Maxillomandibular Fusion Along with Fusion of Dorsal Surface of the Tongue to the Hard Palate-A Case Report.

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.

Free Fibula Flap for the Treatment of Agnathia in a 10-Year-Old With Severe Agnathia-Otocephaly Complex.

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.

Early Surgical Intervention of Congenital Syngnathia.

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.

Predicting Changes in Cephalic Index Following Spring-mediated Cranioplasty for Nonsyndromic Sagittal Craniosynostosis: A Stepwise and Machine Learning Algorithm Approach.

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 (ß=0.035), anterior spring length (ß=0.005), posterior spring length (ß=0.004), and duration of spring placement (ß=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.

Anesthetic management in an infant with tetra-amelia syndrome with congenital maxillomandibular fusion: A case report.

A 10-month-old girl who had tetra-amelia syndrome and congenital maxillomandibular fusion (syngnathia) was scheduled for the surgical fusion separation. Anesthetic management for this case was considerably challenging. Standard monitoring was still applied to the patient's extremities. IV access was suspected to be difficult but firmly needed before intubation to provide resuscitation during an emergency. Connecting anesthetic circuit with nasopharyngeal airway was the preferred technique due to its benefits such as maintaining spontaneous ventilation, providing inhaled anesthetic, as well as monitoring oxygenation and ventilation. Importantly, the cornerstones for handling such complicated cases are multidisciplinary approach and teamwork.

Management of sagittal craniosynostosis: morphological comparison of eight surgical techniques.

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.

Treatment of Sagittal Craniosynostosis Using Osteoplastic Parietal Craniotomy Flap With Distraction Osteogenesis: A Variation on Technique.

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.

Immediate Resection and Reconstruction of Encephalocele in the Craniofacial Region.

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.

Comparison of Osteogenic Potentials of Dental Pulp and Bone Marrow Mesenchymal Stem Cells Using the New Cell Transplantation Platform, CellSaic, in a Rat Congenital Cleft-Jaw Model.

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.

Hope Springs Eternal: Insights Into the Durability of Springs to Provide Long-Term Correction of the Scaphocephalic Head Shape.

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.

Vertical Distraction for the Treatment of Congenital Syngnathia.

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.

Total facial nerve injury during mandibular distraction osteogenesis.

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.

Syngnathia in Spectrum of Oromandibular Limb Hypogenesis Syndrome.

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.

Public Perception of a Normal Head Shape in Children With Sagittal Craniosynostosis.

: 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.

Reconstruction of Congenital Cranial Defect Using Autologous Bone Graft in Aplasia Cutis Congenita.

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.

Computational Evaluation of Potential Correction Methods for Unicoronal Craniosynostosis.

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.

Surgical Approach in a Patient With Agnathia-Otocephaly Complex: Three-Stage Mandibular Distraction Protocol.

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.

Inverse Bell's phenomenon: a rare complication of levator resection surgery in a case of congenital ptosis.

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.

Morphological Eyelids Changing After Orthognathic Surgery in Long-Face Syndrome.

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.