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.

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.

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.

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.

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.

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.

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.

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.

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.

Successful Reconstruction of Agnathia by Intraoral Expansion and Free Vascularized Fibula Flap.

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.

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.

Congenital Unilateral Zygomatico-Maxillo-Mandibular Fusion.

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 Novel Method to Calculate the Volume of Alveolar Cleft Defect Before Surgery.

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 ±â€Š817.90 mm. The mean time spent to calculate an alveolar cleft defect volume was 60.60 ±â€Š11.67 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.

Early Orthognathic Surgery: A Review.

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.

Changes in Soft Tissue Measurements After Mandibular Distraction Osteogenesis.

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.