 2 patient groups, we quantitatively compared the characteristics of endocranial skull base and ectocranial calvarium in 3D computed tomography, in 9 children (mean age 2.9 years) with unilateral lambdoid synostosis and 9 children with positional posterior plagiocephaly. The groups were sex and age matched. Our results show that the skull bases in the lambdoid synostosis are posteriorly shorter and more twisted than in positional posterior plagiocephaly. Anterior twisting was mild in both skull types. Our study confirmed earlier suggested diagnostic feature: prominent ipsilateral mastoidal bossing downward and laterally in all lambdoid skulls. In positional posterior plagiocephaly the bossing was typically not detected. Interestingly, there was a great variation in the position of the ipsilateral ear and external auditory meatus in both patient groups. Thus, neither antero-posterior nor vertical position of ear is a reliable differential diagnostic feature between lambdoid synostosis or positional posterior plagiocephaly.

Diagnosis and treatment of craniosynostosis: Vilnius team experience

Background. The aim of the study was to review the methods of diagnosis of craniosynostosis and to analyze Vilnius (Lithuania) team experience of surgical treatment, surgical methods, aspects of anesthesia for patients with craniosynostosis and to present early results of surgical treatment. Materials and methods. A retrospective review of all patients with various types of craniosynostosis treated surgically during the period from 1 January 2009 to 31 December 2014 was performed. The following data were analyzed: age, type of deformity, surgical technique, surgical time, methods and course of anesthesia, intra- and postoperative complications, parents’ satisfaction, head form. Results. 24 patients were treated. The mean patient’s age at the time of surgery was 13.47 ± 8.2 months (min 7.3, max 46.5). Eliminating 3 patients whose age at the time of surgery was over 2 years (24.5, 29 and 46 months, respectively), the mean age of other 21 patients was 10.63 ± 1.77 (min 7.3, max 14.1) months. There were 9 cases of isolated trigonocephaly (37.5%), 7 cases of isolated scaphocephaly (29.2%), 7 cases of isolated anterior plagiocephaly (29.2%) and 1 case of posterior plagiocephaly combined with scaphocephaly (4.17%). All craniosynostoses were diagnosed clinically and diagnosis was confirmed with computed tomographic scanning. The median duration of surgery was 336.47 ± 59.63 minutes (min 308.13, max 364.82). The medium stay in the intensive care unit was 2.53 ± 1.28 days (min. 1.92, max 3.14). In all cases rigid osteosynthesis was performed. 2 children were diagnosed with syndromic craniofacial abnormalities. In 23 (95.83%) cases an intraoperative or postoperative blood transfusion was required. In 24 treated patients there was no mortality. During the early and late postoperative period no infections, CSF leakage and dural tears were observed. No neurological impairments or any signs of neurological deficits were observed by any of the treated patients. In all of cases parents were satisfied with their children’s changed head shape and aesthetic results. Conclusions. Cranioplasties for correction of craniosynostosis give good aesthetic results and this is a safe method, which helps to correct the head shape as well as improves the social adaptation of patients.

Ocular Torsional Changes in Anterior Plagiocephaly

Introduction: Anterior plagiocephaly has been associated with a mechanical disadvantage to superior oblique secondary to the retrusion of trochlea. This posterior placement should theoretically strengthen the superior oblique in its torsion action both in primary and secondary position (abduction). Materials and Methods: We did an observation study to document the ocular torsional changes by measuring disc foveal angle (DFA) in fundus photograph in five patients with anterior plagiocephaly and unilateral inferior oblique overaction. All patients had right sided anterior plagiocephaly and 2 patients had an associated posterior plagiocephaly. Results: Mean DFA in right eye (affected eye) was 15.8 +/- 1.9 (13 - 18 degrees) and the unaffected eye was 7.6 +/- 2 (range 6-10 degrees). All the patients showed inferior oblique overaction and a V pattern. We found that the affected eye shows excyclotorsion instead of an expected incyclotorsion. This cannot be explained on the basis of retrusion of trochlea and a mechanical disadvantage to the posterior fibres (responsible for depression in adduction) of the superior oblique. Conclusions: The affected eye in anterior plagiocephaly shows excyclotorsion.

Occipital plagiocephaly: unilateral lambdoid synostosis versus positional plagiocephaly

ObjectiveWe defined parameters that could differentiate between positional and synostotic plagiocephaly and defined a diagnostic chart for decision making.DesignProspective study.SettingWe examined 411 children with non-syndromic skull abnormalities between January 2011...

Craniosynostosis of the lambdoid suture.

Craniosynostosis affecting the lambdoid suture is uncommon. The definition of lambdoid craniosynostosis solely applies to those cases demonstrating true suture obliteration, similar to other forms of craniosynostosis. In patients presenting with posterior plagiocephaly, true lambdoid craniosynostosis must be differentiated from the much more common positional molding. It can occur in a unilateral form, a bilateral form, or as part of a complex craniosynostosis. In children with craniofacial syndromes, synostosis of the lambdoid suture most often is seen within the context of a pansynostotic picture. Chiari malformations are commonly seen in multisutural and syndromic types of craniosynostosis that affect the lambdoid sutures. Posterior cranial vault remodeling is recommended to provide adequate intracranial volume to allow for brain growth and to normalize the skull shape. Although many techniques have been described for the correction of lambdoid synostosis, optimal outcomes may result from those techniques based on the concept of occipital advancement.

Results of posterior cranial vault remodeling for plagiocephaly and brachycephaly by the meander technique.

Several techniques to remodel the posterior calvarium in order to increase intracranial volume (ICV) and to improve cosmetic appearance are reported. This study presents the results of meander technique in patients with brachycephaly and posterior plagiocephaly. During December 2011 and July 2013, a total of 12 children (median age: 15 months) underwent posterior cranial vault remodeling by the meander technique (brachycephaly, n = 6; posterior plagiocephaly, n = 6). The available pre- and postoperative MRIs were assessed with regard to ICV, cranial index (CI) and asymmetry index (AI) as well as the position of the cerebellar tonsils. No intra- or postoperative complications were observed. Blood transfusions were necessary in nine of 12 patients. A significant increase of the ICV from 1,178.4 ± 134.5 to 1,293.0 ± 137.5 cm(3) (p < 0.05) is demonstrated. In the patients with brachycephaly the CI was significantly improved from 0.97 ± 0.12 to 0.89 ± 0.12 postoperatively (p < 0.05). The AI in patients with posterior plagiocephaly was significantly ameliorated from 0.83 ± 0.04 to 0.92 ± 0.02 postoperatively (p < 0.05). There was a significant effect on cerebellar tonsil position in relation to foramen magnum level for patients with brachycephaly (right tonsil: 11.9 ± 9.2 to 7.0 ± 9.1 mm, p < 0.05; left: 10.8 ± 9.5 to 9.7 ± 10.6 mm; p < 0.05) as well as in posterior plagiocephaly for the ipsilateral tonsil (3.2 ± 3.5 to 1.6 ± 3.5 mm; p < 0.01). The presented surgical technique is considered to be safe. The technique is capable to significantly increase ICV and improve cosmetic appearance of the remodeled calvarium. Further evidence that posterior cranial vault remodeling influences the position of the cerebellar tonsils is added by the results of the study.

Major clinical features of synostotic occipital plagiocephaly: mechanisms of cranial deformations.

The clinical diagnosis of most common single-suture craniosynostosis is easily set, based on the stereotype of deformities and knowledge of the mechanisms of cranial deformations. However, synostosis of unilateral lambdoid suture, probably due to its lower incidence and similarity with other non-synostotic deformities affecting the posterior portion of the skull, makes its clinical diagnosis more difficult and imprecise. The aim of this study is to evaluate the most easily and accurate clinical characteristics to be recognized in the synostotic occipital plagiocephaly. This study consisted of clinical evaluation of eight patients with synostotic occipital plagiocephaly, whose diagnosis was further corroborated by computed tomography. We identified the following: unilateral occipital flattening in eight out of eight patients (100 %), bulging of ipsilateral mastoid process in eight out of eight (100 %), "edge effect" of ipsilateral lambdoid suture in eight out of eight (100 %), inferior deviation of the ear in eight out of eight (100 %), "Dumbo" ears in eight out of eight (100 %), horizontal slant of the bimastoid line in seven out of eight (87.5 %), tilt of the head viewed from behind in seven out of eight (87.5 %), trapezoidal contour of the skull in top view in six out of eight (75 %), contralateral parietal bossing in six out of eight (75 %), and bossing of the contralateral forehead three out of eight (37.5 %). The most important clinical features specific to the clinical diagnosis of synostotic occipital plagiocephaly, not present in the positional posterior plagiocephaly, were bulging of the ipsilateral mastoid process, edge effect of the synostotic lambdoid suture, tilt of the head, and slant of the bimastoid line viewed from behind, inferior deviation of the ear, and contralateral parietal bossing.

Lagebedingter Plagiocephalus im Säuglingsalter: Diagnose und Behandlung

Positional plagiocephaly in the newborn corresponds to a posterior flattening and asymetry of the head. Its incidence has significantly increased since the "back to sleep" campain in the nineteen nineties to prevent sudden infant death syndrome. The posterior deformation usually worsens during the first six months of life when the skull is susceptible to posterior constant pressure which induces the deformation. Spontaneous outcome can be favorable. Treatment is always conservative and consists in positionnal exercise, physiotherapy and osteopathy. In some cases treatment with a helmet is recommended for a period of three months usually. Posterior positionnal plagiocephaly has no consequences on the brain's developement an is considered as an esthetic issue. In most cases good results are obtained after management with a custom fitted helmet when needed.

Clinical and imaging findings in children with non-syndromic lambdoid synostosis

True unilateral lambdoid synostosis is a very rare type of craniosynostosis. Patients present with unilateral posterior plagiocephaly. The differentiation between frequent, simple positional (deformational) posterior plagiocephaly and lambdoid synostosis is not easy and to date subject of controversy. Accurate and early diagnosis is important, because treatment is different. Simple positional plagiocephaly responds to conservative treatment, but craniosynostosis may require neurosurgical intervention. We analyzed clinical presentation of five patients in whom non-syndromic lambdoid synostosis was confirmed by CT imaging, in one additionally by high-resolution ultrasound, and finally neurosurgical intervention. However, clinical inspection alone did not reliably identify infants with lambdoid synostosis. Diagnosis of lambdoid synostosis is not always possible based on clinical inspection alone. To confirm the diagnosis imaging is recommended. There is evidence that high-resolution ultrasound can be used first-line in the future.

The value of ultrasound-assisted pinned resorbable osteosynthesis for cranial vault remodelling in craniosynostosis

Resorbable osteosynthesis is a widespread tool in craniofacial surgery, however only a limited number of studies have focused on ultrasound-assisted pinned resorbable systems in the treatment of craniosynostosis.Thirty-eight children with various types of craniosynostosis including scaphocephaly, trigonocephaly, anterior and posterior plagiocephaly were treated using the Sonic Welding resorbable osteosynthesis system. All patients were evaluated for operation time, stability of the surgical results, rate of local infections and visibility or palpability of the osteosynthesis material in the follow-up ranging from 15 to 21 month.Mean operation time was not significantly higher compared to conventional osteosynthesis material and all remodelled cranial vaults showed immediate stability. Only one patient showed signs of an inflammatory skin reaction, which recovered spontaneously. The number of palpable or visible plates, respectively, increased during the first months with a maximum at 12 months (34 (89%) plates palpable, 26 (68%) plates visible). After this time point, the number decreased continuously until the end of the follow-up period at 21 months when 3 (20%) plates were palpable, 0 (0%) plates were visible).Ultrasound-assisted pinned resorbable systems seem to be a promising tool in craniofacial surgery providing a timesaving and stable osteosynthesis. An initial swelling of the plates during the first 12 months before the complete degradation might result in a palpable and visible bulge.

Suitable Indication for the Application of Distraction Osteogenesis

In recent years, cranial distraction osteogenesis (DOG) for craniosynostosis has often been performed in Japan. Since 1998, DOG has been conducted in more than 80 patients with craniosynostosis in our institution. It is now important to define the appropriate indications for the application of DOG for craniosynostosis. From the point of view of our more than 10 years of experience with the application of cranial DOG, posterior enlargement is one of the most appropriate applications. Bilateral posterior expansion is conducted for hypertension because of bilateral lambdoidal synostosis, whereas unilateral posterior expansion is conducted for asymmetry because of unilateral posterior plagiocephaly. Posterior expansion by gradual distraction was conducted for 12 cases. Eight cases of syndromic craniosynostosis and 4 of simple unilateral lambdoid synostosis were included. Clinical and radiological improvement was observed in all cases. Postdistraction computed tomography demonstrated a decrease in digital printing within a few months in all cases. Distraction osteogenesis is a useful procedure for posterior enlargement by gradual expansion and guarantees postdistraction rigidity without collapse in a supine position in bilateral and unilateral lambdoid synostosis.

Achondroplasia with multiple‐suture craniosynostosis: A report of a new case of this rare association

We report on a female patient with an exceedingly rare combination of achondroplasia and multiple-suture craniosynostosis. Besides the specific features of achondroplasia, synostosis of the metopic, coronal, lambdoid, and squamosal sutures was found. Series of neurosurgical interventions were carried out, principally for acrocephaly and posterior plagiocephaly. The most common achondroplasia mutation, a p.Gly380Arg in the fibroblast growth factor receptor 3 (FGFR3) gene, was detected. Cytogenetic and array CGH analyses, as well as molecular genetic testing of FGFR1, 2, 3 and TWIST1 genes failed to identify any additional genetic alteration. It is suggested that this unusual phenotype is a result of variable expressivity of the common achondroplasia mutation.

Effectiveness and Safety of Independent Pediatric Nurse Practitioners in Evaluating Plagiocephaly

The American Academy of Pediatrics Back to Sleep program in 1992 dramatically increased the incidence of posterior plagiocephaly in infants. In 2000, the craniofacial disorders program at Oregon Health & Science University established a plagiocephaly screening clinic staffed by pediatric nurse practitioners. Electronic medical records of patients seen in a single accredited craniofacial disorders clinic from 2005 to 2011 were reviewed retrospectively to identify patients screened independently by pediatric nurse practitioners for positional plagiocephaly versus synostosis. Over a 7-year period, 1228 patients were independently screened. Over half [638 patients (52 percent)] were followed through subsequent visits for craniofacial disorders without any direct surgeon input. Of these, 169 (26 percent) were treated with orthotic consultation for a molding helmet, 385 (60 percent) received a combination of counseling and/or physical therapy for torticollis, and 84 (13 percent) were lost to follow-up. No patient managed by pediatric nurse practitioners was identified to have cranial synostosis and no patient underwent early or delayed surgery. All returning patients [n=554 (86 percent)] showed improvement in, or resolution of, their presenting deformity. The remaining 590 patients (48 percent) were referred by the pediatric nurse practitioner for surgical consultation. Computed tomographic imaging revealed synostosis in 121 patients. Pediatric nurse practitioners accurately screened over half of patients referred to a high-volume craniofacial disorders program for positional plagiocephaly versus synostosis, without surgeon input. Based on available information, no synostosis diagnosis was overlooked using this approach. With specific training, pediatric nurse practitioners working in a craniofacial disorders program can safely and independently screen for positional versus synostotic plagiocephaly. Diagnostic, III.

Non-surgical management of posterior positional plagiocephaly: Orthotics versus repositioning

Evaluate from the literature, the evidence of comparative efficiency of non-surgical treatments (orthotics or head repositioning therapy) in posterior positional plagiocephaly. Systematic review from scientific articles (original cohort studies and review of literature), published in French or in English, searched on five online literature data bases, comparing non-chirurgical treatments (repositioning and orthotics therapy) for deformational plagiocephaly. A standardized method guidelines (Critical Review Form-Quantitative Studies) has been used. Only 11 cohort studies met the inclusion criteria and six reviews of literature were analyzed. Many biases have been identified, most of the time, favoring the repositioning groups (older infants and plagiocephaly more severe). Several different orthotics seem to correct head deformities better and faster than repositioning protocols. Evaluation methods, treatment indications and long-term efficacy should be clarified. Studies about treatment risks are warranted.

Posterior remodeling flap for posterior plagiocephaly

Nonsynostotic posterior plagiocephaly has become the most common skull deformation since pediatricians have suggested the supine position for the newborns to reduce the risk of sudden death. Prevention of such a "positional" deformation or its management once it has occurred is mainly based on physical maneuvers such as physiotherapy and active positional corrective measures. Surgical correction, however, may be suggested in rare cases where deformation of the skull is so severe or the referral of the child is so late that physical corrective treatment cannot be taken into consideration. Surgical management is based on the creation of a posterior bone flap to be repositioned after the opportune contouring and rotation. The aim of this paper is to describe the surgical technique used for posterior vault remodeling in posterior plagiocephaly at the craniofacial unit of Hopital Necker Enfants Malades (French National Referral Center for Faciocraniosynostosis) focusing on its advantages and limitations.

Genetic basis of single-suture synostoses: genes, chromosomes and clinical implications

Non syndromic craniosynostoses are the most frequent craniofacial malformations worldwide. They represent a wide and heterogeneous group of entities, in which the dysmorphism may occur in a single (simple forms) or in multiple sutures (complex forms). Simple forms present a higher birth prevalence and are classified according to the involved suture and to the corresponding abnormal cranial shape: scaphocephaly (SC; sagittal suture), trigonocephaly (TC; metopic suture), anterior plagiocephaly (unilateral coronal suture), posterior plagiocephaly (unilateral lambdoid suture). They occur commonly as sporadic forms, although a familiar recurrence is sometimes observed, suggesting a mendelian inheritance. The genetic causes of simple craniosynostosis are still largely unknown, as mutations in common craniosynostosis-associated genes and structural chromosomal aberrations have been rarely found in these cases. This review is intended to dissect comprehensively the state-of-the art on the genetic etiology of single suture craniosynostoses, in the attempt to categorize all known disease-associated genes and chromosomal aberrations. Possible genotype/phenotype correlations are discussed as useful clues towards the definition of optimized clinical management flowcharts.

Posterior plagiocephaly

Asymmetrical cranial vaults resulting from external forces on an infant's head can be caused by abnormal sutural development (synostotic plagiocephaly) or abnormal external forces acting on an intrinsically normal, developing cranium (deformational plagiocephaly). The incidence of posterior plagiocephaly has increased dramatically since the initiation of the "Back to Sleep" campaign against sudden infant death syndrome. The majority of cases are due to deformational plagiocephaly, but rigorous diagnostic evaluation including physical examination and radiological imaging must be undertaken to rule out lambdoid synostosis in extreme or refractory cases. Unique clinical features and radiological examination using computed tomography technology are helpful in confirming the correct cause of posterior plagiocephaly. Plagiocephaly is considered a benign condition, but with the recent increase in cases, new studies have revealed developmental problems associated with cranial vault asymmetries. Treatment of positional/deformational plagiocephaly includes conservative measures, primarily behavior modification, and, in some cases, helmet therapy, whereas lambdoid synostotic plagiocephaly requires surgical intervention, making differentiation of the cause of the asymmetry critical.

Deficient language acquisition in children with single suture craniosynostosis and deformational posterior plagiocephaly

This study examined early language acquisition in children with single suture craniosynostosis (SSC) and in children with deformational posterior plagiocephaly. Our purpose was to determine whether infants with SSC have normal language acquisition at the age of 3 years, and whether infants with deformational posterior plagiocephaly demonstrate parallel development when compared with children with SSC. The study population includes 61 infants. Twenty of them had synostosis of the sagittal suture, 12 synostosis of other suture and 29 deformational posterior plagiocephaly. Forty-nine of them were operated on a mean age of 10.6 months, and 12 were non-operated children with deformational posterior plagiocephaly. Language skills of participants were prospectively evaluated at the mean age of 3 years 4 months. About one half of the subjects (49%) had normal linguistic development, 30% had slight developmental problems and 21% had severe disorders in speech-language-related skills. These figures showed the prevalence of severe language disorders to be three times higher in our study population when compared with the general population. Children with sagittal synostosis managed better in all language skills compared with other types of SSC. Defective language development was found in deformational posterior plagiocephaly, both operated and non-operated. We found a noticeable developmental risk for specific language impairment in children with nonsyndromic SSC, and that the deviant language development is observable already in early infancy. Contrary to previous beliefs, the developmental risk for defective language development in deformational posterior plagiocephaly was found in both operated and non-operated subjects.