Fronto-facial monobloc advancement with internal distraction (FFMBA) is a central procedure in the management of faciocraniosynostoses. In techniques with internal distraction, two sets of devices are generally positioned: bilateral fronto-orbital and temporo-zygomatic distractors, using a temporal tongue and groove osteotomy design. It is believed that distractors must be positioned as parallel as possible in the horizontal and sagittal planes to avoid mechanical conflicts between the sliding bone fragments of the tongue and groove during distraction, and thus optimize the advancement amplitude. Several approaches involving surgical planification and guides for distractor positioning have thus been proposed to monitor distractor placement. To explore the need for surgical planification in distractor placement, the parallelism of the position of the 4 distractors was assessed in 19 FFMBA procedures and we correlated a set of 10 distractor angles with the degree of advancement. We report that the horizontal cut of the tongue and groove can be used as a landmark for the positioning of the lower, temporo-zygomatic, distractor in fronto-facial monobloc advancement. Other parameters (relative position of the two homolateral and the two contralateral distractors and the orientations of the vertical and horizontal cuts of the tongue and groove) do not interfere with distraction, other things being equal. Our results indicate that distractor orientation is not a critical issue in fronto-facial monobloc advancement when devices are positioned as parallel as possible based on visual monitoring.

BACKGROUND: Pitt-Hopkins syndrome (PTHS) is a rare genetic syndrome associated with neurodevelopmental disorders and craniofacial dysmorphisms caused by variations in the TCF4 transition factor. The aim of this article was to report the case of two twin infants diagnosed with PTHS, confirmed by the identification of a heterozygous pathogenic variant in the TCF4 gene through DNA extracted from a buccal swab.
METHODS: Both infants presented with craniofacial asymmetry with a metopic crest and cranial deformity. During the diagnostic investigation, computed tomography with three-dimensional reconstruction of the skull showed premature fusion of the left coronal and metopic sutures in both twins. They underwent craniofacial reconstruction at the 9th month of age using a combination of techniques. The postoperative outcomes were satisfactory in both cases.
CONCLUSIONS: To the best of our knowledge, this is the first case report to describe the occurrence of complex craniosynostosis (CCS) in children with PTHS. Further studies are needed to determine whether the co-occurrence of PTHS and CCS described here indicates an association or is explained by chance.

UNASSIGNED: The Pneumosinus Dilatans (PSD) Frontalis is an uncommon condition characterized by abnormal enlargement of the aerated frontal sinus with normal thickness sinus walls. The major complication is aesthetics; however, some cases present with sinus obstructive symptoms.
METHODS: A 32-year-old male presented with complaints of an asymmetrical protrusion on his forehead, as well as recurrent headaches. No signs of sinusitis were detected by periodic examination. Computed tomography demonstrated the presence of large frontal PSD. Due to aesthetic concerns, the patient was selected for forehead aesthetic surgery. The operation was performed through a bi-coronal incision to expose the supraorbital areas. The anterior wall of the right frontal sinus was removed, divided into 2 sections, and fixed into the proper location, and then the sinus outflow was widened. An asymmetric brow lift was then performed to correct the asymmetric brow position. Good results were attained, the patient\'s headache was resolved, and he was pleased with his appearance.
CONCLUSIONS: Although the most prevalent complaint of patients with PSD is aesthetic, some patients exhibit concomitant symptoms, including headaches and sinus obstruction. The constriction and partial obstruction of the sinus ostium may cause sinus cavity hypertrophy. Therefore, re-establishing sufficient drainage for the sinus by opening the sinus ostium is recommended during the reconstruction of the forehead\'s natural architecture to reduce headaches and recurrence of sinus hypertrophy.
CONCLUSIONS: A combination of a bi-coronal approach regarding aesthetic surgery and sinus outflow widening achieves a desirable outcome that gives a good short-term follow-up result.

Dermoid and epidermoid cysts are congenital or acquired benign developmental cysts that uncommonly occur in the head and neck region. These cysts represent less than 0.01% of all cysts of the oral cavity and can be found on the tongue, lips and other oral mucosa locations. The palate is less frequently affected with most reported cysts being limited to the soft palate. In this study, we report a case of a dermoid cyst of the hard palate, and discuss its management with a review of the literature.

Posterior cranial vault distraction osteogenesis (PCVDO) is a relatively new paradigm in the treatment of syndromic craniosynostosis, having first been introduced in 2009. PCVDO directly addresses the underdeveloped cranial vault and appears to allow for a larger increase in intracranial volume when compared to traditional techniques. Although reported as safe in the literature, critical appraisal is still required as PCVDO is a relatively uncommon procedure that may require greater numbers to detect true complication rates. The overall reported incidence of serious complications in PCVDO to date is low. This presentation highlights a rare case of sagittal sinus obstruction following posterior cranial vault distraction and raises questions as to the safest technical considerations when planning the operation.

Growing skull fracture (GSF) is a rare complication of head trauma in the pediatric population, commonly observed in children younger than 3 years.
In this report, the authors describe a case of a 3-year-old male child, with clinical features of Ehlers-Danlos syndrome (EDS), who developed a GSF in frontal bone after a crib fall, treated with duraplasty and cranioplasty with autologous craft. Here, pertinent literature was reviewed with an emphasis on surgical techniques, and correlation with the mentioned syndrome.
This is the first case of GSF in association with EDS in the literature. The relevance of the case described concerns the rarity of the condition itself, the atypical presentation, and the intraoperative findings, which showed the important fragility of the dura mater, probably due to EDS. Therefore, this syndrome, besides having influenced the pathogenesis, was also a challenging factor in the surgical treatment.

Aneurysms of the middle meningeal artery (MMA) are exceedingly uncommon and mainly result from traumatic brain damage, but this report describes a case of MMA aneurysm induced by cranial surgery. Surgery was performed on a 34-year-old male with cerebrovascular malformation and cerebral hemorrhage. Cerebral angiography revealed no MMA aneurysm before craniocerebral surgery; however, a postoperative angiogram revealed a new MMA aneurysm. Aneurysms of the MMA are uncommon consequences of brain surgery. Based on our findings, the MMA as well as other meningeal arteries should be avoided while suturing the dura mater tent to prevent aneurysms.

Zygomaticomaxillary complex fractures involve four fracture ends. It is difficult to fully expose the operative area through a main coronal incision, an intraoral incision, and an eyelid incision. To address the partial visual field loss in craniofacial fracture reduction, we attempted to use an augmented reality (AR) navigation system.
Patients with zygomaticomaxillary complex fractures underwent three-dimensional (3D) computed tomography (CT) modeling before surgery, and preoperative plans were designed. The control team used traditional optical navigation to perform the surgery. The experimental team used an AR navigation system. From May 2019 to December 2019, 10 patients with zygomaticomaxillary complex fractures were included in this study. Data were collected after surgery and analyzed.
There was a significant difference between the two groups in the fracture point error (1.35 vs. 1.61, P = 0.02) and fracture reduction time (15.40 vs. 20.40, P = 0.03). However, there was no difference in the operative duration (6.60 vs. 6.65, P = 0.92), blood loss volume (620.00 vs. 580.00, P = 0.83), or incidence of complications.
The AR navigation system used by the research team has good auxiliary effects for reducing zygomaticomaxillary complex fractures. The new surgical method has better accuracy and a shorter reduction time than the traditional surgical method.

BACKGROUND: Atlantoaxial rotatory fixation (AARF) is a rare condition that occurs most commonly in children. The torticollis caused by AARF usually presents as abnormal posturing of the head and neck, with rotation of the chin to the opposite side. AARF in children could occur due to congenital bony malformation, minor trauma, upper respiratory tract infections (Grisel\'s syndrome), postoperatively after head and neck (ENT) surgery, and unknown reasons. AARF in the postoperative patient is a rare and poorly understood entity.
METHODS: The authors present a case report of a 2-year-old boy with Crouzon Syndrome undergoing posterior calvarial vault expansion (PVE) surgery that developed AARF as a complication.
RESULTS: The authors believe that cranial vault surgery should be considered a potential risk procedure for AARF, especially if it is done in susceptible populations (syndromic craniosynostosis patients) with other underlying sequelae (tonsillar ectopia or syringomyelia). During surgery, careful attention should be paid to maintaining a neutral alignment of the patient\'s cervical spine as rotatory movements under anaesthesia and muscle relaxation may be contributory factors.
CONCLUSIONS: AARF should be suspected and investigated in children with painful torticollis after craniofacial surgery.

Lateral proboscis is a rare congenital condition characterized by a cylindrical protuberance on the nasofrontal region accompanied by abnormal nasal development on the affected side. We aimed to describe the management of the lateral proboscis in staged repair. A 7-year-old girl came with a tube-like projection on the left medial canthal region and nasal agenesis on the ipsilateral side. She was diagnosed with lateral proboscis, left microphthalmia, lower eye lid coloboma, and asymmetry in the orbital region. The patient has undergone 3 major surgeries at our institution. The first surgery involved the deconstruction of the tube to form the left nasal body and nostril. The second operation involved trimming of the new nose form and the excision of the bony protrusion directly beneath the base of the pedicle through bifrontal craniotomy. The remaining bone defect was closed using a pericranial flap. The orbital floor was reconstructed using titanium mesh. The third operation involved nasal reconstruction using a costal cartilage graft to create a dorsal nasal and alar framework. The patient healed with no complications, had become less reserved and her grades improved significantly after the operation. Further appointments are being scheduled to evaluate growth distortion and the resulting facial asymmetry. Surgical correction will be planned thereafter to further reconstruct the facial features. Evaluation of patient is necessary to explore possible clinical outcomes and corresponding treatment options. Multidisciplinary management is highly recommended, involving plastic surgeons, neurosurgeons, ophthalmologists, pediatricians, and pediatric psychiatrists in order to improve patient\'s quality of life.