Complete Digital Workflow for Manufacturing Presurgical Orthodontic Palatal Plates in Newborns and Infants with Cleft Lip and/or Palate.

Deciding on the implementation or modification of steps in daily clinical care is a nuanced process that demands careful evaluation. This is crucial not only for selecting the most appropriate solution but also for achieving the best treatment outcome. Thus, implementing a workflow for treating cleft lip and/or palate patients with a presurgical orthodontic cleft-covering plate needs to consider objective factors, prioritized from most to least important: safety and quality level, user-friendliness, feasibility, and, finally, efficiency and cost. The goal of this workflow is to integrate CAD/CAM technologies into daily clinical routine to enhance technical and clinical efficiency, reduce the burden of cleft care, and simplify the implementation of these technologies in other facilities. To achieve this, a methodology based on intraoral scanning and additive manufacturing is employed to produce patient-specific passive palatal plates. The approach describes possible pitfalls and their resolution within the routine of a cleft centre, along with an exemplary case scenario. Comparative analysis between the digital workflow and the conventional process demonstrated the digital approach to be safer, higher in quality, more user-friendly, feasible, and cost- and time-effective than the conventional process.

Prospective cohort study on facial profile changes in infants with Robin sequence and healthy controls.

BACKGROUND: Various conservative and surgical approaches exist to treat Robin sequence (RS), but their effects on facial profile and mandibular catch-up growth are unclear. A functional treatment concept, used in our centre for 25 years, includes an individualized palatal plate with a velo-pharyngeal extension and intensive feeding training. METHODS: We performed a prospective study to objectively describe facial profiles in infants with RS treated with the above concept. Infants with isolated RS were admitted to our tertiary perinatal and national referral centre for craniofacial malformations between May 2018 and Nov 2019. Infants with RS received 3D-photographs during clinically indicated visits. Healthy controls were recruited from Dec 2018 to Sep 2019 and received 3D-photographs every 3 months. The digitally measured jaw index (JI), defined as alveolar overjet (O) x maxillary arch (U)/mandibular arch (L), and the soft tissue reference points A'-point, Nasion', B'-point angle (ANB'), describing the relative position of maxilla to mandible, were evaluated. Linear mixed models were used to examine time trajectories in JI and ANB'. RESULTS: A total of 207 3D images, obtained in 19 infants with RS and 32 controls, were analysed. JI and ANB' decreased over time in both groups [for JI - 0.18 (95% CI - 0.25 to - 0.10); for ANB': - 0.40° per month [(95% CI - 0.48 to - 0.32)]] but remained lower in controls [for JI - 2.5 (95% CI - 3.2 to - 1.8); for ANB'-1.7° (95% CI - 2.4 to - 1.0)]. Also, the ANB' model showed a significant effect of the interaction term diagnosis x age. CONCLUSIONS: Based on longitudinal 3D images, we describe changes in objective parameters of facial profile in infants with and without RS during the first year of life. Our findings indicate catch-up growth in infants treated for RS. Video Abstract.

Intracranial Pressure, Autoregulation, and Cerebral Perfusion in Infants With Nonsyndromic Craniosynostosis at the Time of Surgical Correction.

BACKGROUND AND OBJECTIVE: Although an increased intracranial pressure (ICP) is a known problem in children with syndromic craniosynostosis, it remains unclear whether elevated ICP and impaired cerebral perfusion exist in nonsyndromic synostosis and should be defined as targets of primary treatment. This study aimed to investigate ICP, cerebral autoregulation (CAR), and brain perfusion in infants with nonsyndromic craniosynostosis at first surgical intervention. METHODS: Forty-three infants were prospectively included. The patients underwent perioperative measurement of mean arterial blood pressure, ICP, and brain perfusion before and after cranial vault decompression. Physiological parameters with possible influences on ICP and autoregulation/brain perfusion were standardized for age. CAR was assessed by the pressure reactivity index (PRx), calculated using the mean arterial blood pressure and ICP. RESULTS: Biparietal decompression was performed in 29 infants with sagittal synostosis (mean age, 6.1 ± 1.3 months). Fronto-orbital advancement was performed in 10 and 4 infants with metopic and unilateral coronal synostosis, respectively (mean age, 11.6 ± 2.1 months). An elevated ICP (>15 mm Hg) was found in 20 of 26 sagittal (mean, 21.7 ± 4.4 mm Hg), 2 of 8 metopic (mean, 17.1 ± 0.4 mm Hg), and 2 of 4 unilateral coronal synostosis cases (mean, 18.9 ± 2.5 mm Hg). Initial ICP was higher in sagittal synostosis than in metopic/coronal synostosis (P = .002). The postdecompression ICP was significantly reduced in sagittal synostosis cases (P < .001). The relative cerebral blood flow and blood flow velocity significantly increased after decompression. Impaired CAR was found in infants with a mean ICP >12 mm Hg (PRx, 0.26 ± 0.32), as compared with those with a mean ICP ≤ 12 mm Hg (PRx, -0.37 ± 0.07, P = .001). CONCLUSION: Contrary to common belief, an elevated ICP and significantly impaired CAR can exist early in single suture synostosis, particularly sagittal synostosis. Because an influence of raised ICP on long-term cognitive development is known in other diseases, we suggest that preventing increased ICP during the phase of maximal brain development may be a goal for decompressive surgery, at least for sagittal synostosis cases.

Antibiotic prophylaxis and surgical site infections in orthognathic surgery - a retrospective analysis.

BACKGROUND: This study was conducted to determine surgical site infection (SSI) rates and potential risk factors as well as to evaluate antibiotic prophylaxis in orthognathic surgery. METHODS: This retrospective observational study included patients who received orthognathic surgery. SSIs and their management were assessed for up to one year post-operatively. The applied antibiotic regime and other possible influencing factors (smoking, age, site of infection, drainage, duration of surgery, displacement distances, craniofacial malformations) were assessed. RESULTS: In total 291 patient met the inclusion criteria (56.7% female). The mean age at surgery was 25.5 ± 8.5 years. Fifty-four patients (18.6%) were diagnosed with a craniofacial malformation. Relevant previous surgeries were documented in about one quarter of included patients (n = 75). Ninety-two percent of patients (n = 267) received intraoperative single-dose antibiotic prophylaxis. Surgical site infections occurred in 12.4% (n = 36) of patients. There was a significant association between postoperative infections and type of surgery (P = .037) as well as type of drainage (P = .002). Statistical analyses also revealed a higher prevalence of smokers (P = .036) and previous surgically assisted rapid palatal expansion (SARPE) (P = .018) in the infection group. Furthermore, no significant relationships were observed between postoperative infections and various co-factors (i.e. antibiotic regime, age at surgery, gender, associated craniofacial malformations, surgery duration, displacement distances, mandibular setback vs. advancement). CONCLUSION: Low rates of SSIs occurred following an intraoperative single-dose antibiotic regime. None of the SSIs had a significant effect on the final surgical outcome. Present data do not warrant escalation of the antibiotic regimen. Postoperative smoking and capillary drainage should be avoided.

Autologous Tooth Transplantation in Craniofacial Malformations.

OBJECTIVE: To evaluate the applicability of transplanted teeth in young patients with craniofacial anomalies. DESIGN: Observational study. SETTING: Comprehensive Centre for Cleft Palate and Craniofacial Malformations. PATIENTS/PARTICIPANTS: Patients with craniofacial anomalies who underwent tooth transplantation. Only children with complete clinical and radiological documentation and a follow-up period of at least 1.5 years were included. INTERVENTIONS: Tooth transplantation. MAIN OUTCOME MEASURE(S): Retrospective evaluation of clinical records, pre- and postoperative radiographs, and operative charts. Clinical characteristics of patients, preoperative parameters and postoperative outcome parameters were collected. RESULTS: A total of 17 patients with 23 tooth transplantations were included. The median follow-up period was 6.7 years. The pooled survival and success rates were 91%. Notably, one out of two teeth that were transplanted into the bone grafted alveolar cleft site had to be extracted, which might indicating a higher risk for this procedure. In total, two transplanted teeth had to be extracted during the follow-up period, one due to external resorption and the other one due to perio-endo lesion. One patient needed endodontic treatment due to pulp necrosis. CONCLUSION: We consider tooth transplantation to be a reliable and suitable procedure in the dental rehabilitation of young patients with craniofacial anomalies and fitting concomitant circumstances. We encourage craniofacial teams to reconsider this option more frequently in appropriate cases.

Prospective Evaluation of Children with Robin Sequence following Tübingen Palatal Plate Therapy.

BACKGROUND: To assess the long-term functional orthodontic outcome of the Tübingen palatal plate (TPP) in children with Robin sequence (RS) in comparison to age- and sex-matched healthy controls. METHODS: Between 09/2019 and 10/2020, we performed orthodontic assessments in 41 children at our Department of Orthodontics. Included were patients with RS (17 non-syndromic; four syndromic) and healthy controls (n = 22, average age in both groups 9.9 y). Facial analyses of 2D images, digital study casts and cephalometric measurements were made. RESULTS: The orthodontic examinations showed no statistically significant group differences regarding functional extraoral, intraoral and pharyngeal parameters, or in skeletal patterns. The relationship between the upper and lower incisors was significantly increased (overjet 4 (2-10) vs. 3 (0-9) mm; p = 0.01) with a significant deficit in the lower face proportions (Jaw Index 4.15 (1.9-9.6) vs. 2.98 (0-9); p = 0.02; Facial convexity angle 157 (149-173) vs. 159 (149-170); p = 0.01). CONCLUSION: Children with RS treated with the TPP showed normal long-term functional orthodontic outcomes, thanks to the functional adaption of the stomatognathic system. However, soft tissue growth did not completely match skeletal growth, resulting in a more convex facial profile.

Sleep and neurocognitive outcome in primary school children with Robin Sequence.

STUDY OBJECTIVES: To investigate neurocognitive and behavioral outcomes at primary school age in relation to obstructive sleep apnea (OSA) in children with Robin sequence (RS) treated with the Tuebingen palatal plate in infancy and to assess the impact of OSA in these patients. METHODS: Forty-two primary school-aged children (n = 21 with RS, n = 21 age- and sex-matched controls) underwent polysomnography, intelligence testing ("Wechsler Intelligence Scale for Children-Fifth Edition" [WISC-V]), and anthropometrics. Families completed a 7-day sleep diary and questionnaires on sleep and behavior (Children's Sleep Habits Questionnaire [CSHQ] and the Child Behavior Checklist [CBCL]). RESULTS: In children with RS (17 non-syndromic, four syndromic; median age 9.7 [8.5-10.8] years), the obstructive apnea-hypopnea index (OAHI) was significantly higher than in controls (1.3 [0.4-2.7]/h vs. 0.4 [0.1-0.6]/h). Two syndromic children with RS were already on nocturnal respiratory support for OSA prior to our study, and one non-syndromic child was diagnosed with severe OSA (OAHI 57/h) despite an unremarkable medical history and questionnaire. The overall intelligence quotient in children with RS was within the normal range and did not differ between children with RS and healthy peers (102 vs. 108, p = .05). However, children with RS had values in the at-risk clinical range for externalizing behavior. CONCLUSIONS: These children with RS showed an increased risk of OSA and behavioral problems, suggesting regular screening for OSA throughout childhood. Neurocognitive scores in children with RS were within the normal range after adequate treatment of OSA during infancy.

Neurocognitive development in isolated Robin sequence treated with the Tuebingen palatal plate.

OBJECTIVES: We aimed to determine the neurocognitive development of cleft palate patients with and without Robin sequence (RS). MATERIALS AND METHODS: Children with isolated RS with cleft palate and children with cleft palate only (CPO) were contacted at the age of 5-6 years. All RS children had undergone initial polygraphic sleep study (PG) with a mixed-obstructive apnea index (MOAI) of ≥ 3/h and were consequently treated with the Tuebingen palatal plate. A standardized clinical examination as well as a neuropediatric and neuropsychological examination included the Wechsler Pre-school and Primary Scale of Intelligence (WPPSI-III), Kaufman Assessment Battery for Children (K-ABC), and an assessment of developmental milestones. RESULTS: In total, 44 children (22RS, 22CPO) were included. RS children were younger at study (70.5 ± 7.3 and 75.2 ± 7.5 months; P = .035). Both groups achieved the evaluated milestones within the normed time frame. WPPSI-III and K-ABC results showed no group differences. Mean values for Verbal IQ (101.8 ± 11.1 vs. 97.1 ± 15.7), Performance IQ (102.9 ± 12.1 vs. 99.6 ± 14.5), Processing Speed Quotient (98.9 ± 15.6 vs. 94.5 ± 15.7), Full-Scale IQ (103.2 ± 12.1 vs. 98.4 ± 15.3), and Sequential Processing Scale (102.1 ± 13.1 vs. 94.2 ± 17.3) were within the reference range (IQ 85-115) for RS and CPO children, respectively, indicating average performance of both groups. CONCLUSION: No neurocognitive, physical, or mental impairments were detected suggesting that RS children having upper airway obstruction (UAO) treated early and effectively may use their potential for an age-appropriate neurocognitive development. CLINICAL RELEVANCE: Tuebingen palatal plate treatment successfully releases UAO. Thus, isolated RS does not necessarily result in developmental delay or an impaired neurocognitive outcome. TRIAL REGISTRATION: Deutsches Register Klinischer Studien, DRKS00006831, https://www.drks.de/drks_web/.

Speech Development in Cleft Palate with and without Robin Sequence.

BACKGROUND: Robin sequence is defined as the triad of micrognathia, glossoptosis, and upper airway obstruction. In up to 85 percent, it is associated with cleft palate. Many studies have reported worse speech development in Robin sequence children after cleft palate repair. The authors investigated speech development in isolated Robin sequence with cleft palate versus children with cleft palate only at the age of 5 to 6 years. METHODS: All Robin sequence children were treated with the Tübingen palatal plate after birth. Data were collected using the German version of the Great Ormond Street Speech Assessment. Audio and video recordings were reviewed and analyzed separately by two blinded senior phoniatricians based on the German version of the Universal Reporting Parameters for Cleft Palate Speech, and scored to enable comparability of speech outcomes. RESULTS: Forty-four children (Robin sequence, n = 22; cleft palate only, n = 22) were included. Robin sequence children were significantly older at surgery (11.8 months versus 7.1 months; p < 0.001) but younger at study (70.5 months versus 75.2 months; p = 0.035). They also had more severe cleft of the palate (p = 0.006). All children studied showed good to very good speech development without serious impairment. None of the reported parameters on the German version of the Universal Reporting Parameters for Cleft Palate Speech showed significant group differences; the median total score in the Robin sequence group was 23 (interquartile range, 16.5 to 27.5) versus 19 (interquartile range, 17 to 23) in the cleft palate-only group. Statistical analysis revealed no significant effect of group (Z = -1.47; p = 0.14). CONCLUSIONS: No group differences in speech development were found at age 5 to 6 years. Isolated Robin sequence does not necessarily represent a risk for impaired speech development. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, II.

Three-Dimensional Quantification of Facial Asymmetry in Children with Positional Cranial Deformity.

BACKGROUND: The extent of facial involvement in positional plagiocephaly is only little investigated so far. Investigation methods that take into account the challenging anatomical conditions and growth of infants' faces are desirable. In this study, the authors established a new three-dimensional photogrammetry quantification method evaluating pretherapeutic and posttherapeutic facial asymmetry in positional plagiocephaly. Furthermore, a facial asymmetry index was established and evaluated. METHODS: Three-dimensional photographs of 100 children undergoing treatment with head orthoses were analyzed by constructing a standardized interindividual coordinate system. Defining landmarks, section planes, and point coordinates with a computer-aided design software, both sides of the faces were compared. Facial asymmetry was quantified by measuring differences between left and right sides and pretherapeutic and posttherapeutic changes in each patient. The facial asymmetry index was calculated by putting the absolute differences in relation with the coordinates of the nonaffected side. RESULTS: Present results indicate that positional plagiocephaly results in a distinct facial asymmetry (range, -3.8 to 9.6 mm) in nearly all spatial directions and facial regions. Helmet therapy led to a significant reduction (p < 0.05) of intraindividual facial asymmetry (median change in facial asymmetry index, -1.9 to 3.1 percent). However, no correlation of the Cranial Vault Asymmetry Index and facial asymmetry (Spearman rank correlation coefficient, ρ = -0.09 to 0.47) has been seen. According to these results, severe occipital deformation does not necessarily provoke distinct facial asymmetry. CONCLUSIONS: Present three-dimensional photogrammetry method allows the longitudinal quantification of facial involvement in positional plagiocephaly. Asymmetry has been determined in all facial regions. The facial asymmetry decreased through helmet therapy but was not eliminated completely.

Surgical Management of Sporadic Peripheral Nerve Schwannomas in Adults: Indications and Outcome in a Single Center Cohort.

Most sporadic peripheral nerve sheath tumors in adults are schwannomas. These tumors usually present with significant pain but can also cause neurological deficits. Symptomatology is diverse, and successful surgical interventions demand interdisciplinarity. We retrospectively reviewed 414 patients treated between 2006 and 2017 for peripheral nerve sheath tumors. We analyzed clinical signs, symptoms, histology, and neurological function in the cohort of adult patients with schwannomas without a neurocutaneous syndrome. In 144 patients, 147 surgical interventions were performed. Mean follow-up was 3.1 years. The indication for surgery was pain (66.0%), neurological deficits (23.8%), significant tumor growth (8.8%), and suspected malignancy (1.4%). Complete tumor resection was achieved on 136/147 occasions (92.5%). The most common location of the tumors was intraspinal (49.0%), within the cervical neurovascular bundles (19.7%), and lower extremities (10.9%). Pain and neurological deficits improved significantly (p ≤ 0.003) after 131/147 interventions (89.1%). One patient had a persistent decrease in motor function after surgery. Complete resection was possible in 67% of recurrent tumors, compared to 94% of primary tumors. There was a significantly lower chance of complete resection for schwannomas of the cervical neurovascular bundle as compared to other locations. The surgical outcome of sporadic schwannoma surgery within the peripheral nervous system is very favorable in experienced peripheral nerve surgery centers. Surgery is safe and effective and needs a multidisciplinary setting. Early surgical resection in adult patients with peripheral nerve sheath tumors with significant growth, pain, neurological deficit, or suspected malignancy is thus recommended.

Perioperative complications in cleft palate repair with Robin sequence following Tuebingen palatal plate treatment.

Our study aimed to evaluate perioperative complications following our institutional pre- and intraoperative management in cleft palate repair with Robin sequence (RS). RS patients who underwent cleft palate repair between 2000 and 2020 were retrospectively analysed. RS children with complete documentation and whose initial treatment involved the Tuebingen palatal plate (TPP) were included. Clinical records and operative charts were reviewed with regard to clinical characteristics as well as the neonatal and perioperative course. Results before and after adjustment of the anesthesiology protocol in 2014 were compared. 143 RS patients (41% male, 59% female) were included. Median pretherapeutic mixed-obstructive apnea index (MOAI) after birth was 9.4/hour (IQR 20.0). TPP treatment was associated with normalisation of the MOAI and adequate weight gain until surgery. At surgery, median age was 10 months (IQR 3), MOAI 0.1/h (IQR 0.5), and weight 8.7 kg (IQR 1.7). In 93% of cases (n = 133), the postoperative course was uneventful. Refinement of the anesthesiology protocol showed positive effects on the perioperative course and led to a reduction in perioperative events (10.7% vs. 2.9%). No severe perioperative complications occurred. We recommend the adoption of TPP treatment in the therapy of RS children. Our favourable results show that early TPP treatment minimizes perioperative complications in cleft palate repair by effectively and sustainably correcting upper airway obstruction.

Surgical management of peripheral nerve sheath tumours in children, with special consideration of neurofibromatoses.

INTRODUCTION: Peripheral nerve sheath tumours in children are a rare and heterogeneous group, consisting mostly of benign tumours as well as malignant neoplasms. Especially in the paediatric population, diagnostics and indication for therapy pose relevant challenges for neurosurgeons and paediatric neurologists alike. Most paediatric cases that need surgical intervention are associated to neurofibromatosis type 1 (NF1). METHODS: We retrospectively reviewed all paediatric cases treated at the Department of Neurosurgery in Tübingen between 2006 and 2017 for peripheral nerve sheath tumours. We analysed clinical signs, symptoms, histology, association to an underlying phacomatosis and sensory/motor function. RESULTS: Of the 82 identified patients, the majority had NF1 (76.8%). Nine children bore a sporadic tumour without underlying phacomatosis (11%), 8 had NF2 (9.8%) and 2 schwannomatosis (2.4%), A total of 168 surgical interventions were performed, and 206 tumours were removed. Indication for surgery was in most instances significant tumour growth (45.2%) followed by pain (33.9%). New deficits led to surgery in 12.5% of interventions; malignancy was suspected in 8.3%. Histopathology revealed mostly neurofibromas (82.5%), divided into cutaneous neurofibromas (10.7%), infiltrating plexiform neurofibromas (25.7%) and peripheral nerve-born neurofibromas (46.1%). 12.1% of tumours were schwannomas, 2.9% MPNST, 1.5% ganglioneuroma (n = 3) and 1 hybrid-neurofibroma and perineurinoma each. Leading symptoms, such as pain and motor and sensory deficits, improved after 125/166 interventions (74.4%), remained unchanged following 39 interventions (23.2%) and worsened in 4 occasions (2.4%). CONCLUSION: Surgery is safe and effective for (neurofibromatosis associated) peripheral nerve sheath tumours in the paediatric population; however, management needs a multidisciplinary setting. We propose early surgical resection in paediatric patients with peripheral nerve sheath tumours with significant growth, or pain, or motor deficit, or suspected malignancy.

Isolated gluteal metastasis 3 years after sufficiently treated oral squamous cell carcinoma: Case report and literature review.

Metastases from oral squamous cell carcinoma usually occur in the cervical lymph nodes. Distant skeletal muscle metastases are exceedingly rare and have been reported only in sporadic cases. A 70-year-old male patient was diagnosed with nonmetastatic oral squamous cell carcinoma (pT4a, N0, M0). The patient underwent successful surgical treatment involving tumor resection and selective neck dissection of both sides, including adjuvant radiotherapy. After 3 years of uneventful course, with no clinical or radiographic evidence for local recurrence or metastasis, an isolated distant metastasis in the gluteal muscles was diagnosed. The patient died 7 months after diagnosis despite further surgical resection and adjuvant chemotherapy. Distant metastases are mostly accompanied by systemic spread of tumor cells. Thus, the prognosis is generally very poor. The therapeutic concept of surgical tumor resection and adjuvant chemotherapy failed to significantly improve the patient's prognosis in the present case.

Fungus Ball of the Maxillary Sinus-Modern Treatment by Osteoplastic Approach and Functional Endoscopic Sinus Surgery.

PURPOSE: Functional endoscopic sinus surgery (FESS) is considered standard surgical therapy for fungus ball of the maxillary sinus. However, recent findings have indicated an odontogenic etiology, which requires simultaneous treatment of the dental origin. This study presents the authors' treatment results of fungus ball of the maxillary sinus using a combination of FESS and an endoscopically assisted osteoplastic approach through the anterior wall of the maxillary sinus, enabling simultaneous treatment of the dental origin. MATERIALS AND METHODS: A cohort of 22 patients with histopathologically confirmed fungus ball of the maxillary sinus was retrospectively analyzed. Clinical records and medical imaging data were reviewed to evaluate the etiology, clinical and radiologic findings, and postoperative outcome. RESULTS: Only 15 patients presented nonspecific clinical symptoms compatible with chronic unilateral maxillary sinusitis. Computed tomography visualized complete opacity of the maxillary sinus in 11 patients and intralesional hyperdensity in 12 patients. An odontogenic association was verified in 18 patients. Twenty-one patients underwent endoscopically assisted osteoplastic surgery through the anterior maxillary sinus wall. In 12 cases, the assumed persistent odontogenic source was treated simultaneously. Depending on the patency of the ostiomeatal complex, the accompanying chronic sinusitis was treated by FESS. CONCLUSIONS: The present data support the assumption of an odontogenic etiology of fungus ball of the maxillary sinus. Hence, surgical management requires simultaneous treatment of the fungal mass, the odontogenic origin of the disease, and the accompanying chronic sinusitis. To properly treat fungus ball, the authors present a modern treatment concept, using a minimally invasive endoscopically assisted osteoplastic approach through the anterior maxillary wall, for sufficient and necessary surgical treatment.

Health-related quality of life in cleft lip and/or palate patients - A cross-sectional study from preschool age until adolescence.

PURPOSE: Cleft lip and/or palate (CLP) is the most common congenital craniofacial anomaly. Multiple operations, long-lasting supplementary treatments, as well as impaired functional and esthetic outcome might have a negative impact on patients' social-emotional functioning and self-esteem, resulting in a lower health-related quality of life (HRQoL). PATIENTS AND METHODS: This cross-sectional study aimed to evaluated CLP patients' HRQoL from preschool age (4 years) until adolescence (18 years) using the age-specific German KINDLR questionnaire. We compared self-reports and parent proxy-reports, as well as reference values from an age-matched German norm population. Multivariate analysis was applied to identify mediating factors, e.g. cleft type, age, and gender. Additionally, a KINDSCREEN-10 questionnaire was used to implement a screening tool in the clinical routine. RESULTS: In total, 134 participants (average age 9.0 ± 3.8 years; 47.8% female) were included. Compared with German normative data, the evaluation revealed a significantly higher 'total QoL' in all self-report groups (Kiddy-, Kid-, Kiddo-KINDLR) and a significantly higher proxy rating for children aged 7-13 years. Multivariate analysis verified a significant disparity between self-reports and parents' conceptions of HRQoL, as well as a deterioration of the ratings with increasing age. No significant effects of other contributing factors, e.g. cleft type and gender, were found. The KIDSCREEN-10 questionnaire successfully confirmed these findings. CONCLUSIONS: Surprisingly, our survey revealed a higher HRQoL in cleft patients compared with normative data from healthy controls. But because the return rate of the forwarded questionnaire was low, this may have contributed to bias. Keeping this in mind, we may conclude at least that the HRQoL in our cleft patients was not significantly lower than in healthy children. On the other hand, it can be assumed that the special attention of the parents and the support from speech therapy and other medical professionals may have contributed to a positive effect on family interaction, communication skills, and self-esteem.

Three-dimensional accuracy of mandibular reconstruction by patient-specific pre-bent reconstruction plates using an "in-house" 3D-printer.

PURPOSE: The purpose of this study was to compare the three-dimensional accuracy of mandibular reconstruction following mandible continuity resection in patients treated with patient-specific, pre-bent reconstruction plates, using an 'in-house' 3D printer, with that in patients treated with conventional, intraoperatively bent plates. PATIENTS AND METHODS: We retrospectively analyzed 42 alloplastic mandibular reconstructions following continuity resection. 21 patients received patient-specific, pre-bent reconstruction plates using an in-house 3D printer. The control group consisted of 21 patients provided with intraoperatively bent reconstruction plates. Distances between corresponding anatomical landmarks on the mandibular angle and condyle (A-A', BB', CC', BC'), as well as the intercondylar angle (ICA), were measured and compared on the pre- and postoperative CT scans. RESULTS: Multivariate analysis of variance (MANOVA) showed significant multivariate main effect of group (F(5, 36) = 5.58, p = 0.001; Wilks lambda 0.564; partial η2 = 0.436), indicating more accurate postoperative results for the pre-bent group. Post-hoc comparison revealed significantly larger postoperative deviation in the distances between the mandibular angle and the intercondylar angle (ICA) for the intraoperatively bent group in comparison with the pre-bent group. However, there was no significant effect on the distance between the condyles. CONCLUSIONS: Mandibular reconstructions employing patient-specific, pre-bent reconstruction plates made using an in-house 3D printer show significantly better three-dimensional accuracy compared with intraoperatively bent plates. The described method prevents rotational error of the mandibular angle and improves restoration of the physiological intercondylar angle in mandible reconstruction after continuity resection.

Less invasive treatment of sleep-disordered breathing in children with syndromic craniosynostosis.

BACKGROUND: Infants and children with syndromic craniosynostosis (SCS), such as Apert-, Crouzon- or Pfeiffer syndrome, are prone to sleep disordered breathing (SDB) including obstructive sleep apnea and upper airway resistance syndrome (OSAS, UARS), potentially leading to tracheostomy. We modified the Tübingen Palatal Plate (TPP), an oral appliance with a velar extension effectively treating airway obstruction in Robin sequence, by attaching a tube to its velar extension to bridge the narrow pharyngeal airway in SCS patients. Here, we evaluated this treatment concept. METHODS: Our hospital's electronic patient files were searched for all children with a diagnosis of SCS admitted between 01/01/2004 and 31/12/2016. Children with isolated craniosynostosis were excluded. OSAS was defined as a mixed-obstructive apnea-hypopnea index (MOAHI) > 1, and UARS as more than 1 episode with nasal flow limitation/h, but absent OSAS. Children with a diagnosis of OSAS received the TPP and fiberoptic nasopharyngoscopy to assess the type of obstruction and to adjust the plate. Growth and weight gain, determined as standard deviation scores, were also evaluated before and during treatment. RESULTS: Of 34 patients included, 24 presented with SDB (19 OSAS, 5 UARS) and 27 had midface hypoplasia. Proportions of SDB were 78% in those with, and 22% in those without midface hypoplasia. In the OSAS group (n = 19), 13 patients were treated with palatal plates, with the remaining receiving continuous positive airway pressure, midface surgery or tracheal intubation. The MOAHI decreased across all children receiving palatal plate treatment from 14.6 (range 0.0-50.7) at admission to 0.9 (range 0.0-3.5) at discharge (p = 0.002). SDS for weight and body length also improved (p < 0.05 for weight and p = 0.05 for body length). Only one child required tracheostomy. CONCLUSION: Treatment of upper airway obstruction by a modified TPP in these children with SCS was shown to be mostly effective and safe. If confirmed in larger prospective studies, it may help to avoid more invasive interventions.

Donor site morbidity of the anterolateral thigh flap.

INTRODUCTION: The purpose of this study was to investigate donor site morbidity after reconstruction of defects in the head and neck region with an anterolateral thigh (ALT) flap. METHODS: From 07/2006 to 12/2015 the anterolateral thigh flap was used for reconstruction of large intra- or extraoral defects in 138 patients. The donor sites were available to examine for morbidity in 51 patients (36.7%). All patients completed a standardized questionnaire, received a physical examination, and underwent isokinetic muscle exercises of the lower extremity with a Dynamometer. RESULTS: The average postoperative follow-up was 29 months and showed significant donor site morbidity in the form of sensory disturbance as well as revealing significant differences in extension and flexion between donor and normal thighs in the isokinetic power test. Range of motion at the hip and knee was moderately compromised. CONCLUSION: The results of this study suggest that dissection of the musculocutaneous perforators can compromise the function of vastus lateralis, which is the largest compartment of the quadriceps group. This could also affect the antagonistic muscles such as the semitendinosus, semimembranosus and biceps femoris. Because of its versatility, adequate tissue stock, possibility of simultaneous two-team approach large and long pedicle and minimal donor site morbidity, the ALT flap has become one of the most preferred transplants for soft tissue reconstruction in the head and neck region. Most authors described the donor site morbidity as minimal (Song et al. 1984; Wolff et al., 2006; Xu et al., 1988; Kimata et al., 1997; Kuo et al., 2001; Collins et al., 2012). In summary, the study shows that functional impairment of the donor thigh can occur but does not affect the patients in their daily life.