Long-term ophthalmological outcomes in patients with anterior plagiocephaly managed with a fronto-orbital advancement procedure.

BACKGROUND: Anterior plagiocephaly results from early fusion of a unilateral coronal cranial suture, which affects 1/10,000 infants at birth and can be associated with ophthalmological complications. The study aimed to evaluate the frequency of oculomotor disorders and refractive errors in children with non-syndromic anterior plagiocephaly. METHODS: Patients with anterior plagiocephaly undergoing surgery using a fronto-orbital advancement technique were included in this retrospective study between 2011 and 2017. The following data were collected: cycloplegic refraction in diopters (D), best-corrected visual acuity, manifest strabismus in primary position, ocular motility, head tilt in primary position, slit-lamp and fundus examination. The refractive errors were determined by autorefraction after cycloplegia with cyclopentolate. RESULTS: Among the sixteen patients included, hyperopia >2.5D was found in 10 patients, astigmatism >1D in 10 patients and anisometropia >1D in 7 patients. Astigmatism was contralateral to the synostosis in 7 cases. In total, significant refractive errors were found in 92.9%. Amblyopia was found in 33.3% patients. Strabismus was found in 11 patients, most frequently combined horizontal and vertical, of which 4 required strabismus surgery. The most frequent vertical deviation was ipsilateral hypertropia in 54.5%. We found superior oblique muscle limitation in 3 patients. Optic disc pallor was found in 2 patients. CONCLUSION: In the years following fronto-orbital advancement for non-syndromic anterior plagiocephaly, refractive errors and oculomotor disorders are frequently encountered, mainly contralateral astigmatism and strabismus with both horizontal deviation and ispilateral hypertropia.

Craniosynostosis and oculomotor disorders.

Simple, complex or syndromic craniosynostosis may be responsible for ocular and especially oculomotor pathologies. Among simple craniosynostosis, anterior plagiocephaly is the most frequently associated with oculomotor disorders. Oculomotor disorders encountered in craniosynostosis are specific to this pathology. They may be related to orbital deformities or oculomotor muscle malformations. Early craniofacial surgery reduces the onset and severity of these oculomotor disorders which is very important for ophtalmological patient care. Indeed, these oculomotor disorders are difficult to treat for the ophthalmologist with most of the time several surgeries needed, and lead to amblyopia if neglected.

Isolated Lambdoid Craniosynostosis.

INTRODUCTION: Lambdoid craniosynostosis is an extremely rare anomaly in which there is premature fusion of one or both lambdoid sutures. The mainstay of treatment is surgical intervention, for which various procedures have been described, but there is a paucity of data on long-term outcomes. This study examines the long-term outcomes in the surgical management of this challenging condition, showing that accurate diagnosis and careful planning can lead to safe and consistent results. MATERIALS AND METHODS: A retrospective chart review was performed looking at all cases of isolated lambdoid craniosynostosis treated with surgical intervention by the senior author from 1999 to 2016. Data collected included gender, age at diagnosis, age at surgery, length of follow up, method of diagnosis, side of affected suture, pre-operative and post-operative physical exam findings, surgical technique, complications, re-operation rate, and associated torticollis. RESULTS: Twenty-five patients (N = 25) were included in the study. All patients underwent posterior calvarial remodeling with/without barrel stave osteotomies and full thickness calvarial bone grafts. Mean length of follow up after operative intervention was 43.8 months (+/- 23.2 months). All patients were judged to have significantly improved head contour which was near-normal at conversational distance during post-operative follow up by the senior author. Residual plagiocephaly was present in 24% of patients. There were no major complications in this series. Reoperation rate was 8%. Seventy-six percent of patients also presented with torticollis, of which 37% had refractory torticollis that required sternocleidomastoid (SCM) release by the senior author. DISCUSSION: The authors present one of the largest series of operative cases of isolated lambdoid craniosynostosis to date. Our data show that with accurate diagnosis and careful planning, safe and consistent long-term results can be achieved with surgical intervention. A significant number of patients in our series also presented with concomitant torticollis. The authors recommend that all patients being evaluated for posterior plagiocephaly should also be evaluated for torticollis, because without recognition and intervention, patients may continue to have residual facial asymmetry and head shape abnormalities despite optimal surgical correction of the lambdoid synostosis.

Telescoping With Multiple Revolution Cranial Osteotomies in Patients With Simple Craniosynostosis.

Simple craniosynostosis is a cranial disease characterized by the premature closure of the cranial sutures, it develops during the first years of life and affects 1 in every 2000 to 2500 births worldwide (1). The cranial growth alteration occurs as parallel flattening to the compromised sutured with compensatory bulging in a perpendicular vector. Currently, The Suturectomy is the gold validated surgical treatment, that besides the dynamic Cranioplasties of multiples revolutions allows the design of bone flaps and therefore the correction of the secondary deformities caused by the synostosis. This multicenter descriptive study assessed a 20 series of cases (6 Plagiocephaly, 13 Scaphocephaly, 1 Brachycephaly) obtained in Cali, Colombia, that underwent surgery between January of 2014 and December of 2017, applying a Suturectomy surgery with additional telescoping of multiple revolution cranial osteotomies. The authors observe no clinical complications in the recruited patients regarding postoperative period of the described surgical technique (1, 90, and 180 days), thereby obtaining excellent outcomes on the maintained suture distraction focused on the assessment of the 3D reconstruction computed tomography scans.

Role of Autologous Fat Injection in Neglected Patients With Anterior Plagiocephaly.

Anterior plagiocephaly is a type of non-syndromic craniosynostosis requiring surgery. In this study, the authors have presented 15 neglected patients with anterior plagiocephaly. Two of patients had moderate plagiocephaly whereas others had mild plagiocephaly. A total of 38 procedures (fat injections) were performed on these patients. Following procedures, 2 of patients were under corrected, 2 of who were over corrected, and others were adequate corrected. One of the patients developed fat necrosis. None of them showed persistent over correction. Authors believe that autologous fat transplantation is a safe and effective method, in patients with anterior plagiocephaly who have not been operated, to improve patients' appearance and, subsequently, their self-esteem as well as their social function.

Use of Navigation System in Craniofacial Surgery.

The use of navigated surgery in the treatment of craniofacial malformations can help obtain optimal results. In this article, the authors will discuss a case of anterior plagiocephaly, corrected with frontorbital bandeau remodeling. Navigation was used during the osteotomy and the reposition phase to ensure the correct positioning of the osteotomy instruments. It was also used to ensure that the bandeau was correctly repositioned in accordance with the surgical plan determined during the virtual simulation phase of the surgery.

Anterior Unilateral Plagiocephaly in Patient with Alagille Syndrome: Case Report.

BACKGROUND: The polymalformative syndromes and craniofacial anomalies association is a well-known phenomenon in patients with Crouzon, Pfeiffer, Apert, or Muenke disease. Recently, other less frequent pathologies, such as Alagille syndrome, have shown an association with alterations in the development of cranial sutures, resulting in serious cosmetic defects and neurologic disorders. CASE DESCRIPTION: We report an exceptional case of a 30-month-old girl, a nephroblastoma survivor diagnosed with Alagille syndrome, who was referred to our department with progressive anterior plagiocephaly and premature left coronal suture closure associated with a large compensating right bossing. Despite the patient's age, we offered aggressive surgical treatment performing a new forehead harvested from the skull vertex with orbital rim reconstruction. CONCLUSIONS: Alagille syndrome is a complex multisystem pathology with a poor craniosynostosis association and only 3 cases have been described in the literature.

Craniofacial Distraction in the Management of Anterior Plagiocephaly: A Novel Idea and a Systematic Review of the Literature.

BACKGROUND: The objective of this work is to present a review of all reports involving the management of unicoronal synostosis with distraction osteogenesis (DO). Also, we have innovated a new method in the treatment of cases of anterior plagiocephaly at a young age using DO. METHODS: We present a case in which anterior plagiocephaly was treated by DO of both metopic and hemicoronal sutures in a 4-month-old female patient. A comprehensive systematic literature review was completed using the search terms "distraction osteogenesis," "unicoronal synostosis," "anterior plagiocephaly," and "craniosynostosis." We excluded all experimental articles and reviewed clinical reports detailing the use of DO in the management of unicoronal synostosis. RESULTS: The study sample of this review consisted of 16 reports published over a period of 17 years that were analyzed in detail. The total number of patients treated by DO was 120, and the mean age at operation was 12 months. In the case presented, successful correction of the unicoronal synostosis was achieved. CONCLUSION: Hemicoronal and metopic suture distraction in anterior plagiocephaly achieves considerable improvement in the midline shift of the anterior cranial base and naso-orbital complex.

A New Operative One-Wing Technique to Correct Fronto-Orbital Region in Unilateral Nonsyndromic Plagiocephaly.

Anterior plagiocephaly is a premature closing of unilateral coronal suture. This premature suture fusion causes a series of cranial asymmetry and alteration of the fronto-orbital region.The authors described a new surgical technique to correct the facial asymmetry that reduces the operative time and the possible complications.In a period between 2013and 2016, 12 children affected by nonsyndromic anterior plagiocephaly were treated with this new procedure.Clinical, cranial development, and absence of postoperative complication demonstrated that one-wing surgical bone correction is a useful and innovative technique.

The self-defining axis of symmetry: A new method to determine optimal symmetry and its application and limitation in craniofacial surgery.

PURPOSE: Analysis of symmetry represents an essential aspect of plastic-reconstructive surgery. For cases in which reference points are either not fixed or are changed due to corrective intervention the determination of a symmetry axis is sometimes almost impossible and a pre-defined symmetry axis would not always be helpful. To assess cranial shape of surgical patients with craniosynostosis, a new algebraic approach was chosen in which deviation from the optimal symmetry axis could be quantified. MATERIALS AND METHODS: Optimal symmetry was defined based on a single central point in the fronto-orbital advancement (FOA) hyperplane and a corresponding landmark pair. The forehead symmetry evaluation was based on 3D-scans series of 13 children, on whom cranioplasty with FOA was performed and 15 healthy children who served as control group. RESULTS: Children with plagiocephaly showed considerable improvement in symmetry postoperatively, with stable values over one year, while those with trigonocephaly and brachycephaly showed constant good symmetry in the forehead both pre- and postoperatively. CONCLUSIONS: With the help of an optimally calculated symmetry axis this new analysis method offers a solution, which is independent of preset dimensions. Patients can be evaluated according to their individual needs regarding symmetry and also be compared with one another.

Squamosal Suture Synostosis: Incidence, Associations, and Implications for Treatment.

Squamosal suture craniosynostosis is thought to be a relatively rare entity. In the authors' experience, it is underreported in imaging examinations and the existing literature. The authors sought to determine the incidence of squamosal synostosis, whether it is increasing in frequency, and its relationship with synostosis of the major calvarial sutures.Patients undergoing computed tomography imaging for suspected craniosynostosis over a 15-year period were reviewed by a plastic surgeon and pediatric neuroradiologist. Patients with synostosis of the squamosal sutures were identified and involvement of additional sutures, gender, and the presence of a known syndromic diagnosis were recorded. Patients greater than 4 years of age or those with prior craniofacial surgery were excluded.One hundred twenty-five patients met inclusion criteria, 26 of whom had squamosal suture synostosis (26/125, 20.8%). Squamosal synostosis was found in isolation in 3 patients (3/26, 11.5%), with 1 additional major suture in 10 patients (10/26, 38.5%), and ≥2 major sutures in 13 patients (13/26, 50%). Squamosal synostosis was more common in patients with a syndromic diagnosis (11/26 syndromic, 15/99 nonsyndromic, P < 0.001). Eleven of 26 patients with squamosal synostosis were identified in the radiology report (42.3%).Craniosynostosis of the squamosal suture is much more common than previously reported and can contribute to abnormal head shape in isolation, or in combination with major sutures. Squamosal suture synostosis is underdiagnosed clinically and radiologically, although insufficient evidence exists to determine if its true incidence is increasing.

Effect of fronto-orbital advancement on astigmatism in patients with anterior plagiocephaly.

The purpose of this study was to determine the effect of unilateral fronto-orbital advancement (FOA) or bilateral FOA on ocular aspects of plagiocephaly. A retrospective review of ocular findings in 16 patients with plagiocephaly was performed. Patients were divided into 2 groups: 12 patients who underwent bilateral FOA (bFOA) and 4 patients who underwent unilateral FOA (uFOA), and ocular findings were compared. One-half of patients showed strabismus in both groups, and all had exotropia. Hypertropia was found only on the same side of the plagiocephaly in 17% of the bFOA group and 25% of the uFOA group. One-third of the patients in the bFOA group and one-half of patients in the uFOA group had oblique muscle dysfunction. In terms of astigmatism, the degree of with-the-rule astigmatism on the contralateral side was larger in the bFOA group compared to the uFOA group (p = 0.030). The degree of with-the-rule astigmatism was larger on the contralateral side than the ipsilateral side (p = 0.005) in the bFOA group. Patients with abnormalities in ductions/versions had larger astigmatism on the contralateral side than those without abnormalities in ductions/versions. In conclusion, bilateral FOA could induce unwanted outcomes of larger astigmatism on the contralateral side. Astigmatism should be carefully evaluated after bilateral FOA.

Long-Term Assessment of Suturectomy in Trigonocephaly and Anterior Plagiocephaly.

INTRODUCTION: Techniques for treating trigonocephaly and anterior plagiocephaly have evolved from the initial suturectomy technique to frontal cranioplasty. The authors evaluated the suturectomy technique in adolescent patients with these craniosynostoses, by carrying out a retrospective, long-term assessment at the end of growth. METHODS: Patients with anterior plagiocephaly or trigonocephaly were included. All had undergone coronal or metopic suturectomy with bilateral orbitofrontal bandeau resection between 1997 and 2005. The primary endpoint was the Whitaker classification. The secondary endpoints were anthropometric measurement, assessments of the bone defects on computed tomography scan, and the comments of patients and their relatives concerning the final skull outcome. Patients with anterior plagiocephaly also attended an ophthalmological consultation. RESULTS: Seventeen patients were included in the study: 8 with anterior plagiocephaly and 9 with trigonocephaly. Mean age at the time of surgery was 6.91 months. Mean age at the time of craniofacial consultation was 14 years. Fourteen patients (82%) were classified as Whitaker Class III and IV, corresponding to poor esthetic results and persistent bone defects. Seven patients requested further surgery. CONCLUSION: This study shows that suturectomy seems to yield poor esthetic results in the long term and patients should be followed up throughout adolescence to correct any craniofacial deformities.

Surgical results of cranioplasty with a polymethylmethacrylate customized cranial implant in pediatric patients: a single-center experience.

OBJECTIVE Cranioplasty is a reconstructive procedure used to restore skull anatomy and repair skull defects. Optimal skull reconstruction is a challenge for neurosurgeons, and the strategy used to achieve the best result remains a topic of debate, especially in pediatric patients for whom the continuing skull growth makes the choice of material more difficult. When the native bone flap, which is universally accepted as the preferred option in pediatric patients, is unavailable, the authors' choice of prosthetic material is a polymethylmethacrylate (PMMA) implant designed using a custom-made technique. In this paper the authors present the results of their clinical series of 12 custom-made PMMA implants in pediatric patients. METHODS A retrospective study of the patients who had undergone cranioplasty at Gaslini Children's Hospital between 2006 and 2013 was conducted. A total of 12 consecutive cranioplasties in 12 patients was reviewed, in which a patient-specific PMMA implant was manufactured using a virtual 3D model and then transformed into a physical model using selective laser sintering or 3D printing. All patients or parents were administered a questionnaire to assess how the patient/parent judged the aesthetic result. RESULTS Patient age at craniectomy ranged from 5 months to 12.5 years, with a mean age of 84.33 months at cranioplasty. The mean extension of the custom-made plastic was 56.83 cm(2). The mean time between craniectomy and cranioplasty was 9.25 months. The mean follow-up duration was 55.7 months. No major complications were recorded; 3 patients experienced minor/moderate complications (prosthesis dislocation, granuloma formation, and fluid collection). CONCLUSIONS In this patient series, PMMA resulted in an extremely low complication rate and the custom-made technique was associated with an excellent grade of patient or parent satisfaction on long-term follow up.

The usage of the three-dimension distractor in the NAVID system for plagiocephaly-three case reports.

BACKGROUND: Distraction osteogenesis is a standard method for craniosynostosis. However, the technique using conventional devices still has some disadvantages, especially for anterior or posterior plagiocephaly with complex deformities. In the Nakajima's angle-variable internal distraction (NAVID) system originally for maxillary surgeries, the cranial three-dimension (D) distractor with three dimensionally movable joint at the anterior arm has been developed recently in order to prevent the interference in the distraction process and excessive force. CASE REPORTS: In this paper, we first reported two cases of anterior plagiocephaly, and one case of posterior plagiocephaly received distraction osteogenesis using new 3-D distractor in the NAVID system. In two cases of anterior plagiocephaly, the reshaping of supra-orbital bar in reference of simulating by the 3-D skull model was performed. In all cases, we fixed two paralleled 2-D distractors and a 3-D distractor in the upper frontal or parietal region. CONCLUSION: Within the limitations of this study, we believe that the NAVID system is suitable for infant plagiocephaly due to the simple and small joint arm. Furthermore, the usage of the 3-D distractor would reduce the interference with 2-D distractors and easily lead to attainment of targeted distracting distance.

Frontal cranial modeling using endocranial resorbable plate fixation in 27 consecutive plagiocephaly and trigonocephaly patients.

INTRODUCTION: Premature ossification of coronal and metopic sutures is treated by fronto-orbital remodeling. Such operations require stable fixation of the reshaped cranial bones. Currently, biodegradable plating systems are used to provide sufficient stability over the time that takes for the osteotomies to ossify. Plates that are placed traditionally on the outer surface of the cranium are often palpable and even visible through the thin overlying skin, compromising the cosmetic results of these operations. Improved aesthetics could be achieved by placing the plates endocranially. PURPOSE: This study aimed to evaluate endocranial resorbable plate fixation and its clinical and radiographic results in frontal remodeling cranioplasty for plagiocephaly and trigonocephaly patients with follow-up sufficiently long for the plates to have been completely resorbed. METHODS: A poly(lactide-co-glycolide) (PLGA) resorbable plating system was used on the inner aspect of frontal bone in 27 patients treated for coronal and metopic craniosynostoses. The outcome was evaluated at follow-up visits. The mean follow-up was 79.2 months. RESULTS: Three patients had complications that required reoperations. None of these complications were related to the endocranial location of the plates. There were no problems with ossification of the osteotomy sites. All but one patient's outcome was judged as good or excellent. CONCLUSION: Placement of resorbable fixation on the endocranial surface of the calvarial bones is safe, stable, and results in satisfactory aesthetics without interfering with the ossification of the cranial bones.

Surgical correction of craniosynostosis. A review of 100 cases.

PURPOSE: Provide outcome data for open cranial vault reshaping at a single institution by a single craniofacial surgeon treating 100 patients. METHODS AND SUBJECTS: A total of 100 patient records were reviewed. Criteria for selection included patients less than three years of age undergoing primary surgery with open cranial vault reshaping and a minimum follow up time of 2 years. RESULTS: Of the 100 patients (27 female, 73 male) treated 6 were syndromic and 94 nonsyndromic. Average age and weight were 8.9 months and 9.51 kg, respectively. The oldest child was 30 months and the youngest 5 months at the time of surgery. The estimated blood volume lost was 42.7% of total calculated blood volume ranging from 16.6% to 336%. Average surgical time was 216.7 min. Complications included 2 hematomas, 2 wound infections, 1 subgaleal abscess, 6 dural tears, 3 patients requiring reoperation for residual deformity, 4 cases requiring coronal scar revision, 1 sagittal sinus bleed, and 1 intraoperative death. CONCLUSIONS: Our review of 100 open repairs of patients with craniosynostosis demonstrates good long-term results with an overall low complication rate. The outcome data will assist in developing future prospective studies aimed at improving the multidisciplinary care of these patients.

The assessment of relationship between the skull base development and the severity of frontal plagiocephaly after bilateral fronto-orbital advancement in the early life.

PURPOSE: The deformation of the skull base in patients with unilateral frontal plagiocephaly (UFP) is well known, but the mechanism is not still clear. We analyzed the skull base in the patients with UFP who underwent fronto-orbital advancement (FOA) in the early life during the last decade. METHODS: We assessed the treatment results and outcome of FOA performed in six patients, four girls and two boys younger than 2 years, in the last decade. Also, the basal cranium's angles were measured by 3D reconstruction images on computed tomography (CT) scan. RESULTS: The mean patients' age at FOAs was 11 months. Two cases were classified as grade 2A, two cases as grade 2B, and two cases as grade 3 (the classification of Di Rocco and Velardi). The ethmoidal axis was deviated a mean of 8.2° to the affected side. The mean angle between the petrosal pyramids and the midline (anterior-petrosal-sagittal angle, APSA) was 75.3° on the affected side and 66.2° on the normal side. The mean difference of APSA was 9.2°. On the follow-up CT images 5 years after surgery, the deviations of the ethmoidal axis clearly decreased, 5.7°, but the differences of APSA did not change, 8.8°. CONCLUSIONS: The midline distortion of anterior skull base should be considered to be spontaneously corrected during the follow-up periods in patients with all types of UFP who underwent FOA, unlike posterior skull base in the patients with grades 2B and 3 classification.

Suitable indication for the application of distraction osteogenesis: occipital enlargement for lambdoid synostosis.

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.

Cranial vault growth patterns of plagiocephaly and trigonocephaly patients following fronto-orbital advancement: a long-term anthropometric outcome assessment.

Fronto-orbital advancement (FOA) is the most common technique used to correct plagiocephaly and trigonocephaly. The aim of this study was to investigate the cranial vault growth following FOA and to compare the growth pattern to the normal standard. Fifty-two Swiss children (25 with anterior plagiocephaly and 27 with trigonocephaly) between the surgical ages of 8 and 10 months were included in this 5-year follow-up study. A standardised time protocol (before the procedure; 6 weeks and 6 months after the surgery; and then annually until the age of six) for anthropometric skull measurements (head circumference, cranial length, and breadth and cephalic indices) was used to analyse the pre- and post-operative cranial vault growth patterns. Data were converted into the Z-score for standardised inter-centre comparison. For all the patients, a significant improvement in the shape of the cranial vault following FOA was observed. In both the plagiocephaly and trigonocephaly groups, the long-term cranial growth patterns (head circumference and cranial length and breadth) significantly differed following this procedure in comparison to those of the control group. The cephalic indices remained constant; no significant changes were found in either study population. Fronto-orbital advancement in anterior craniosynostotic patients between the ages of 8 and 10 months seems to be ideal, as the procedure promotes solid cranial vault growth, ensuring positive surgical results.