Modelling growth curves of the normal infant's mandible: 3D measurements using computed tomography.

OBJECTIVES: Data on normal mandibular development in the infant is lacking though essential to understand normal growth patterns and to discriminate abnormal growth. The aim of this study was to provide normal linear measurements of the mandible using computed tomography performed in infants from 0 to 2 years of age. MATERIAL AND METHODS: 3D voxel software was used to calculate mandibular body length, mandibular ramus length, bicondylar width, bigonial width and the gonial angle. Intra- and inter-rater reliability was assessed for these measurements. They were found to be sufficient for all distances; intra-class correlation coefficients were all above 0.9. Regression analysis for growth modelling was performed. RESULTS: In this multi-centre retrospective study, 109 CT scans were found eligible that were performed for various reasons (e.g. trauma, craniosynostosis, craniofacial abscesses). Craniosynostosis patients had larger mandibular measurements compared to non-craniosynostosis patients and were therefore excluded. Fifty-one CT scans were analysed. CONCLUSIONS: Analysis showed that the mandible increases more in size vertically (the mandibular ramus) than horizontally (the mandibular body). Most of the mandibular growth occurs in the first 6 months. CLINICAL RELEVANCE: These growth models provide insight into normal mandibular development in the first 2 years of life. This reference data facilitates discrimination between normal and abnormal mandibular growth.

Management and outcomes of obstructive sleep apnea in children with Robin sequence, a cross-sectional study.

OBJECTIVE: The objective of this cross-sectional study is to assess the prevalence, course, and management of obstructive sleep apnea (OSA) in children with Robin sequence (RS) aged 1-18 years. MATERIALS AND METHODS: A cross-sectional study was conducted in 63 children aged 1 to18 years with RS. Patient data were collected on baseline characteristics and management. OSA was evaluated by polysomnography. RESULTS: Sixty-three children with RS were included (median age 8.0 years) and divided into two groups based on the initial treatment: prone positioning or respiratory support. Respiratory support was more often indicated in children with a non-isolated RS (p < 0.05). At cross section, in the prone positioning group (n = 32), one child was diagnosed with OSA. In the respiratory support group (n = 31), 13 children (42 %) had respiratory problems of whom 10 needed respiratory support. CONCLUSIONS: Between the age of 1 and 18 years, almost one out of four children with RS still has respiratory problems. Children with RS, who can be treated with prone positioning only as an infant, are not likely to develop obstructive airway problems at a later age. In contrast, children who need respiratory support early after birth are at risk of continuing or re-developing OSA after the age of 1 year. CLINICAL RELEVANCE: This study shows that those who need respiratory support at an early age need careful monitoring until adulthood.

Respiratory Distress Following Cleft Palate Repair in Children With Robin Sequence.

Objective The aim of this study is to assess prevalence, severity, and duration of respiratory distress following palatoplasty in children with Robin sequence and to evaluate perioperative management. Design Retrospective chart review study. Data were collected for patients who were born between 2009 and 2012 and underwent palatoplasty in the Sophia Children's Hospital-Erasmus Medical Center. Results Of the 75 patients with cleft palate, 30 with Robin sequence and a control group of 45 cleft without Robin sequence underwent palatoplasty. Prior to closure, 26 of 30 patients with Robin sequence had been treated by prone positioning, and four needed additional treatment. The mean age at closure was 12.4 months for patients with Robin sequence and 10.9 months for patients without Robin sequence (P = .05). On the basis of the results of preoperative polysomnography with palatal plate, closure was postponed in two patients with Robin sequence. In the Robin sequence group, eight of the 30 patients developed postoperative respiratory distress within 48 hours and one patient, after 7 days; whereas none within the non-Robin sequence group developed respiratory distress. In all nine cases of Robin sequence the obstructive problems resolved within a few days, with four children requiring a temporary nasopharyngeal tube (NPT). There were no significant differences between preoperative polysomnography results of the nine patients with Robin sequence who developed postoperative respiratory distress compared with those patients with Robin sequence who did not. Conclusion Despite delayed closure compared with children without Robin sequence, 30% of the children with Robin sequence developed respiratory distress following palatoplasty, which resolved within a few days. This study emphasizes the need for close perioperative monitoring of patients with Robin sequence who undergo palatoplasty.

Quality of life in children with Robin Sequence.

INTRODUCTION: Parents may play an important role during the management of children with Robin Sequence (RS). However, so far only one study has been done on both parent-reported health-related quality of life (HRQoL) and obstructive sleep apnea (OSA) symptoms in children with RS. METHODS: Overall, 63 children with RS, aged 1 and 18, were included in this cross-sectional study. Fifty-three parents of children with RS with a median age of 8.9 [IQR 5.1-12.7] completed questionnaires on HRQoL (OSA-18) and symptoms of OSA (the Brouillette score) in their child with RS. Ten children between 12 and 18 years filled out the self-reported HRQoL questionnaire OSA-12. RESULTS: At cross-section, 10 children still had respiratory problems. Overall, parents of children with RS reported a lower HRQoL in their child compared with parents in the general population. Parents of children with RS who still had respiratory problems, i.e. OSA or airway support, reported significantly worse OSA-18 scores compared with parents of RS children without OSA. Children with RS themselves (n = 10) reported less physical suffering and less emotional distress on the OSA-12 compared with children in the norm population. Parental perceptions of OSA-related symptoms were accurate, except for the incidence of snoring. In assessing snoring, the multidimensional OSA-18 sleep domain was more informative. CONCLUSIONS: Parents of children with RS reported a lower HRQoL in their child compared with parents in the general population. Parental perceptions of health and HRQoL in children with RS might have an additional value to recognize and treat respiratory problems.

Non-surgical and surgical interventions for airway obstruction in children with Robin Sequence.

There is widespread lack of consensus regarding treatment of airway obstruction in children with Robin Sequence. This study aimed to systematically summarize outcomes of non-surgical and surgical options to treat airway obstruction in children with Robin Sequence. The authors searched the Medline, EMBASE and CENTRAL databases. Studies primarily on mandibular distraction were excluded. Study quality was appraised with the Methodological Index for Non-Randomized Studies (MINORS) score. Forty-eight studies were included, of which 45 studies had a retrospective non-comparative set up, two studies had a prospective design and one study was a clinical trial. The mean MINORS score was 7.3 (range 3-10). The rates of successful relief of the airway obstruction (SRoAO) were: not available for orthodontic appliance (2 studies, n = 24), 67-100% for nasopharyngeal airway (6 studies, n = 126); 100 % for non-invasive respiratory support (2 studies, n = 12); 70-96% for tongue-lip adhesion (11 studies, n = 277); 50-84% for subperiosteal release of the floor of the mouth (2 studies, n = 47); 100% for mandibular traction (3 studies, n = 133); 100% for tracheostomy (1 study, n = 25). The complication rate ranged from zero to 55%. Although SRoAO rates seemed comparable, high-level evidence remains scarce. Future research should include description of the definition, treatment indication, and objective outcomes.

Treacher Collins Syndrome: A Systematic Review of Evidence-Based Treatment and Recommendations.

BACKGROUND: No reviews or guidelines are available on evidence-based treatment for the multidisciplinary approach in Treacher Collins syndrome. The authors' aim is to provide an evidence-based review of multidisciplinary treatment of Treacher Collins syndrome based on levels of evidence and supported with graded recommendations. METHODS: A systematic search was performed by means of the PubMed, Web-of-Science, Embase, and Cochrane Central databases (1985 to January of 2014). Included were clinical studies (with five or more Treacher Collins syndrome patients) related to therapy, diagnosis, or risk of concomitant diseases. Level of evidence of the selected articles was rated according to the American Society of Plastic Surgeons evidence-based clinical practice guidelines. After two panelists had reviewed each abstract separately, a consensus method was used to solve any disagreements concerning article inclusion. RESULTS: Of the 2433 identified articles, 63 studies (Level of Evidence II through V) were included. Conclusions and recommendations were extracted consecutively for the following items: upper airway; ear, hearing, and speech; the eye, eyelashes, and lacrimal system; growth, feeding, and swallowing; the nose; psychosocial factors; and craniofacial reconstruction. CONCLUSIONS: In this systematic review, current evidence for the multidisciplinary treatment of Treacher Collins syndrome is provided, recommendations for treatment are made, and a proposed algorithm for treatment is presented. Although some topics are well supported, others, especially ocular, nasal, speech, feeding, and swallowing problems, lack sufficient evidence. In addition, craniofacial surgical reconstruction lacks a sufficient level of evidence to provide a sound basis for a full treatment protocol. Despite the rarity of the syndrome, more research is needed to compare outcomes of several surgical treatments, especially in orbitozygomatic/maxillary regions.

Robin sequence: A European survey on current practice patterns.

To provide an overview of current practice patterns with regard to Robin sequence (RS) patients in Europe, a survey was conducted among European clinicians. This online survey consisted of different sections assessing characteristics of the respondent and clinic, definition, diagnosis, treatment, and follow-up. In total, surveys from 101 different European clinics were included in the analysis, and 56 different RS definitions were returned. The majority (72%) of the respondents used a sleep study system to determine the severity of the airway obstruction. A total of 63% used flexible endoscopy and 16% used rigid endoscopy in the diagnostic process. Treatment of the airway obstruction differed considerably between the different countries. Prone positioning for mild airway obstruction was the treatment modality used most often (63%). When prone positioning was not successful, a nasopharyngeal airway was used (62%). Surgical therapies varied considerably among countries. For severe obstruction, mandibular distraction was performed most frequently. Three-quarters of the respondents noted the presence of catch-up growth in their patient population. This first European survey study on definition and management of RS shows that there are considerable differences within Europe. Therefore, we would encourage the establishment of national (and international) guidelines to optimize RS patient care.

Unravelling Robin sequence: considerations of diagnosis and treatment.

OBJECTIVES/HYPOTHESIS: The airway management of children with Robin sequence is controversial. This study provides an overview of a single-center experience with the diagnosis and treatment of 59 children with Robin sequence. STUDY DESIGN: Retrospective cohort study. METHODS: We conducted a retrospective cohort study of 59 children (<1 year old) with Robin sequence managed between 2000 and 2010. Robin sequence was defined as the presence of mandibular hypoplasia and clinical signs of airway obstruction. Data were collected on demographic characteristics, the presence of a syndrome, the perinatal period, and the management of airway and nutritional problems. RESULTS: Eighteen children (31%) needed respiratory support because of severe respiratory distress, and a sleep study found obstructive apneas in another eight children who had been managed by prone positioning and/or monitoring. In the isolated group significantly fewer children needed respiratory support compared to the nonisolated group. After the age of 1 year, 10% of the Robin sequence cohort was still in need of treatment for obstructive symptoms. Almost half (47%) needed temporary nutritional support. CONCLUSIONS: The prevalence of respiratory distress in children with Robin sequence is high. In most children, treatment with prone positioning was sufficient to relieve the airway obstruction. Successful treatment with prone positioning was significantly more often seen in children with an isolated Robin sequence. About one-third of all Robin sequence children needed respiratory support in the neonatal and/or infant period. However, in childhood, only 10% of the total Robin sequence cohort was still dependent on respiratory support.

Long-term assessment of facial features and functions needing more attention in treatment of Treacher Collins syndrome.

AIM: This study aimed to determine which facial features and functions need more attention during surgical treatment of Treacher Collins syndrome (TCS) in the long term. METHOD: A cross-sectional cohort study was conducted to compare 23 TCS patients with 206 controls (all≥18 years) regarding satisfaction with their face. The adjusted Body Cathexis Scale was used to determine satisfaction with the appearance of the different facial features and functions. Desire for further treatment of these items was questioned. For each patient an overview was made of all facial operations performed, the affected facial features and the objective severity of the facial deformities. RESULTS: Patients were least satisfied with the appearance of the ears, facial profile and eyelids and with the functions hearing and nasal patency (P<0.001). Residual deformity of the reconstructed facial areas remained a problem in mainly the orbital area. The desire for further treatment and dissatisfaction was high in the operated patients, predominantly for eyelid reconstructions. Another significant wish was for improvement of hearing. CONCLUSION: In patients with TCS, functional deficits of the face are shown to be as important as the facial appearance. Particularly nasal patency and hearing are frequently impaired and require routine screening and treatment from intake onwards. Furthermore, correction of ear deformities and midface hypoplasia should be offered and performed more frequently. Residual deformity and dissatisfaction remains a problem, especially in reconstructed eyelids. LEVEL OF EVIDENCE: II.