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Purpose: The aim of this study was to clarify the three-dimensional changes in maxillofacial morphology following orthognathic surgery using 3D-CT in patients with asymmetrical jaw deformities., Methods: The subjects were 30 patients with asymmetrical jaw deformities. Three-dimensional images taken preoperatively and 6 months postoperatively were re-aligned using image processing software with the horizontal plane, coronal plane, and midsagittal plane as reference axes. Thirty-nine measurements including 28 distances, nine angles, and two volumes were recorded, and differences between preoperative and postoperative measurements and percentage differences between paired measurements on the deviated and unevolved sides were calculated., Results: Translational and rotational movements of the maxillary dentition and mandibular body and the mandibular ramus internally rotating contributed to improved symmetry of the maxillofacial morphology, but the degree and proportion of these changes varied from case to case and mild asymmetry remained even after surgery., Conclusions: 3D-CT analysis of maxillofacial morphology is essential to accurately evaluate the asymmetry of hard and soft tissue morphology in the maxillofacial region and the degree of improvement after orthognathic surgery, and the tooth movement during preoperative orthodontic treatment should be determined taking into account the movement of the upper and lower jaws during orthognathic surgery., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: To compare the outcome of silicone sling frontalis suspension (FS) surgery in children with simple congenital ptosis and children with complex ptosis., Design: A retrospective cohort study., Participants: All pediatric patients who underwent silicone sling FS surgery between 2009 and 2020 at a single centre., Methods: Patients were divided based on etiology into simple congenital ptosis and complex congenital ptosis. Pre- and postoperative margin-to-reflex distance (MRD 1 ) measurements were determined from clinical photographs. Main outcome measures were assessed as differences in improvement in eyelid height and reoperation rate between the groups., Results: Two-hundred and eight children were included: 139 simple and 69 complex cases, with 83 females (40%). Mean (±SD) age at intervention was 1.9 ± 2.9 years. Complex cases included blepharophimosis epicanthus inversus syndrome (n = 35), Marcus Gunn jaw-winking syndrome (n = 12), oculomotor palsy (n = 8), congenital fibrosis of extraocular muscles (n = 3), chronic progressive external ophthalmoplegia (n = 3), and others. Mean MRD 1 improved by an average of 1.6 mm in both groups. Repeat ptosis correction was performed in 50 of 171 patients (29%) without a history of failed ptosis procedures, and this rate was similar between simple and complex cases. Children under 3 years of age had higher rates of repeat ptosis repair than older children (n = 59 of 175 [34%] vs n = 5 of 33 [15%]; p = 0.03, χ 2 test)., Conclusions: Silicone sling FS has a favourable outcome in 70% of pediatric patients. Preoperative and final MRD 1 and reoperation rates were similar between both groups, suggesting that despite the higher complexity in atypical cases, the outcome is similar., (Copyright © 2023 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved.)

Background: Congenital maxillomandibular fusion is a rare disorder. Maxillomandibular fusion is usually discovered immediately after birth because the newborn is unable to open mouth or feed normally. The fusion defects can present with a wide range of severity, ranging from mucosal band (synechiae) to complete bony fusion (syngnathia)., Case Description: We report a case of congenital alveolar synechiae with posterior cleft palate in a 3-day-old male neonate. The newborn was managed with the help of a multi-disciplinary approach., Conclusion: Fusion of the gums is a very rare congenital anomaly. Early division of fibrous bands not only allows effective feeding but also prevents the development of facial deformities. However, anesthesia for this procedure can be challenging.

Rationale: This case report presents a rare combination of congenital anomalies in an otherwise healthy male infant born at 36 weeks. The infant was diagnosed with congenital maxillomandibular synechia, ectrodactyly, and ankyloglossia superior syndrome (ASS)., Patient Concerns: Inability to open the mouth completely, feeding challenges, and a cleft palate. The infant was stabilized through successful positive pressure ventilation via a face mask at birth and enteral feeding was initiated via a feeding gastrostomy., Examination: Diagnostic tests revealed a midline palatal cleft, hypoplastic jaws, persistent metopic suture, and a bony fusion at the midline., Treatment: Sectioning of the bony spur along the midline and achieving a mouth opening of 2 cm post-manipulation. The patient is under follow-up, with future treatment plans including cleft palate correction at 12 months and potential frontomandibular and lower jaw advancement depending on growth trajectories., Takeaway Lessons: This case underscores the complexity of managing multiple congenital anomalies and the need for individualized treatment plans., (Copyright © 2024 Copyright: © 2024 Indian Journal of Dental Research.)

Congenital syngnathia is a rarely reported malformation when there is a fusion between the maxilla and the mandible. It is necessary to modify it in childhood because congenital syngnathia causes incongruity in pronunciation, diet, and esthetics during the growth process. In this case report, 1 case of syngnathia, a rare craniofacial anomaly, is presented with a review of reports. Prompt diagnosis and surgery were performed right after birth for the present case. A partial limitation point was resolved for further growth. Herein, the authors present the case of a female infant (7 d after birth) diagnosed with congenital syngnathia and treated by early surgical intervention., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 by Mutaz B. Habal, MD.)

Agnathia-otocephaly complex (AOC), a first branchial arch defect, is characterized by mandibular hypoplasia or aplasia, ear abnormalities, microstomia, and macroglossia and is a rare and often fatal diagnosis. Herein, the technical considerations and details of mandibular reconstruction using virtual surgical planning (VSP) and a vascularized free fibula flap for further mandibular reconstruction in a 10-year-old boy are presented. The patient's preoperative examination was consistent with agnathia (absence of mandibular symphysis, bilateral mandibular bodies, condyles, coronoids, rami, and temporomandibular joint), severe microstomia, and a Tessier # 30 cleft (maintained to allow oral access until later in treatment). Virtual surgical planning was utilized to plan a 3-segment fibula for the reconstruction of the mandibular symphysis and bilateral body segments, and bilateral costochondral grafts were planned for the rami. To the authors' knowledge, this represents the first application of virtual surgical planning for mandibular reconstruction with a vascularized free fibula flap in a pediatric patient with severe agnathia-otocephaly complex., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 by Mutaz B. Habal, MD.)

Background: Spring-mediated cranioplasty (SMC) is an increasingly utilized technique to treat patients with nonsyndromic sagittal craniosynostosis, but variables impacting outcomes are incompletely understood. The purpose of this study was to determine variables most predictive of outcomes following SMC, primarily changes in cephalic index (CI)., Methods: Patients with nonsyndromic sagittal craniosynostosis undergoing SMC at our institution between 2014 and 2021 were included. Cephalic index was measured from patient computed tomography scans, x-rays, or by caliper-based methods. Parietal bone thickness was determined from patient preoperative computed tomography. Stepwise multiple regression analysis, least absolute shrinkage and selection operator, and random forest machine learning methods were used to determine variables most predictive of changes in CI., Results: One hundred twenty-four patients were included. Stepwise multiple regression analysis identified duration of spring placement ( P =0.007), anterior spring force ( P =0.034), and anterior spring length ( P =0.043) as statistically significant predictors for changes in CI. Least absolute shrinkage and selection operator analysis identified maximum spring force (β=0.035), anterior spring length (β=0.005), posterior spring length (β=0.004), and duration of spring placement (β=0.0008) as the most predictive variables for changes in CI. Random forest machine learning identified variables with greatest increase in mean squared error as maximum spring force (0.0101), anterior spring length (0.0090), and posterior spring length (0.0056)., Conclusions: Maximum and total spring forces, anterior and posterior spring lengths, and duration of spring placement were the most predictive variables for changes in CI following SMC. Age at surgery and other demographic variables were inferior predictors in these models., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 by Mutaz B. Habal, MD.)

Abstract: Late presentation sagittal craniosynostosis presents a unique challenge due to the decreased ability of the skull to repair the bony defects created by standard of care techniques. Distraction osteogenesis is a viable strategy to correct this defect in late presenting cases. The authors describe a variation in technique in which the temporalis muscle origin is retained, creating an osteoplastic bone flap with retained vascularity through the tem-poralis muscle. This may improve postoperative bony healing of bony defects in this compromised population. The authors present two patients who presented to them late with sagittal synostosis who were treated with distraction osteogenesis in which vascular continuity to the parietal bones is preserved through the temporalis muscle., Competing Interests: The authors report no conflicts of interest., (Copyright © 2021 by Mutaz B. Habal, MD.)

The aim of this study was to carry out a retrospective multicentre study comparing the morphological outcome of 8 techniques used for the management of sagittal synostosis versus a large cohort of control patients. Computed tomographic (CT) images were obtained from children CT-scanned for non-craniosynostosis related events (n = 241) and SS patients at preoperative and postoperative follow-up stages (n = 101). No significant difference in morphological outcomes was observed between the techniques considered in this study. However, the majority of techniques showed a tendency for relapse. Further, the more invasive procedures at older ages seem to lead to larger intracranial volume compared to less invasive techniques at younger ages. This study can be a first step towards future multicentre studies, comparing surgical results and offering a possibility for objective benchmarking of outcomes between methods and centres., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2021 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.)

Introduction: Congenital meningoencephalocele is a herniation of brain and meninges through a skull base defect. It may result not only in neural defects, sensorimotor deficits, neurological morbidities, visual impairment, impaired nasal function, and a potential risk of intracranial infection. Goals of surgery include removal or repositioning of nonfunctional cerebral tissue, closure of the dura, and reconstruction of skeletal and cutaneous structures., Materials and Methods: The authors present the case of a 4-months-old infant who was found to have a frontoethmoidal encephalomeningocele that was only discovered after birth, the volume increased gradually. After multiple department discussions, the procedures were planned in 2-staged surgical protocol comprising of the first stage urgently performed by neurosurgeon and craniomaxillofacial surgeon, which aimed at removal or repositioning of nonfunctional cerebral tissue, closure of the dura, and reconstruction of skeletal; then second stage was performed by plastic surgeon to correct craniofacial hard and soft tissue deformities., Results and Conclusions: The surgical procedures for frontoethmoidal encephalomeningocele are complicated, particularly for the infant. In order to achieve the final surgical purpose, it needs multiple department cooperation to make the surgical plans., Competing Interests: The authors report no conflict of interest., (Copyright © 2021 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of Mutaz B. Habal, MD.)

Scaffolds stimulate cell proliferation and differentiation and play major roles in providing growth and nutrition factors in the repair of bone defects. We used the recombinant peptide Cellnest™ to prepare the three-dimensional stem cell complex, CellSaic, and evaluated whether CellSaic containing rat dental pulp stem cells (rDPSCs) was better than that containing rat bone marrow stem cells (rBMSCs). rDPSC-CellSaic or rBMSC-CellSaic, cultured with or without osteogenic induction medium, formed the experimental and control groups, respectively. Osteoblast differentiation was evaluated in vitro and transplanted into a rat model with a congenital jaw fracture. Specimens were collected and evaluated by microradiology and histological analysis. In the experimental group, the amount of calcium deposits, expression levels of bone-related genes (RUNX2, ALP, BSP, and COL1), and volume of mineralized tissue, were significantly higher than those in the control group ( p < 0.05). Both differentiated and undifferentiated rDPSC-CellSaic and only the differentiated rBMSC-CellSaic could induce the formation of new bone tissue. Overall, rBMSC-CellSaic and rDPSC-CellSaic made with Cellnest™ as a scaffold, provide excellent support for promoting bone regeneration in rat mandibular congenital defects. Additionally, rDPSC-CellSaic seems a better source for craniofacial bone defect repair than rBMSC-CellSaic, suggesting the possibility of using DPSCs in bone tissue regenerative therapy.

Spring-mediated cranial vault expansion (SMC) has become a primary treatment modality at our institution to correct scaphocephalic head shape in the setting of isolated sagittal craniosynostosis (CS). Spring-mediated cranioplasty is associated with minimal procedural morbidity and reliable clinical efficacy, although long-term outcomes are not well elucidated. Herein we describe our institutional experience and lessons learned with SMC. We hypothesize that SMC performed in young infants offers durable scaphocephalic correction as measured by cephalic index (CI) at the 1, 3, and 5-year postoperative timepoints.Patients with isolated sagittal CS who underwent SMC at our institution during an 8-year period were retrospectively studied. The primary outcome measure was long-term head shape determined by CI at the 1, 3, and 5-year postoperative timepoints. Secondary outcomes included patient and spring factors associated with change in CI, including age and spring force. All statistical tests were 2-tailed with P < 0.05 denoting significance.In total, 88 patients underwent SMC at a median age of 3.3 months with a median preoperative CI 69 (interquartile range: [66, 71]). The postoperative CI increased to 73 [71, 76] at postoperative day 1. At 1 month, the CI increased by 8.6 to 77 (P < 0.0001) and appeared to reach a plateau at 3 months (76, [74, 78]) without further improvement (P < 0.10). At 5 years, CI remained stable without relapse (76, [75, 81], demonstrating an 8.9 increase from preoperative CI. Age at time of spring placement and change in CI were inversely related (P < 0.005). Total spring force directly correlated with increased change in postoperative CI at the 6-month postoperative timepoint (P < 0.02).In summary, SMC offers durable correction of scaphocephaly as measured by CI for patients with isolated sagittal CS at the 5-year postoperative timepoint. The cranial expansion observed 1-month post-spring implantation may serve as a proxy for long-term CI.

Introduction: Over the last 10-15 years, usage of internal mandibular distraction systems has increased in the pediatric population, particularly for craniofacial syndromes. Mandibular distraction osteogenesis (MDO) has been shown to be effective in avoiding tracheostomy or achieving early decannulation in patients with micro-retrognathic mandibles in hemifacial microsomia or Pierre Robin sequence. As the frequency of the application of MDO has increased, so has the awareness and management of subsequent complications from the procedure. In this study, we discuss a complication involving paresis and eventual recovery of cranial nerve (CN) VII after the application of an MDO internal device at our institution in two cases. We also review the literature and propose multiple anatomic considerations that can impact more than just the marginal branch of CN VII., Method: This study is a retrospective case study from our institution and a review of the literature. Pubmed was queried for terms singularly and in combination including "mandibular distraction osteogenesis", "facial nerve", "cranial nerve", "complications", "micrognathia", "retrognathia". After reviewing the results, studies discussing complications of MDO that involved CN VII were reviewed and included., Results: In the literature review and our retrospective review, CN VII injuries from MDO vary in their length and timing of onset. Management of this complication depended on the timing of onset and ranged from conservative management to removal of the distraction device. Majority (7/9) of the cases resolved to an eventual House-Brackmann of 0/6 with conservative measures., Conclusion: Total facial nerve injury in association with MDO, has been scarcely reported, though the facial nerve is at great risk given its intimate location near the mandible particularly in neonates. It is encouraging that though it could be a devastating complication, out of all the cases reported, the large majority resolve with a combination of time and steroids., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Congenital syngnathia is a very rare condition that presents with several unique challenges. There is no consensus on the best operative approach to manage this condition. Readhesion of surgically separated tissues is a relatively common occurrence. The authors propose that the use of a vertical distraction technique helps maintain adequate separation to prevent relapse of the bony fusion. Long-term follow-up will be required to follow mandibular growth and implications of this treatment option.

: A question that remains unanswered is at what level of surgical correction does the public perceive a head shape to be "normal" or "acceptable?" For most cases of non-syndromic asymptomatic craniosynostosis, the parents desire for surgical correction is to improve the cosmetic appearance of head shape. At the time of this writing, the intraoperative surgeons' perspective of what constitutes an acceptable head shape is the target for surgical correction. In introducing an improved objectively cosmetic goal, an appropriate outcome measure would be to assess what the general public considers a normal or acceptable head shape in children with craniosynostosis., Method: Twenty-two unique images were presented via an online crowdsourcing survey of a severe case of non-syndromic sagittal craniosynosis gradually corrected to an age and gender matched normalized head shape. Participants were recruited via the Sick Kids Twitter account. Participants were invited to rate the head shapes as "normal" or "abnormal.", Results: The 538 participants completed the online survey. Participants were able to reliably and consistently identify normal and abnormal head shapes with a Kappa Score >0.775. Furthermore, participants indicated that a correction of 70% is required in order for the cranial deformity to be regarded as "normal." This threshold closely reflects a normal Cranial Index, which is a widely used morphometric outcome in craniosynostosis., Conclusion: Crowdsourcing provides an ideal method for capturing the general population's perspective on what constitutes a normal and acceptable head shape in children with sagittal craniosynostosis. Laypersons are able to reliably and consistently distinguish cranial deformities from a "normal" head shape. The public indicates a threshold correction of 70% in sagittal craniosynosis to regard it as a "normal" head shape.

Syngnathia is a rare facial anomaly associated with neonatal problems, including a compromised airway, and the inability to take in solids and/or liquids. Syngnathia is included within the spectrum of oromandibular limb hypogenesis syndrome, an extremely rare condition characterized by varying degrees of congenital malformation involving the tongue, mandible, and limbs. In this report, we describe the case of a 41-day-old Caucasian female infant who was unable to open her mouth beginning at birth. The authors performed osteotomies to separate fused bone, and placed a bite block at the osteotomy sites to prevent bone fusion recurrence. At 2 years of follow-up patient remained with 14 mm of mouth opening.

Many methods have been devised to repair cranial defects. Here, we report the use of a simple technique for the repair of a congenital cranial defect associated with aplasia cutis congenita (ACC).A newborn baby at 39 weeks of gestation was consulted with a scalp and cranial defect at the vertex measuring 3 × 1.5 cm. A 3-D CT scan of the skull confirmed the presence of a cranial defect at the sagittal suture and a normal brain structure. On the 13 day of life, the newborn was taken to an operating room. An autologous bone graft was harvested from adjacent normal parietal bone and grafted into the debrided congenital cranial defect. The soft tissue defect was then covered by rotation flaps.The postoperative 3-D CT scan presented a well-positioned autologous bone graft. At 1 month postoperatively, the skull contour was normal and there was no palpable defect.We report a successful surgical outcome for a congenital cranial and soft tissue defect in ACC treated using an autologous bone graft and rotation flaps. Although conservative therapy may be an alternative option, we recommend appropriate surgical reconstruction in patients at risk of potentially fatal complications.

Unicoronal craniosynostosis is the second most common type of nonsyndromic craniosynostosis: it is characterized by ipsilateral forehead and fronto-parietal region flattening with contralateral compensatory bossing. It is a complex condition; therefore, which is difficult to treat because of the asymmetry in the orbits, cranium, and face. The aim of this study is to understand optimal osteotomy locations, dimensions, and force requirements for surgical operations of unicoronal craniosynostosis using a patient-specific finite element model and - at the same time - to evaluate the potential application of a new device made from Nitinol which was developed to expand the affected side of a unicoronal craniosynostosis skull without performing osteotomies. The model geometry was reconstructed using Simpleware ScanIP. The bone and sutures were modeled using elastic properties to perform the finite element analyses in MSc Marc software. The simulation results showed that expanding the cranium without osteotomy requires a significant amount of force. Therefore, expansion of the cranium achieved by Nitinol devices may not be sufficient to correct the deformity. Moreover, the size and locations of the osteotomies are crucial for an optimal outcome from surgical operations in unicoronal craniosynostosis.

Agnathia-otocephaly complex (AOC) is a rare malformation complex of the first pharyngeal arch that is characterized by agnathia/dysgnathia, microstomia, aglossia/hypoglossia and variable displacement of the ears. Only 11 post-infancy patients with severe AOC have been described in the literature, and the incidence of this malformation complex is estimated to be 1 per 70,000 births. In this brief clinical study, the authors describe the case of an 18-year-old female diagnosed with AOC who underwent a 3-step mandibular distraction protocol with an external distraction device. The surgical protocol the authors used was unique in that we first placed a tissue expander in the submental area to enlarge the skin envelope in an effort to mitigate skeletal relapse from soft tissue forces. Furthermore, the way in which the authors slowed the activation of the distraction device to allow for soft tissue healing behind the pins was a novel component of the patient's treatment. The 3-step mandibular distraction protocol the authors present in this study increased the length of the mandible by 20 mm, and nearly doubled the size of the patient's mandible from an initial volume of 3.62 cm to a post-operative volume of 6.89 cm. Future surgeries will aim to improve the function of our patient's expanded mandible. Most important of all, the surgical treatment authors are presenting led to a significant improvement in our patient's physical appearance and 3d quality of life.

A 27-year-old woman with moderate congenital ptosis and a positive Marcus-Gunn jaw winking reflex underwent levator resection surgery to correct the ptosis. Preoperatively, a normal Bell's reflex was documented. Postoperatively, she developed an inverse Bell's reflex and increased symptoms of ocular surface exposure. The Bell's reflex normalised in a week, with resolution of the corneal exposure. Reversal of the Bell's reflex can be an unforeseen complication following maximal levator resection. The early postoperative care in such cases is crucial, and the cornea must be protected from exposure changes. Accurate documentation of the Bell's phenomenon preoperatively is vital to recognise this rare event and plan management., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Purpose: The aim of the authors study is to demonstrate the soft tissues changes in the eyelid-brow area (ELBA) in patients with long-face syndrome after LeFort I osteotomy and impaction movements., Materials and Methods: To perform this study the authors have analyzed retrospectively orthognathic patients with at least 1 years of completely follow-up. The inclusion criteria were: long-face syndrome according to Farkas' studies and vertical maxillary shortening movement without considering whether movements have been made in the other 2 planes of space (sagittal and horizontal).Orthognathic patients in which the maxillary impaction movement did not correlate to the long-face syndrome represented the control group. In this group too the authors did not consider if other movements were performed.Size and shape of the eyelid and the eyebrow was assessed on the frontal patients photos calibrated on the three-dimensional soft tissue volume imported from cone beam computed tomography.Two reference lines were taken: a horizontal line from the nasal point passing through both medial canthal angles and a perpendicular line through the pupil centre bilaterally. Then the measures were taken.The same operator (A.C.) took all of the measurements.In both groups, the preoperative measures were then compared with the postoperative ones. Then the soft tissue changes in both groups were compared., Results: The results demonstrate different reaction of the ELBA after orthognathic surgery. The ELBA's position changes in long-face patients in a higher position after maxillary impaction. The authors did not obtain the same results in patients who do not have long-face syndrome., Conclusions: Repositioning skeletal bases in patients with long face causes a change in the ELBA's morphology.

Congenital fusion of the jaws (syngnathia) is a rare and severe disorder. The authors report a case of bony fusion of the left mandible with the maxilla and zygomatic complex in a 5-day-old male who was not able to feed and open his mouth normally. Early surgery was performed to release the bony fusion on the tenth day of life. The authors present a new technique based on the intraoperative use of 3-dimensional surgical guides.

A successful surgical reconstruction case of a 9-year-old boy with agnathia is presented. The reconstruction was preceded by intraoral expansion of the oral lining and submandibular skin. Four months after initiation of the expansion, the mandible was reconstructed by free vascularized fibula osteocutaneous flap. The 14.5 cm segment of harvested fibula was osteotomized in 4 places to build a replica of the mandible with 3 cm ascending ramus, 3.5 cm body and 1.5 cm symphysis, fixated by titanium micro plating system. The reconstructed mandible was placed between the expanded oral lining and the submandibular skin, with its condylar head placed under the glenoid fossa. After reconstruction, he looked more natural with a chin projection, which he never had. His mother was very happy to see his son being able to show his feeling with facial expression. At last follow-up, he is 14 years and 10 months old, 5 years and 2 months after surgery leading a happy life with his family.

Background: Postural control is classically described as being based on the visual, vestibular, and proprioceptive musculo-articular sensory systems. The influence of mandibular proprioception on postural stabilization remains controversial. Most previous studies analyzed how postural stability is influenced by partial changes in mandibular proprioception (dental occlusion and jaw position)., Research Question: In the present experiment, we asked whether drastic mandibular changes, resulting from orthognathic surgery (including dental, joint and muscular efferents), modify postural control., Methods: The analyzes were performed in 22 patients tested before, and 2.5 months, after orthognathic surgery for treatment of dysmorphic jaws. Experiments were performed under 4 experimental conditions: 2 visual conditions: Eyes Open (EO) and Eyes Closed (EC), and 2 occlusal conditions: Occlusion (OC: mandible positioned by the contact of the teeth), and Rest Position (RP: mandible positioned by the muscles without tooth contact). The analyses focused on head orientation in the frontal plane and on postural stabilization in a static task, consisting of standing upright., Results: The results show that, 2.5 months after orthognathic surgery, head orientation in the frontal plane was improved, since patient's external intercanthal lines became closer to the true horizontal line when they were tested EC and in OC condition. Postural responses, based on the wavelet transformation data, highlight an improvement in maintaining an upright stance for all the tested sensory conditions. However, such improvement was greater in the EC and RP conditions., Significance: These results show, for the first time, that after drastic mandibular changes, the weight of proprioceptive cues linked to the mandibular system may be so enhanced that it may constitute a new reference frame to orient the head in space, in darkness, and improve static postural stabilization, even in the presence of visual cues., (Copyright © 2018 Elsevier B.V. All rights reserved.)

The aim of this study was to evaluate the precision of bimaxillary surgery performed to correct vertical maxillary excess, when the procedure is sequenced with mandibular surgery first or maxillary surgery first. Thirty-two patients, divided into two groups, were included in this retrospective study. Group 1 comprised patients who received bimaxillary surgery following the classical sequence with repositioning of the maxilla first. Patients in group 2 received bimaxillary surgery, but the mandible was operated on first. The precision of the maxillomandibular repositioning was determined by comparison of the digital prediction and postoperative tracings superimposed on the cranial base. The data were tabulated and analyzed statistically. In this sample, both surgical sequences provided adequate clinical accuracy. The classical sequence, repositioning the maxilla first, resulted in greater accuracy for A-point and the upper incisor edge vertical position. Repositioning the mandible first allowed greater precision in the vertical position of pogonion. In conclusion, although both surgical sequences may be used, repositioning the mandible first will result in greater imprecision in relation to the predictive tracing than repositioning the maxilla first. The classical sequence resulted in greater accuracy in the vertical position of the maxilla, which is key for aesthetics., (Copyright © 2017 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.)

Background: This study aimed to evaluate a novel subtractive method for the precise calculation of alveolar cleft defect volume before surgery using the three-dimensional imaging software Mimics., Methods: Ten patients (3 female, 7 male, age range 8-12 years) with unilateral alveolar cleft were enrolled in the study. Preoperative helical computed tomography scans were performed for all patients. A new subtractive method was introduced to precisely calculate the alveolar cleft defect volume before surgery with the aid of Mimics. The DICOM data of every patient were processed using the subtractive method. Statistical analyses were performed using Pearson's correlation test and t test., Results: The mean volume of the alveolar cleft defect was 1811.97 ± 817.90 mm. The mean time spent to calculate an alveolar cleft defect volume was 60.60 ± 11.67 minutes. The Pearson correlation test (r = 0.38, P = 0.279) indicated a poor correlation between alveolar cleft defect volume and calculation time., Conclusions: Preoperative knowledge of defect volume is crucial in alveolar cleft repair. Accurate estimation of graft volume in alveolar cleft patients can be performed using this new subtractive method. Compared with previous methods, this new subtractive method is time-saving and suitable for both unilateral and bilateral clefts. Surgeons can use this method to design a surgical plan for each patient before surgery within a short time, and with less cost.

This case report presents two cases of rare congenital malformation, i.e syngnathia. First case is of 2-day infant with bilateral fusion of maxilla and mandible, leaving a small anterior portion. After consultation with other concerned specialties, early intervention was planned and fusion was released to facilitate feeding. The infant suffered from frequent respiratory tract infections and subsequently died at the age of ten months. The second case is of 8-month baby girl with unilateral congenital maxillomandibular bony fusion without any other anomaly. She underwent general anesthesia for thorough examination and release of soft tissue union. Second surgery was performed after few months for removal of bony fusion. Good mouth opening was seen on 1 month follow-up.

The sagittal split ramus osteotomy and intraoral vertical ramus osteotomy carry the potential risk of postoperative nerve paralysis, bleeding, and fracture and dislocation of the condyle. In 1992, Choung first described the intraoral vertico-sagittal ramus osteotomy for the purpose of avoiding postoperative dislocation of the condyle. However, there is still potential for damaging the inferior alveolar nerve and maxillary artery with this technique. The authors have developed a modified technique to minimize these risks. An evaluation of surgical experience and patient outcomes with the use of this technique is presented herein. One hundred twenty-two sides in 97 Japanese patients diagnosed with a jaw deformity were analyzed. This technique includes a horizontal osteotomy that is performed at a higher position than in the original Choung procedure. Intraoperatively, there was no unexpected bleeding from the operative site. Proximal segment dislocation from the glenoid fossa was observed on one side (0.82%). Non-union of the osteotomy was not observed in any patient. Intraoperative fracture of the coronoid process occurred in 2.46%, but none necessitated treatment of the fracture. Nerve dysfunction was found in 2.46% at the 12-month postoperative follow-up. The modified technique presented herein was developed to reduce postoperative nerve dysfunction and intraoperative hemorrhage., (Copyright © 2017 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.)

Background: The surgical excision of anatomic obstructions such as adenoids, palatine or lingual tonsils are commonly performed in children with sleep disordered breathing (SDB). Imaging studies measuring airway changes post-surgery in the SDB pediatric population are scarce, rarely addresses the nasal cavity, and are based on global measures (e.g. volume) that do not represent the complexity of the upper airway anatomy. The purpose of this pilot is to test the feasibility in using cone beam CT (CBCT) to analyze the nasal and pharyngeal airway space post-surgery using meaningful methods of analyses, and correlating imaging findings with clinical outcomes in children with SDB symptoms and maxillary-mandibular disproportion., Methods: Twelve non-syndromic children with SDB symptoms and jaw disproportions were evaluated by interdisciplinary airway team before and after upper airway surgery. CBCT and OSA-18 quality of life questionnaire pre and post-operatively were completed. Conventional and new airway variables were measured based on 3D models of the upper airways and correlated with OSA-18. Conventional measures include volume, surface area, and cross-sectional area. New airway measures include constriction and patency; point-based analyses., Results: Eight females and four males were 8.8 ± 2 years with mean BMI of 18.7 ± 3. OSA-18 improved, median (lower quartile-upper quartile) from 64.2 (54.7-79.5) to 37.6 (28.7-43) postoperatively, p < 0.001. The median of all airway measures improved however with very wide range. Subjects with the smallest amounts of constriction relief and/or gain in airway patency presented with least improvement in OSA-18. New airway measures show strong correlation with changes in OSA-18 (ρ = 0.44 to 0.71) whereas conventional measures showed very weak correlation (ρ = -0.04 to 0.37)., Conclusions: Using point-based analyses, new airway measures better explained changes in clinical symptoms compared to conventional measures. Airway patency gained by at least 150% and constriction relief by at least 15% showed marked improvement in OSA-18 by 40-55%, after surgery in the tested cohort.

Introduction: Management of growing patients with severe developmental jaw abnormalities can be very difficult. Early surgical intervention may be warranted in situations where function (e.g., mastication, swallowing, breathing, or speech) and/or psychological well-being could be negatively affected. Many surgeons and orthodontists are reluctant to recommend a surgical treatment option for growing patients with severe developmental jaw abnormalities because of their age. Specific surgical procedures can be performed during growth to correct developmental jaw abnormalities with predictable results. A sound understanding of the facial growth and the effects of the surgical procedures on subsequent growth is essential when managing growing patients with severe developmental jaw abnormalities. Children with severely progressive congenital deficiencies affecting function/health should be distinguished from ones with severe developmental jaw abnormalities that can be managed later in life. In this review, we will focus on the management of growing patients with developmental jaw abnormalities who seek orthodontic treatment, rather than patients with progressive congenital deformities affecting function and/or health.

The purpose of this study was to clarify the impact of orthognathic surgery on oral health-related quality of life (OHRQOL) in patients with jaw deformities. The subjects were 65 patients (21 males and 44 females) who underwent orthognathic surgery. The mean age of the patients was 23.6 years. Forty-seven patients had skeletal class III malocclusions, eight patients had skeletal class II, and 10 patients had skeletal class I with facial asymmetry and/or open bite. OHRQOL was assessed using the Japanese version of the Oral Health Impact Profile (OHIP-J54) before and 6 months after surgery. While OHIP-J54 scores in the patients before surgery were significantly higher than those in the control subjects, OHIP-J54 scores after surgery were significantly lower than those before surgery. OHIP-J54 scores in older patients were significantly higher than those in younger patients. In conclusion, most patients with jaw deformities have lower OHRQOL than individuals with normal occlusion, and orthognathic surgery has a positive impact on OHRQOL. The determination of OHRQOL in patients with jaw deformities seems to be very useful for understanding the patients' problems and for assessing the extent of changes in terms of patient well-being., (Copyright © 2016 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.)

Three-dimensional (3D) imaging is an important tool for diagnostics, surgical planning, and evaluation of surgical outcomes in craniofacial procedures. Gold standard for acquiring 3D imaging is computed tomography that entails ionizing radiations and, in young children, a general anaesthesia. Three-dimensional photographic imaging is an alternative method to assess patients who have undergone calvarial reconstructive surgery. The aim of this study was to assess the utility of 3D handheld scanning photography in a cohort of patients who underwent spring-assisted correction surgery for scaphocephaly. Pre- and postoperative 3D scans acquired in theater and at the 3-week follow-up in clinic were postprocessed for 9 patients. Cephalic index (CI), head circumference, volume, sagittal length, and coronal width over the head at pre-op, post-op, and follow-up were measured from the 3D scans. Cephalic index from 3D scans was compared with measurements from planar x-rays. Statistical shape modeling (SSM) was used to calculate the 3D mean anatomical head shape of the 9 patients at the pre-op, post-op, and follow-up. No significant differences were observed in the CI between 3D and x-ray. Cephalic index, volume, and coronal width increased significantly over time. Mean shapes from SSM visualized the overall and regional 3D changes due to the expansion of the springs in situ. Three-dimensional handheld scanning followed by SSM proved to be an efficacious and practical method to evaluate 3D shape outcomes after spring-assisted cranioplasty in individual patients and the population.

Craniofacial clefts are rare, severe challenges for surgeons about which there is limited literature. Tessier Number 4 (No. 4) clefts are one of the most complex craniofacial anomalies and present difficulties in surgical treatment. The most-common deformities associated with Tessier No. 4 clefts are displacements of the lower eyelids, medial canthus, and ala and decreased distance between the lower eyelids and lips. In surgery to correct these deformities, the greatest challenges are the design and the placement of the landmarks and incisions. Because of its relative rarity and wide range of severity, no definitive operative methods have been accepted for Tessier No. 4 facial cleft. The present study presents a new lip-rescue flap technique as an alternative approach for reconstructing Tessier No. 4 facial clefts.

Background: Mandibular distraction osteogenesis is the process of generating new bone in a gap between 2 bone segments in response to the application of graduated tensile stress across the bone gap. These distraction forces applied to bone also create tension in the surrounding soft tissues resulting in simultaneous expansion of the functional soft tissue matrix., Aim: To investigate and evaluate the changes in facial soft tissue following mandibular distraction osteogenesis and their relationship with the changes in bone movements., Materials and Methods: Mandibular elongation of 13 patients was done by the use of bilateral distraction osteogenesis in 10 patients and unilateral distraction osteogenesis in 3 patients, soft tissue evaluation was done by measuring 4 facial lines (tragus-commissure of mouth, lateral canthus-commissure of mouth, medial canthus-commissure of mouth, and lower lip length). The measurements were done by the use of measurement tape preoperatively and after the consolidation period., Results: Soft tissue changes were observed in all patients. There was highly significant increase in tragus-commissure line in all patients, significant increase in lateral canthus-commissure line in 10 patients, and significant increase in medial canthus-commissure line and lower lip length in 9 patients. The lines' relation with the overjet was highly significant in right side of tragus-commissure line, lateral canthus-commissure line, medial canthus-commissure line, and lower lip length, and significant in left side of tragus-commissure line, lateral canthus-commissure line, and medial canthus-commissure line CONCLUSION:: Distraction osteogenesis has recognizable effect on facial soft tissue profile in addition to its effect on skeletal structure.

Purpose: This article will review the etiology, risk factors, history, and physical assessment of temporomandibular joint disorders (TMDs). In particular, this article discusses the role of the pediatric provider in diagnosing and beginning the initial treatment of TMDs, as well as the appropriate treatment plans. It also reveals some of the controversies regarding etiology and treatment of TMDs, as well as the paucity of research specific to TMDs in pediatrics., Data Sources: A computerized search in PubMed and Ovid Medline, from 2006 to 2012, was conducted. A few seminal articles were included that were published before 2006. Hand searching was also performed, which included a few articles between 2012 and 2015., Conclusions: Although TMDs are mostly found in adults, it is also a finding in pediatrics, which increases in prevalence during adolescence. More research specific to pediatric patients with TMDs needs to be conducted so that pediatric-specific care can be provided., Implications for Practice: TMD is a condition found in the pediatric population, and it is important for providers to take an adequate history and physical examination that incorporates the temporomandibular joint (TMJ) and muscles of mastication. It is also critical that providers begin initial education and management, followed by appropriate referrals., (©2015 American Association of Nurse Practitioners.)

Facioscapulohumeral muscular dystrophy (FSHD) is a subtype of muscular dystrophies which reduces the muscle strength, especially the regions of scapular, shoulder, and upper arms, progressively. According to progressive muscle weakness in FSHD, postoperative stability of patient with FSHD after orthognathic surgery is not reliably acquired same as healthy subjects. A 32-year-old woman with FSHD underwent orthodontic and orthognathic surgical treatment due to jaw deformity. She has been followed up more than 3 years after surgery and acquired skeletal stability. This patient is the first report that showed long-term skeletal stability after orthognathic surgery in patient with FSHD. This patient report suggests that it is possible to apply orthognathic surgical treatment to patients with FSHD.

Introduction: Treacher Collins syndrome is a rare disorder characterized by several orofacial findings including malar deficiency and hypoplastic mandibles. These patients often require a combined orthodontic-orthognathic approach to correct their malocclusion. This is most often characterized by a short posterior vertical height and an anterior open bite. Orthognathic correction often requires Le Fort I and bilateral sagittal split osteotomies. No long-term stability results have been reported after bimaxillary surgery in Treacher Collins patients., Methods: A retrospective review of all Treacher Collins patients evaluated for orthognathic surgery by a single surgeon from 1993 to 2007 was performed. Patients were divided into groups who required surgery and those who did not. Part I analyzed the cephalometric differences between the surgical (S) and nonsurgical (NS) groups. Part II of the study assessed the preorthodontic treatment (T1), preoperative (T2), immediate postoperative (T3), and 1-year postoperative (T4) cephalometric measurement variables to determine the net surgical movement (T3 - T2) and relapse (T4 - T3)., Results: Twenty-two patients met the inclusion criteria, of which 11 had occlusal relationships requiring orthognathic surgery. Nine out of 11 chose to have surgery. At baseline, surgical patients exhibited a statistically significant retruded maxilla as measured by SNA and midface length compared to the NS group. In addition, the S group also had an increased gonial angle. There were significant movements in all maxillary and mandibular measurements. There was a significant relapse in the palatal plane angle when the maxilla was anteriorly impacted, with a 2.8-mm average relapse of the advancement. Relapse of the counterrotation movement of the mandible was identified, but this was not significant. Relapse did not affect the final occlusal result, which may have been compensated with postsurgical orthodontic treatment., Conclusion: Bimaxillary orthognathic surgery in the Treacher Collins patients may be performed safely with long-term dental and skeletal stability., (Copyright © 2015 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.)

Agnathia-otocephaly complex (AOC) is characterized by mandibular hypo- or aplasia, ear abnormalities, microstomia, and microglossia. Though rare and often fatal, this is the first report detailing various reconstructive strategies beyond infancy as well as longitudinal follow-up into adulthood. All patients with AOC treated at our institution over a 30 year period were reviewed. Four patients were identified, one with agnathia, one with micrognathia. Two males with nanognathia (defined as a symphyseal remnant without body nor ramus) were also included. The mean follow-up was 17 years. All four underwent perinatal tracheostomy and gastrostomy-tube placement. Commissuroplasties were typically performed before 3 years of age and repeated as necessary to allow for oral hygiene. Mandibular reconstruction was most successful with rib between ages 3 and 8, after which time, free fibula transfer was utilized. Due to some resoprtion or extrusion, all patients underwent repeated bone grafting procedures. Tissue expansion of the neck was used to restore the lower third of the face, but was most successful in the teenage years. At last follow-up of the eldest patients, one was in college while another was pursuing graduate education. AOC need not be a fatal nor untreatable condition; a reasonable quality of life can be achieved. Although the lower-facial contour may be improved, and a stoma created, the lack of musculature make deglutition virtually impossible with current therapies. Just as transplantation has emerged as a modality for facial restoration following severe trauma, so too may it be a future option for congenital deformities.

Cleido-cranial dysplasia, often referred to as Scheuthauer-Marie-Sainton syndrome, is an autosomal dominant disorder of the musculo-skeletal system. Patients with cleido-cranial dysplasia are characterized by short stature, frequent varus or valgus hip, kyphoscoliosis, underdevelopment of the scapulas and the sternum, incorrect number of ribs. The most characteristic feature is unilateral or bilateral, partial or total underdevelopment of clavicles. Mental development is not affected in this syndrome. Malocclusion, occlusal irregularities, multiple supernumerary teeth, impacted teeth, and persistent milk teeth are found in the stomatognathic system. Teeth often have abnormal anatomy. Gothic palate, cleft hard and soft palate are diagnosed. The aim of this paper is to present a case of a 12-year-old boy diagnosed with irregularities in the masticatory system involving an additional number of retained teeth. The boy was referred by an orthodontist for surgical and orthodontic team therapy. The case presented confirms the observations of other authors that only the multi-specialty collaboration of a pediatrician, a geneticist, an orthopedist, an orthodontist, a maxillofacial surgeon, an implant prosthetic surgeon and a physiotherapist can provide proper diagnosis and treatment.

Purpose: A significant number of patients suffer from craniomaxillofacial (CMF) deformity and require CMF surgery in the United States. The success of CMF surgery depends on not only the surgical techniques but also an accurate surgical planning. However, surgical planning for CMF surgery is challenging due to the absence of a patient-specific reference model. Currently, the outcome of the surgery is often subjective and highly dependent on surgeon's experience. In this paper, the authors present an automatic method to estimate an anatomically correct reference shape of jaws for orthognathic surgery, a common type of CMF surgery., Methods: To estimate a patient-specific jaw reference model, the authors use a data-driven method based on sparse shape composition. Given a dictionary of normal subjects, the authors first use the sparse representation to represent the midface of a patient by the midfaces of the normal subjects in the dictionary. Then, the derived sparse coefficients are used to reconstruct a patient-specific reference jaw shape., Results: The authors have validated the proposed method on both synthetic and real patient data. Experimental results show that the authors' method can effectively reconstruct the normal shape of jaw for patients., Conclusions: The authors have presented a novel method to automatically estimate a patient-specific reference model for the patient suffering from CMF deformity.

Introduction: Congenital maxillomandibular syngnathia, or fusion of the jaws, is a rare condition that has a broad spectrum of presentations. The restricted mouth opening can lead to issues with feeding, swallowing, and respiration resulting in failure to thrive and temporomandibular joint ankylosis. Early recognition and treatment is necessary for proper growth and development., Case Report: We report a 1-day-old male with isolated bilateral soft tissue alveolar fibrous bands. He presented with difficulty feeding secondary to trismus. No bony or muscular involvement in the synechiae was noted and the remainder of the physical exam was unremarkable. The bilateral alveolar synechiae were divided under local anesthesia using surgical scissors. The patient immediately showed improvement in mouth opening and had resolution of his feeding problems. He is now gaining weight and developing appropriately., Discussion: The accompanying review of the literature demonstrates only 11 cases worldwide of isolated maxillomandibular fusion. Depending upon the composition of the synechiae, simple surgical division under local anesthesia can be curative., (Published by Elsevier Inc.)

The purpose of this study was to determine the effect of orthognathic surgery on psychological status. The subjects were 119 patients (38 males and 81 females, mean age 25.5±9.4 years) who underwent orthognathic surgery. They were divided into class III (84 patients), class II (20 patients), and class I (15 patients) groups according to the anteroposterior skeletal pattern, and they were also divided into an asymmetry group (51 patients) and a symmetry group (68 patients). We assessed psychological status using the Minnesota Multiphasic Personality Inventory (MMPI) before surgery and at more than 6 months after surgery. The MMPI scores for the depression, hysteria, psychasthenia, and social introversion scales were significantly higher than standard values before surgery, and the hypomania scale significantly lower. The cannot say scale, depression scale, and hysteria scale decreased significantly after surgery. A comparison of MMPI scores among the groups showed the depression scale in the class III group to be higher than those in the class I and II groups; there was no significant difference between the asymmetry and symmetry groups. In conclusion, orthognathic surgery has a positive influence on the psychological status of patients with jaw deformities, especially patients with skeletal class III malocclusion., (Copyright © 2015 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.)

Background: The aesthetic and functional outcomes surrounding Le Fort-based, face-jaw-teeth transplantation have been suboptimal, often leading to posttransplant class II/III skeletal profiles, palatal defects, and "hybrid malocclusion." Therefore, a novel technology-real-time cephalometry-was developed to provide the surgical team instantaneous, intraoperative knowledge of three-dimensional dentoskeletal parameters., Methods: Mock face-jaw-teeth transplantation operations were performed on plastic and cadaveric human donor/recipient pairs (n = 2). Preoperatively, cephalometric landmarks were identified on donor/recipient skeletons using segmented computed tomographic scans. The computer-assisted planning and execution workstation tracked the position of the donor face-jaw-teeth segment in real time during the placement/inset onto recipient, reporting pertinent hybrid cephalometric parameters from any movement of donor tissue. The intraoperative data measured through real-time cephalometry were compared to posttransplant measurements for accuracy assessment. In addition, posttransplant cephalometric relationships were compared to planned outcomes to determine face-jaw-teeth transplantation success., Results: Compared with postoperative data, the real-time cephalometry-calculated intraoperative measurement errors were 1.37 ± 1.11 mm and 0.45 ± 0.28 degrees for the plastic skull and 2.99 ± 2.24 mm and 2.63 ± 1.33 degrees for the human cadaver experiments. These results were comparable to the posttransplant relations to planned outcome (human cadaver experiment, 1.39 ± 1.81 mm and 2.18 ± 1.88 degrees; plastic skull experiment, 1.06 ± 0.63 mm and 0.53 ± 0.39 degrees)., Conclusion: Based on this preliminary testing, real-time cephalometry may be a valuable adjunct for adjusting and measuring "hybrid occlusion" in face-jaw-teeth transplantation and other orthognathic surgical procedures.

To conduct a study on ptotic eyelids with Marcus Gunn jaw-winking ptosis operated via a technique of modified levator plication, prospective interventional case series. Ten ptotic eyelids with Marcus Gunn jaw-winking phenomenon (MGJWP) underwent modified levator plication surgery. Postoperatively, all cases were followed up for at least 6 months. Outcome parameters included amount of ptosis correction, amount of MGJWP correction, palpebral aperture height, lid lag, and lagophthalmos. The mean amount of ptosis was 4.25 ± 0.79 mm (range of 3-6 mm), mean amount of MGJWP was 5.10 ± 2.27 mm (range 2-9 mm), and the mean levator function was 8.3 ± 2.27 mm (range of 4-12 mm). At 6 months follow-up, good correction of ptosis was seen in nine out of ten patients. Resolution of MGJWP (≤1 mm of excursion of upper eyelid with synkinetic mouth movement) was seen in three patients. Improvement in MGJWP (>1 mm of excursion of upper eyelid with synkinetic mouth movement) was seen in seven patients. The mean post-operative lagophthalmos was 0.80 ± 0.88 mm. The modified levator plication technique was effective in the treatment of MGJWP. This modified technique of levator plication is anatomically less destructive and hence more acceptable, with the added advantages of less post-operative lagophthalmos and no lid contour defects.

An array of genetic syndromes has been associated with intra-oral adhesions in neonates. The primary medical issues arise from airway obstruction, feeding difficulties and poor oral development, specifically with cleft palate lateral synechia syndrome (CPLSS). Despite this, a paucity of data exists for the clinical management of intra-oral adhesions in this population. We report the cases of a father and daughter diagnosed with CPLSS who presented with respiratory and feeding difficulties at birth undergoing surgical correction. Early surgical ligation of intra-oral bands allows for a stabilization of the airway, improved feeding and oral development with a good long-term outcome., (Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.)