Ongkosuwito, E.M.

Changes of mandibular ramal height, during growth in unilateral hemifacial microsomia patients and unaffected controls (Article)

2013-03-01T00:00:00Z

The aim of this study was to design mandibular ramal height growth curves for patients with HFM and compare those with the curves for a Dutch reference population. Two hundred fifty-one pre-operative orthopantomograms (OPTs) from 84 patients with unilateral HFM were used in conjunction with a control set of 2260 OPTs from 329 healthy individuals from the Nijmegen Growth Study (NGS) to determine mandibular ramal distances. For grades I/IIa and IIb/III, and for both sides, growth curves were constructed for mandibular ramal height with a linear curve-fitting procedure. This procedure revealed a significant difference between HFM patients and the NGS control group (p < 0.001); both in the mild and severe group mandibular ramal height differed significantly between the affected and non-affected side (p < 0.001). Growth was similar between HFM patients and the NGS control group. HFM patients therefore start with a smaller mandible and end with a smaller mandible, but experience growth similar to the Dutch normal population. These growth curves may aid the timing and determination of the combined surgical orthodontic treatment plan for HFM patients.

Craniofacial morphology in unilateral hemifacial microsomia (Article)

2012-11-30T00:00:00Z

Hemifacial microsomia (HFM) is a complex three-dimensional congenital condition that is characterized by mandibular hypoplasia and unilateral or bilateral microtia; although, other facial structures may be affected. Little is known about craniofacial growth and morphology in patients with HFM; therefore, we examined 75 HFM patients by means of a cephalometric analysis in a longitudinal study on serial lateral cephalograms. We hypothesized that the growth of several facial structures on both sides of HFM patients would be different compared to Dutch controls. We determined patients with HFM had more retruded mandibles and maxillae and a more vertical morphology compared to the reference population. In addition, there was a more retruded and vertical pattern on the affected side compared to the unaffected side and in patients with a severe condition compared to those with a mild condition. 'Mild' HFM patients were more similar to the Dutch reference population than the 'severe' HFM patients. Individual HFM growth curves showed very high inter-variability, further strengthening the need for individualized treatment plans that consider all three dimensions and the severity of the condition.

Aspects of Hemifacial Microsomia (Doctoral Thesis)

2012-11-20T00:00:00Z

Hemifaciale microsomia (HFM) is een aangeboren asymmetrische afwijking van het gelaat met grote variatie. Het ontstaan is onduidelijk. Buiten de craniofaciale (aangezichts) afwijkingen, komen ook andere defecten voor. Het klinisch beeld toont een enkelzijdige afwijking van het oor en een onderontwikkeling van het skelet, de weke delen en de aangezichtsspieren aan de aangedane gezichtshelft. Behandeling moet leiden tot een verbeterde functie en optimale gelaatssymmetrie aan het einde van de groei. Het uiteindelijke behandelresultaat wordt beïnvloed door externe factoren, zoals de chirurgische techniek, maar ook door intrinsieke patiënt factoren zoals genetische achtergrond, groei en ontwikkeling. Het eindresultaat is afhankelijk van het psychosociale welzijn van de patiënt en de psychosociale invloed van ouders op de patiënt. Een van de bestudeerde factoren is de aangezichtsgroei in HFM. We vonden dat patiënten meer terug liggende kaken hadden in vergelijking met de normale bevolking. De aangedane zijde vertoonde dit sterker dan de niet aangedane zijde. Voor de onderkaak geldt dat HFM patiënten starten met een kleinere onderkaak en ook eindigen met een kleinere onderkaak. Tijdens de groei blijft de verhouding tussen aangedane en niet aangedane zijde gelijk. Er is geen toenemende gezichtsasymmetrie zichtbaar. Deze kleinere onderkaak suggereert dat er ook een afwijking aan de lokale tandontwikkeling bestaat. We vonden een vertraagde tandontwikkeling in ernstig aangedane jonge HFM patiënten. Dit en de verdeling van aangeboren afwezige gebitselementen gerelateerd aan de ernst van de aandoening doen vermoeden dat er een vroege interactie bestaat tussen tand- en onderkaaksontwikkeling. De psychosociale implicaties van HFM op patiënt en ouders zijn onvoldoende bestudeerd. Voor het aanpassen aan de stressvolle gebeurtenis van het krijgen van een kind met een aangezichtsafwijking, zoals HFM, kan een grote rol zijn weggelegd voor verwerkingsstrategieën door de ouders. We onderzochten ouderlijke stress in relatie tot zowel kenmerken van het kind als de ouderlijke cognitieve verwerkingsprocessen. De manier van verwerking door ouders blijkt een groot deel van de ouderlijke stress te verklaren naast bepaalde kenmerken van hun kind. Vervolg onderzoek zou zich ook op functionele veranderingen moeten richten, zoals beweging van de onderkaak, esthetiek bij het lachen en de effectiviteit van kauwen waarbij rekening wordt gehouden met de drie dimensionale aard van de afwijking. Er kan dieper in gegaan worden op de manier waarop ouders verwerken dat zij een kind hebben met HFM en de strategie die zij daarvoor gebruiken. Dit kan het eindresultaat van de behandeling van hun kind beïnvloeden en daarmee, de kwaliteit van leven van hun kind.

Decreasing prevalence of oral cleft live births in the Netherlands, 1997-2006 (Article)

2011-05-01T00:00:00Z

Objectives: The number of new oral cleft patients has fallen in the Netherlands. This may be explained by two hypotheses: (1) greater prenatal detection of congenital anomalies has led to more pregnancy terminations and (2) increased folic acid use has reduced the oral cleft risk. Both hypotheses would mainly apply to the category cleft lip/alveolus ± cleft palate (CL±P), since, unlike cleft palate only (CP), CL±P can be detected prenatally by two-dimensional (2D) ultrasound and develops during the period recommended for folic acid use. The authors aimed to determine trends in prevalence over 1997-2006 and to evaluate the hypotheses by stratifying trends by cleft category. Methods: This study was a time-trend analysis of infants born alive with oral clefts in the Netherlands during 1997-2006 and registered in the national oral cleft registry. The authors calculated prevalence rates and the estimated annual percentage change (EAPC) for all oral clefts and the two categories. Results: In 1997-2006, 3308 infants out of 1 970 872 live births had oral clefts, an overall prevalence per 10 000 live births of 16.8 (CL±P: 11.3; CP: 5.5). Time-trend analysis showed that the prevalence of all oral clefts decreased (EAPC -1.8%; 95% CI: -3.0% to -0.6%), as did the CL±P prevalence (EAPC -2.3%; 95% CI: -3.8% to -0.9%). No significant trends were found for the CP prevalence. Conclusions: Because the live-birth prevalence of CL±P decreased, that of all oral clefts decreased. These findings are in line with both hypotheses and may therefore have implications for prenatal counselling and folic acid policy.

Dental development in hemifacial microsomia (Article)

2010-12-01T00:00:00Z

Hemifacial microsomia (HFM) is a congenital disorder marked by facial asymmetry. Whether facial asymmetry accounts for asymmetrical dental development is unknown. There are few data on dental development relative to mandibular development or severity of HFM, or on development over time. We hypothesized that when mandibular development was severely disturbed, local dental development was also affected. We compared dental development scores between affected and non-affected mandibular sides in patients with HFM (n = 84) and compared these data with those collected from Dutch control children (n = 451). Logistic functions were constructed for dental age over time for all four Pruzansky/Kaban types. The results showed a tendency toward delayed dental development in Pruzansky/Kaban types IIb and III at younger ages. The temporary delay of tooth formation in patients with severe forms of HFM and the distribution of agenic teeth suggest an interaction between mandibular and dental development.

Long-term functional outcome in 167 patients with syndromic craniosynostosis; Defining a syndrome-specific risk profile (Article)

2010-10-01T00:00:00Z

Objective: Little is known about the long-term prevalence of elevated intracranial pressure (ICP), obstructive sleep apnoea (OSA), level of education, language and motor skills, impaired sight and hearing in craniosynostosis syndromes. The objective of this study was to define the prevalence per syndrome of elevated ICP, OSA, impaired sight and impaired hearing. Methods: A retrospective study was undertaken on 167 consecutive patients diagnosed with Apert, Crouzon, Pfeiffer, Muenke or Saethre-Chotzen syndrome, aged 1-25 years and treated between 1983 and 2008. The mean age at time of referral and review was 1 years and 2 months and 10 years and 3 months, respectively. Results: Patients with Apert and Crouzon/Pfeiffer syndromes had the highest prevalence of elevated ICP (33% and 53%, respectively) and OSA (31% and 27%, respectively), while Saethre-Chotzen syndrome was also associated with a fair risk for elevated ICP (21%). The prevalence of impaired sight (61%) and hearing (56%) was high in all syndromes. Conclusion: Based on these data, a syndrome-specific risk profile with suggestions for screening and treatment is presented.

Validation of the NVSCA registry for common oral clefts: Study design and first results (Article)

2010-09-01T00:00:00Z

Objective: Since 1997 the Dutch Association for Cleft Palate and Craniofacial Anomalies (NVSCA) has maintained a national registry of congenital craniofacial anomalies. This study validates data on three common oral cleft categories (cleft lip/alveolus = CL/A; cleft lip/alveolus and palate = CL/AP; and cleft palate = CP) and general items. Design: Retrospective observational study. Setting: All 15 Dutch cleft palate teams registered presurgery patients with common oral clefts (n = 2553) from 1997 to 2003. Patients: A random sample of 250 cases was used; 13 cases were excluded. Main Outcome Measures: The corresponding medical data were reviewed; these medical data served to validate the NVSCA registry data. Prevalence comparisons, 2 X 2 tables and validity measures were performed. Results: The cleft categories most accurately recorded were CL/A and CP. Both categories had an observed agreement of 98%, kappa of 0.94, and a sensitivity and specificity of 97%. Cleft lip/alveolus and palate had an observed agreement of 95%, kappa of 0.89, a sensitivity of 90%, and a specificity of 99%. Regarding the general items, observed agreement and kappa were highest for adoption/foster child (99%; 0.76) and lowest for remarks about pregnancy (63%; 0.20). Sensitivity ranged from 25% (consanguinity) to 97% (white mother) and specificity was high for all items (>93%) except for white father and mother (approximately 35%). Conclusions: The NVSCA registry is a valuable tool for quality improvement and research because validity on all three common oral cleft categories is very good. Validity on the general items is reasonable to satisfying and appears to be related to the type of information.

Infant orthopedics and facial growth in complete unilateral cleft lip and palate until six years of age (Dutchcleft) (Article)

2009-12-04T00:00:00Z

Objective: To evaluate longitudinally the effect of infant orthopedics (IO) on dentofacial cephalometric variables in unilateral cleft lip and palate (UCLP) patients from 4 to 6 years of age. Design: Prospective two-arm randomized controlled clinical trial in three cleft palate centers in The Netherlands (Dutchcleft trial). Patients: Fifty-four children with complete UCLP. Interventions: Patients were divided randomly into two groups. Half of the patients (IO+) had IO until surgical closure of the soft palate at the age of 652 weeks; the other half (IO-) received no intervention. Mean Outcome Measures: Cephalometric values representing soft tissue, hard tissue, and dental structures, measured on lateral headfilms made at 4 and 6 years of age. Results: In the IO+ group, 21 patients were analyzed; in the IO- group, 20 patients were analyzed at age 4 and 22 at age 6. No differences were found between IO+ and IO-, except for two measurements: The interincisal angle was larger and the mentolabial angle was smaller in the IO+ group. Conclusions: For infants with UCLP whose surgical management included soft palate repair at 12 months and delayed hard palate closure, cephalometric outcomes at ages 4 and 6 provide no indication for the type of IO used in this study.

Distraction assisted treatment of a unilateral complex facial cleft (Article)

2009-07-01T00:00:00Z

Unilateral maxillary hypoplasia is a characteristic feature of an oblique facial cleft. This three-dimensional shortage of osseous structures and soft tissue becomes more prominent over the years and is difficult to correct. The authors describe a 17-year-old boy born with a unilateral nasomaxillary dysplasia and nasal dysplasia (Tessier type 1, 2, 3) who underwent a hemi-Le Fort III distraction with a rigid external distraction (RED) system. This distraction, in combination with initial peroperative advancement and retained with elastic traction with a facial mask, achieved a substantial horizontal improvement of the hemi-midface; this resulted in a better projection of the left cheek, infra-orbital rim, nasal ala, and improved occlusion. Owing to the underdevelopment of the maxilla and zygoma in all three dimensions, contour deformities remain. Creating several bone segments for multidirectional distraction would jeopardize vascularization of the bone. Good long-term planning is essential in these complicated cases, and more long-term results need to be assessed. The major reconstructive challenge for this complex pathology continues.

On the origin of bitemporal hollowing (Article)

2009-05-01T00:00:00Z

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.

Linear mandibular measurements: Comparison between orthopantomograms and lateral cephalograms (Article)

2009-03-01T00:00:00Z

Objective: To investigate the reliability of length measurements of the mandible by comparing orthopantomograms (OPTs) with lateral cephalograms. Design: Observational study. Setting: OPTs and lateral cephalograms were taken of 20 human dry skulls. Four orthodontists and four maxillofacial surgeons located landmarks on all radiographs using a computer program for cephalometric measurements. Intraobserver and interobserver variability in locating landmarks was assessed, as well as positioning of the skulls prior to radiography between the x-ray assistants. Magnification differences between the left and right side of the mandible on the OPT were determined for five skulls. Kappa statistics were used to calculate the intraclass correlation coefficient for intraobserver and interobserver differences. An F test was used to assess differences between methods and between type of observer. Results: No significant differences were found in the magnification factor of the left and right side of the mandible. Compared with a lateral cephalogram, the OPT had comparable reliability in measuring mandibular distances condyliongonion, gonion-menton, and condylion-menton. No significant differences were observed between the x-ray assistants in taking the OPTs and lateral cephalograms or in repositioning the skulls. Significant differences were found between orthodontists and maxillofacial surgeons for landmark measurements. Conclusion: An OPT is as reliable as a lateral cephalogram for linear measurements of the mandible (condylion-gonion, gonion-menton, and condylion-menton).

Surgically assisted rapid maxillary expansion in two cases of osteopathia striata with cranial sclerosis (Article)

2008-05-01T00:00:00Z

Osteopathia striata with cranial sclerosis (OS-CS) is a rare skeletal dysplasia characterized by linear striations of the long bones, osteosclerosis of the cranium, and extraskeletal anomalies. Osteosclerosis of the cranial and facial bones can lead to disfigurement and to disability due to the pressure on the cranial nerves. We report two cases of OS-CS where surgically assisted rapid maxillary expansion was performed for widening the extremely narrow maxilla. One should be aware of the disease-related problems and the possible complications that might occur with this type of patient.