An Analysis of Aesthetic Refinements in 120 Secondary Cleft Rhinoplasties.

BACKGROUND: Secondary cleft nose rhinoplasty remains a challenging procedure. Cartilage memory and scar contraction are problematic factors. The need for more detailed procedures for secondary reconstruction in this patient population has arisen. Contemporary refinements demonstrate a highly structured approach. We conducted a retrospective study evaluating the aesthetic results of cleft patients who underwent secondary rhinoplasty. METHODS: In a retrospective study, a photometric analysis of cleft patients operated in the period 2003-2011 was conducted. Reconstructive methods were documented. Pre- and postoperative photographs of cleft rhinoplasty patients were evaluated using a standardized protocol. Nostril width ratio, columellar angle, tip projection ratio, and nasolabial angle served as objective instruments. The Unilateral Cleft Lip Surgical Outcomes Evaluation score was chosen for external photometric rating and rated blindly by 2 external individual plastic surgeons as independent nonbiased reviewers. The interrater and intrarater reliabilities were calculated using the Cohen kappa coefficient (κ). RESULTS: A total of 120 secondary rhinoplasties in 85 uni- and bilateral cleft patients could be included. Mean follow-up was 20 months. A total of 60 (71%) patients needed additional bone grafting (chin/pelvis), and 23 (27%) patients a LeFort I osteotomy. In one third of the secondary rhinoplasties, a medial and/or lateral osteotomy was performed (34%). In one fourth (24%), an external septoplasty was considered necessary. In 55% (47 patients) of the cases, a columellar strut was used. Excluding bone grafts, a total of 173 other grafts (mean of 2 grafts/patient) were applied. Postoperative measurements for nostril width ratio and columellar angle were statistically significant. A structured approach with contemporary refinements is described in detail. Intra- and interrater reliabilities for photometric assessment according to the Unilateral Cleft Lip Surgical Outcomes Evaluation score are shown. CONCLUSIONS: A structured approach for secondary cleft rhinoplasty yields satisfying, reproducible, and stable results.

Rotational Advancement in Cleft Nose Rhinoplasty - Buccal Mucosal Grafts Serve as a Powerful Tool.

OBJECTIVE: Our aim was to analyze our technique of a modified rotational advancement in conjunction with buccal mucosal grafts (BMGs) in a subgroup of severe cleft cases. DESIGN: A retrospective clinical and photographic evaluation was conducted. Columella angle (CA) and tip projection (TPR) served as instruments in a photometric analysis. SETTING: Academic university hospital and specialized craniofacial cleft center. PATIENTS: At the time of the secondary rhinoplasty, 61 cleft patients were included, all 17 years or older. INTERVENTIONS: Rotational advancement with usage of BMGs was performed in selected cases by a single surgeon. MAIN OUTCOME MEASURES: Nasal symmetry and aesthetic appearance. RESULTS: From 2003 to 2011, 29 unilateral severe cleft cases (group I) underwent a modified alar rotational advancement with BMGs. Group II, with 32 cases, represented patients without BMGs. Technique and management of BMGs were described in detail. The complication rate of donor and recipient site presented as very low. The CA was improved significantly in both groups. Also, TPR improved (not significantly) in group I. Using our technique, we considerably enhanced the aesthetic results and symmetry in secondary cleft rhinoplast. CONCLUSIONS: Rotating the vestibular skin makes it possible to eliminate the traction of this skin on the repositioned alar cartilage, therefore achieving a more pleasing and stable nasal symmetry in secondary cleft rhinoplasty. Versatile BMGs close this gap, thus making them a powerful tool in the arsenal of the reconstructive cleft surgeon.

Metopic synostosis.

Premature closure of the metopic suture results in a growth restriction of the frontal bones, which leads to a skull malformation known as trigonocephaly. Over the course of recent decades, its incidence has been rising, currently making it the second most common type of craniosynostosis. Treatment consists of a cranioplasty, usually preformed before the age of 1 year. Metopic synostosis is linked with an increased level of neurodevelopmental delays. Theories on the etiology of these delays range from a reduced volume of the anterior cranial fossa to intrinsic malformations of the brain. This paper aims to provide an overview of this entity by giving an update on the epidemiology, etiology, evolution of treatment, follow-up, and neurodevelopment of metopic synostosis.

Papilledema in isolated single-suture craniosynostosis: prevalence and predictive factors.

The purpose of this retrospective study was to assess the prevalence of papilledema in patients with isolated craniosynostosis. Second, we wanted to assess if the presence of ventricular dilatation on computed tomography (CT) scan is a predictive factor for the occurrence of papilledema. We included 205 consecutive children with an isolated single-suture craniosynostosis, who had at least 1 fundus examination. Preoperative CT scans of the brain were examined for the presence of ventricular dilatation.Papilledema developed in 14 of 205 patients: 10 developed papilledema before surgery and 4 during the follow-up period. Ten of the patients with papilledema had a synostosis of the sagittal suture, and 4 of the metopic suture. Prevalence of papilledema in scaphocephaly was 9.7%, and in trigonocephaly, 5.6%. Based on evaluation of all CT scans, ventricular dilatation seemed not to be a predictive factor for papilledema in children with isolated craniosynostosis.The incidence of papilledema in almost 10% of scaphocephaly patients is remarkably higher than expected. Therefore, we recommend routine preoperative screening, especially for patients with scaphocephaly, but also for patients with trigonocephaly. Postoperative screening is recommended in all patients when there is any uncertainty.

On the origin of bitemporal hollowing.

INTRODUCTION: Long-term results after cranioplasty for trigonocephaly often show bitemporal hollowing and a residual hypotelorism. Both findings fuel the perception that the growth of the periorbital region and the forehead as a whole continues to be restricted, even after correction. The aim of this study was to evaluate the growth process of the periorbital region after correction for trigonocephaly in the long term. MATERIALS AND METHODS: From 1972 to 2004, 184 patients underwent a cranioplasty for the correction of nonsyndromatic trigonocephaly. Cephalometric analysis was performed in 33 of these patients who had their radiographs taken on the same day as the photograph, at least 1 year postoperative and before the age of 6 years. Cephalic landmarks were used to analyze the growth of the forehead. Because of the lack of standardized cephalograms, growth ratios were used instead of absolute measurements. For visual analysis, normal anteroposterior photographs were used, which were taken on the same day as the radiograph. Two observers evaluated the anteroposterior photographs for the presence and level of temporal hollowing. A score of 0 (normal), 1 (moderate deformity), or 2 (severe deformity) was assigned to each of the photographs. RESULTS: A significant relation was found between a severe deformation seen at postoperative photographic evaluation and a lower growth ratio. The preoperative photo score was not of predicting value for the postoperative growth ratio and therefore, indirectly, for the postoperative photo score. The mean preoperative photo score dropped 5% after surgery. The age at operation had no influence on this postoperative photo score. The experience of the surgeon, however, was a significant contributing factor. CONCLUSIONS: Temporal hollowing seems to be of bony origin and can be explained by skeletal growth inhibition in the affected area. When present immediately after operation, they seem to persist through the years, which makes surgical skill another factor of importance.

The increase of metopic synostosis: a pan-European observation.

Metopic synostosis is thought to have an incidence of about 1 in 15,000 births. Traditionally, this makes it the third most frequent single-suture craniosynostosis, after scaphocephaly (1 in 4200-8500) and plagiocephaly (1 in 11,000). Our units have, independently from each other, noted a marked increase in the number of metopic synostosis over the recent years. This is a pan-European, retrospective epidemiological study on the number of cases with metopic synostosis born between January 1, 1997, and January 1, 2006. This number was compared to the prevalence of scaphocephaly, the most frequently seen craniosynostosis. In the 7 units, a total of 3240 craniosynostosis were seen from 1997 until 2006. Forty-one percent (n = 1344) of those were sagittal synostosis, and 23% (n = 756) were metopic synostosis. There was a significant increase of the absolute number as well as of the percentage of metopic synostosis over these years (regression analysis, P = 0.017, R2 = 0.578) as opposed to a nonsignificant increase in the percentage of sagittal synostosis (P > 0.05, R2 = 0.368). The most remarkable increase occurred around 2000-2001, with the average of metopics being 20.1% from 1997 to 2000 and 25.5% from 2001 to 2005 (independent t-test, P = 0.002). The sagittal synostosis showed a smaller and nonsignificant increase in the same years: from 39.9% in 1997-2000 leading up to 42.5% in 2001-2005 (independent t-test, P > 0.05). The number of metopic synostosis has significantly increased over the reviewed period in all of our units, both in absolute numbers as in comparison to the total number of craniosynostosis.

Prenatal ultrasound parameters in single-suture craniosynostosis.

INTRODUCTION: Although single-suture craniosynostosis is diagnosed sporadically during pregnancy, timely referral is critical for its treatment. Additionally, craniosynostosis leads to increased maternofetal trauma during birth. In the Netherlands, 95% of pregnant women receive a standard ultrasound at around 20 weeks of gestation, potentially an ideal setting for detecting craniosynostosis prenatally. To enhance the prenatal detection of the metopic and the sagittal suture synostosis, we wished to identify new screening parameters. MATERIALS AND METHODS: We retrospectively analyzed data of the 20-week anomaly scan in trigonocephaly patients (n = 41), scaphocephaly patients (n = 41), and matched controls (n = 82). We measured six different cranial dimensions, including head circumference, biparietal diameter, and occipito-frontal diameter, defining the cephalic index as the ratio between biparietal and occipito-frontal diameter. RESULTS: Prenatal biometric measurements did not differ significantly between trigonocephaly patients and controls. Although significantly lower in scaphocephaly patients (0.76 versus 0.79; p = .000), the cephalic index by itself is not appropriate for screening at 20 weeks of gestation. Longitudinal analysis suggests that a deflection in BPD curve is found in scaphocephaly patients, starting at 20 weeks of gestation. CONCLUSIONS: Prenatal biometric measurements do not differ significantly between trigonocephaly patients and controls. The CI is lower in scaphocephaly patients. A deflection in BPD curve should be followed by 3 D imaging of the cranial sutures.

Insight into the Pathophysiologic Mechanisms behind Cognitive Dysfunction in Trigonocephaly.

BACKGROUND: The relationship between trigonocephaly and cognitive problems might be explained by: secondary mechanical factors related to growth restriction of the skull, and primary structural defects caused by a shared mechanism related to brain developmental disorder(s) and skull malformation. However, because the exact pathophysiology remains unknown, we examined the pathophysiologic mechanisms behind cognitive dysfunction in patients with trigonocephaly, with an aim of providing a model for cognitive dysfunction based on routinely available variables. METHODS: Included were 72 patients with trigonocephaly who were operated on. Postoperatively, intelligence was assessed prospectively. The two independent variables, secondary mechanical and primary brain developmental mechanisms, were evaluated retrospectively. Computed tomographic imaging was used to assess skull volume and severity of the frontal stenosis (secondary mechanical factors), width of the central part of the lateral ventricles, and other structural brain anomalies (primary brain developmental factors). Extracranial congenital anomalies were also taken into account. RESULTS: No association was found between secondary mechanical factors and postoperative IQ score. Width of the central part of the lateral ventricles, and an interaction effect between this width and additional extracranial anomalies, showed a significant negative association with postoperative IQ. CONCLUSIONS: Primary brain developmental disorders seem to play an important role in the development of cognitive problems in trigonocephaly. Assessment of width of the central part of the lateral ventricle scores and additional extracranial congenital anomalies for the early prediction of cognitive problems in patients with trigonocephaly could be clinically valuable and can be performed using routinely available tools.

Prevention of halo pin complications in post-cranioplasty patients.

INTRODUCTION: Distraction Osteogenesis has been successfully implemented in the treatment of maxillary hypoplasia. By using the Rigid External Distraction device (RED) the maxilla can be advanced without the need for bone grafts, providing more stability to the repositioned maxilla. BACKGROUND: The introduction of the RED system in craniofacial surgery has given rise to previously unseen problems. AIM: To provide a set of protocol improvements that might prevent the intracranial pin migration seen at the removal of a RED-II in one patient. CONCLUSIONS: Although the RED device has been shown to achieve good clinical results, there are some disadvantages to the system. There is a high incidence of pin tract infections - leading to loosening of the pins and loss of rigidity. Also, the external ring is prone to traumatic injury. Furthermore, the positioning of the pins can be difficult in thin bone. The improvements used in our protocol might prevent this complication.

Nasal sequelae of Treacher Collins syndrome.

OBJECTIVE: This study aimed to determine external and endonasal deformity, and satisfaction with nasal functioning and appearance, in Treacher Collins syndrome. STUDY DESIGN: A cross-sectional cohort study was conducted. METHODS: Eleven adult patients with Treacher Collins syndrome were compared with 151 controls in terms of satisfaction with nasal functioning and appearance by means of the Nasal Appearance and Function Evaluation Questionnaire. In all patients with Treacher Collins syndrome, external nasal deformities were scored on standardized digital photographs of the nose as rated independently by three experienced physicians. Endonasal deformity was determined by standardized nasal endoscopy. RESULTS: The patients were relatively satisfied with the various esthetic nasal subunits. The most significant functional problems were snoring (P = 0.001) and quality of phonation (P = 0.003). The main external nasal deformities were the dorsal hump (73%), external deviation (≤55%), the bifid or bulbous nasal tip (55%), and columellar septal luxation (55%). In 82% of the patients, a septal deviation was found, often associated with spurs. CONCLUSION: Satisfaction with esthetics of the nose was fair, but these patients suffer from the functional problems of snoring and impaired quality of phonation. A structured nasal ENT physical examination with nasal endoscopy might determine aspects requiring more attention during treatment. Septorhinoplasty can be performed at an adult age if there is a considerable esthetic wish of the patient and/or nasal obstruction combined with septal deviation. Attention should be paid to dorsal hump reduction, correction of the deviated external osseous framework, septoplasty, and correction of the nasal tip shape. LEVEL OF EVIDENCE: 2b.

Unilateral versus bilateral correction of unicoronal synostosis: an analysis of long-term results.

INTRODUCTION: Hollowing of the temporal region is a common problem after cranioplasty for unicoronal synostosis. In this study, first, the development of temporal hollowing pre- and postoperatively is evaluated. Second, the origin of temporal hollowing is investigated by comparing two operative techniques. Ultimately, the relation between timing of surgery and the development of hollowing is investigated. METHODS: From 1979 to 2010, 194 patients with unicoronal synostosis were operated at our center. Patients were treated with a unilateral or bilateral correction of the supraorbital rim. A total of 48 patients qualified for the present study. Mean age at follow-up was 7.5 years. Cephalic landmarks were identified on radiographs prior to and after surgery to determine the growth of the forehead. For visual analysis, two independent observers evaluated normal photographs for the presence and severity of temporal hollowing. RESULTS: Preoperative osseous asymmetry improved significantly after surgery. A total of 21 patients show an increase of temporal hollowing on photographs after surgery (46%). In 35 out of 48 patients, postoperative temporal hollowing was noted (73%). Bilaterally treated patients showed more severe temporal hollowing compared to unilaterally treated patients, though not significantly (23% vs. 6%, p = 0.229). Timing of surgery (before or after the age of 1 year) did not influence the occurrence of severe temporal hollowing. CONCLUSIONS: Fronto-supraorbital advancement was unable to achieve normal growth in the temporal region in a large proportion of patients, although more symmetry was achieved. The operative technique itself did not seem to influence the occurrence of temporal hollowing, nor did the timing of surgery.

Cognitive and behavioral functioning in 82 patients with trigonocephaly.

BACKGROUND: The main objective of the present study was to assess the prevalence rates of attention deficit hyperactivity disorder, oppositional defiant disorder, conduct disorder, and features of autism spectrum disorders in trigonocephalic patients, using validated instruments and by ruling out the confounding influence of IQ. The second aim was to assess the association between extracranial anomalies and cognitive and/or behavioral problems in patients with trigonocephaly. METHODS: Objectives were studied in 82 trigonocephalic patients aged 4 to 18 years at the Erasmus Medical Center in Rotterdam, The Netherlands. Features of autism spectrum disorders were assessed using the Social Communication Questionnaire. Attention deficit hyperactivity disorder, oppositional defiant disorder, and conduct disorder were assessed with the Diagnostic Interview Schedule for Children-Parent Version. The presence and nature of extracranial anomalies were ascertained by a clinician. RESULTS: Mental retardation (IQ <70) was present in 9 percent of patients with trigonocephaly. Findings indicated a 70 percent versus 24 percent prevalence of psychopathology (attention deficit hyperactivity disorder, oppositional defiant disorder, conduct disorder, or features of autism spectrum disorder) in patients with IQ levels of, respectively, <85 and ≥85. In the latter group, psychopathology was not significantly more common than expected based on prevalence rates reported in community samples. Extracranial anomalies were significantly correlated with lower IQ levels. However, when adjusted for IQ, the presence of extracranial malformations was not associated with an increased risk of behavioral problems. CONCLUSION: The relatively high prevalence of behavioral problems in patients with trigonocephaly seems to be mainly attributable to the co-occurrence of trigonocephaly and low intelligence.

Long-term results after 40 years experience with treatment of rare facial clefts: Part 1--Oblique and paramedian clefts.

BACKGROUND: Oblique and paramedian rare facial clefts impose a major reconstructive challenge and long-term assessments of the outcomes remain scarce. This study provides new details regarding surgical techniques and timing, influence of growth, and difficulties of this pathology on the long-term; a guideline for surgical treatment is given. METHODS: Twenty-nine adults with an oblique or paramedian facial cleft and surgically treated in the authors' unit between 1969 and 2009, were included. The long-term evaluation was based on series of photographs, 3D-CT's, X-rays, operation data, and was specified per facial area. RESULTS: The mean number of performed operations per patient was 10.6 (range: 1-26). Vertical dystopia is not caused by previous surgery, but by growth deficiencies of the maxilla. In all patients with vertical dystopia, its presence and severity were clear at the age of five, and it should ideally be treated shortly after that age. In mild cases grafting seems sufficient, but in more severe cases orbital translocation is necessary. Costochondral grafts showed the best long-term results in both orbital and nasal reconstructions. Major nose reconstruction is best delayed until adolescence. For an optimal final result in selected cases, correction of midface hypoplasia at adolescence is necessary. CONCLUSION: The three-dimensional underdevelopment of the midface region plays a central role in the deformities of most patients, but is complex and difficult to correct. The provided guideline should help to minimize the number of operations and ameliorate long-term results.

Long-term results after 40 years experience with treatment of rare facial clefts: Part 2--Symmetrical median clefts.

BACKGROUND: Median facial clefts are reconstructive challenges, requiring multiple operations throughout life. Long-term results are often still far from ideal and could be improved. Due to surgical intervention and diminished intrinsic growth potential, surgical results may change from initially good into a progressively disappointing outcome. If, however, the ideal timing and type of surgery are known, in combination with the intrinsic growth potential, the results can be ameliorated. A guideline for surgical treatment is given. METHODS: Twenty patients with a pure symmetrical median cleft were evaluated on intermediate and long-term surgical results. The final result was scored based on severity of the initial and the remaining facial deformities, and the need for revisional surgery. RESULTS: The long-term surgical outcome was initially good for each of the affected facial parts and the face in general, but worsened over time, especially in the zone of the nose. An adequate and stable result of hypertelorism correction was observed for both the orbital box osteotomy and medial faciotomy, even when performed at a young age. CONCLUSIONS: The intrinsic growth restriction is mainly localised in the central midface. This leads to a complex and often unpredictable growth of the maturing face. It makes it difficult to achieve perfect reconstructions. Caution with surgical interventions of the nose at a young age is required. Once the face has matured, a midface advancement and secondary nose correction should be considered for satisfactory projection. Early referral to a specialised centre is essential.

Guidelines for reconstruction of the eyelids and canthal regions.

A large number of reports are available on the options for reconstructing specific defects of the eyelids or (peri) orbital area, which may complicate decision making in choosing the most optimal technique for a particular defect. Based on more than 40 years' experience in reconstruction of eyelids and periorbital defects, combined with an extensive literature review, general principles on reconstruction are presented and illustrated. Surgical techniques are outlined with respect to anatomical layer, depth, size and location of the defect. Adherence to specific principles for eyelid and canthal reconstruction will lead to predictable, stable and functionally good results.

Internal carotid dissection after Le Fort III distraction in Apert syndrome: a case report.

A 10-year-old girl with Apert syndrome underwent a Le Fort III osteotomy with the positioning of internal and external distraction devices. The operation was straightforward with no intraoperative complications. Very soon after completion of surgery an anisocoria (unilateral dilation of a pupil) was noticed. This was followed by intracranial oedema which was fatal. The aetiology was dissection of the right internal carotid artery is reported. The complications of Le Fort osteotomies are discussed regarding patients with complex syndromal craniosynostosis and midface hypoplasia, such as Apert syndrome.

Unblocking the nose by unplugging the ear: the use of foam ear defenders as a splint following secondary cleft rhinoplasty.

OBJECTIVE: The functional and aesthetic result from secondary cleft rhinoplasty is commonly impaired by a bulge in the lateral nasal vestibule, which can relapse in the postoperative period despite careful intraoperative correction. We aim to improve our results by using a foam ear defender as a postoperative splint to prevent relapse. DESIGN: Case series of 10 patients with photographs of a typical clinical case. INTERVENTIONS: The postoperative nasal pack was exchanged with a foam ear defender on day 5. The splint was worn continuously for 3 to 4 weeks, then nightly for 3 months. The splint was changed daily by the patient. MAIN OUTCOME MEASURES: The results were assessed clinically by the senior author. RESULTS: The patients had little or no recurrence of the lateral vestibular bulge following nasal splintage. CONCLUSIONS: Foam ear defenders are a simple, cheap, and comfortable method to provide splintage to the nasal vestibule following secondary cleft rhinoplasty.

The risk of psychopathology in children with craniosynostosis.

BACKGROUND: The purpose of this study was to assess the prevalence of behavioral and emotional problems in patients with craniosynostosis and to determine the prospective association of a beaten-copper pattern before 18 months of age with behavioral and emotional problems in patients with craniosynostosis. METHODS: The authors performed a follow-up study of 115 craniosynostosis patients at the Erasmus Children's University Hospital in Rotterdam. Behavioral and emotional problems were assessed with the Child Behavior Checklist at a mean age of 8 years. The presence of beaten-copper pattern before the age of 18 months was assessed on presurgical radiographs. Analyses were adjusted for intelligence quotient. RESULTS: Whereas craniosynostosis patients with intelligence quotients of 85 or greater did not differ from children in the normal group, craniosynostosis patients with intelligence quotients less than 85 had a higher risk of behavioral and emotional problems. However, these results were comparable to the findings of other studies assessing psychopathology in children with lower intelligence levels. Type of craniosynostosis (single suture versus complex) and a beaten-copper pattern before the age of 18 months did not affect the risk for behavioral and emotional problems in children with craniosynostosis. CONCLUSION: When intelligence is taken into account, craniosynostosis is not associated with an increased risk of behavioral and emotional problems, nor is type of craniosynostosis or a beaten-copper pattern before the age of 18 months.

Early beaten-copper pattern: its long-term effect on intelligence quotients in 95 children with craniosynostosis.

OBJECT: The aim of this study was to analyze the presence of beaten-copper patterns (BCPs) in children with craniosynostosis before 18 months of age and its association with their IQ at a later age. METHODS: The authors conducted a retrospective analysis of 538 cephalograms (obtained at a mean patient age of 1.16 years) from 95 patients. The BCP location and percentage of brain surface area covered were related to patient IQ scores obtained by the same psychologist using the Snijders-Oomen Nonverbal Intelligence Test-Revised, 51/2-17, and the Wechsler Preschool and Primary Scale of Intelligence-Revised. RESULTS: As much as 71.6% of patients presented with a BCP before 18 months of age (mean surface area of BCP 20.3%, 93% of patients presented with bilateral BCPs). The mean IQ was 95 +/- 21.3 (range 50-136) at a mean patient age of 8.4 +/- 2.59 years. There was a significant increase in the surface area covered by BCPs in the first 3 years of life (p < 0.001) and a significant difference in IQs between syndromic (30 cases, mean IQ 88.9) and nonsyndromic craniosynostosis cases (54 cases, mean IQ 98.9, p = 0.03). No significant correlation was found between IQ and the appearance of BCPs on presurgery radiographs (Pearson correlation coefficient = 0.143, p = 0.19) or their location (Spearman rank correlation coefficient = 0.091, p = 0.45). The BCPs appeared predominantly in the occipital region (41.1%). CONCLUSIONS: Although the radiographic appearance of a BCP before the age of 18 months is an uncommon finding in healthy children, a craniosynostosis study group showed a preoperative BCP incidence of 71.6% and an increased incidence during the period of rapid brain expansion in the first 3 years of life. Note, however, that the presence of such a pattern had no significant long-term effect on patient intelligence levels.