Nasofrontal encephalocele: A case report with literature and management review.

Encephalocele is a rare congenital anomaly characterized by the protrusion of intracranial contents through a defect in the skull base or calvarial. In Southeast Asia, frontoethmoidal encephaloceles are more frequently observed compared to their occurrence in Western countries. Typically, frontoethmoidal encephaloceles present as a visible mass adjacent to the nasal region. In this report, we delineate the case of a 9-year-old boy who presented with a palpable mass on the nasal bridge. Subsequent ultrasound and CT scan evaluations identified a nasofrontal defect with a communicating connection to the intracranial compartment.

Encephalocele presenting with bacteremia and meningitis.

An encephalocele is a cranial defect that allows brain matter to present in other portions of the skull. We present a case of a 57-year-old female who presented to the ICU with fever and unresponsiveness and was diagnosed with meningitis, bacteremia, and pneumonia. After diagnostic imaging was performed, a nasofrontal encephalocele was found and corrected via surgical intervention. The patient's nasofrontal encephalocele caused a predisposition for opportunistic infections due to the exposed and unprotected brain matter, which caused a simple case of sinusitis to turn into a life-threatening case of meningitis and bacteremia.

Nasofrontal meningiomas: retrospective series and review of literature.

INTRODUCTION: Lesions affecting the anterior skull base and involving the paranasal sinuses (PNS), nasal cavity (NC), and orbit are infrequent and include predominantly a wide variety of sinonasal malignancies. Less than 3% of intracranial meningiomas extend extracranially and involve PNS and NC. Given their relatively low incidence, little is known about the treatment outcome of this subset of meningiomas. METHODS: Systematic literature and retrospective review of own institutional series of midline anterior skull base meningiomas with significant PNS and NC involvement were performed. RESULTS: Overall, 21 patients-16 in the literature review group and 5 of our institutional series-were included. Eleven (52.4%) patients had had a prior surgery for midline anterior skull base meningioma. Of patients having reported WHO grade, two were WHO II. Gross total resection was achieved in 16 (76.2%) of patients, utilizing solely transcranial approach in 15 patients, combined endoscopic and transcranial in five patients and purely endoscopic in one patient. Postoperative radiotherapy was administered in three (14.3%) patients, all after total resection via transcranial route, without a history of prior treatment. A postoperative cerebrospinal fluid leak was reported in four (10%) patients, requiring surgical repair in two. There were no reports of postoperative meningitis. No neurological complications were observed except of a reported worsening of vision in one patient. CONCLUSION: Midline anterior skull base meningiomas infrequently extend significantly into the PNS and NC. Despite their significant involvement, along with concomitant involvement of orbit, gross total resection is possible in the majority of cases with low morbidity using either purely transcranial or combined endoscopic/transcranial approach.

Intracranial nasofrontal dermoid cyst and a hair in the nose tip without a sinus tract identified with single-staged combined approach excision done.

This is the first reported case of intracranial nasofrontal dermoid without sinus tract, with complete excision done in single-staged combined approach frontal craniotomy and open rhinoplasty, and satisfactory nasal reconstruction.

Analysis of Imperative Facial Angles for Rhinoplasty: An Endowment to the Surgeon.

To study the nasal framework of patients in relation to CT scan and observe the Nasofrontal angle and Nasofacial angle in patients. This study is a prospective observational study. It was done from January 2020 to December 2020. It involved selection of patients with nasal complaints and external nasal deformity followed by their CT scan evaluation. It included a 3D analysis of the CT scan using a computer software. Preoperative photographic evaluation of the patient was done in frontal, lateral and basal view for a better understanding and correlation. Thirty patients were evaluated and their 3D reconstructed CT scans were observed. The mean Nasofrontal angle was 116.69°. The mean was 110.43° in males and 126.08° in females. The mean Nasofacial Angle was 37.68. The mean was 43.05° in males and 29.62° in females. There was a significant statistical difference seen. The demand for rhinoplasty in India is on rise. The studies done in the past have evaluated patients using plain 2D CT films. In this study, 3D reconstruction of CT scan has been done. There are meagre studies which describe the aesthetics of Indian population. It aids in preoperative evaluation and plan of surgery, enables a good doctor-patient communication. Also, it can be used for clinical learning of resident doctors.

Managing an open nasofrontal encephalocele after birth.

Encephaloceles are relatively uncommon in western countries. Most of the reported cases involve occipital encephaloceles. Open frontal encephaloceles comprise a rare entity. Most of them will be detected during early prenatal diagnostic, whereas the majority of the pregnancies will be terminated after the consent of the parents. Open frontal encephaloceles pose a great challenge to neurosurgeons as well as anesthesiologists, as these infants present with a microcephaly, non-physiological intracranial anatomy, and low birth weight, thus making the infant prone to excessive blood loss, hypothermia, and death. Neonates born with an incomplete cutaneous coverage are exposed to an imminent threat to life due to the risk of meningitis, necessitating surgical repair in the first days of life. We represent a rare case of an open nasofrontal encephalocele managed surgically in the first day of life. Surgery did not influence the neurological outcome of the patient.

Management of an open nasofrontal encephalocele during the first day of life.

Nasofrontal encephaloceles are extremely rare craniofacial defects that present with herniation of cerebral tissue through the junction of the frontal and nasal bones. They often have a cutaneous covering which allows management to be delayed until early infancy, decreasing the risks of anesthesia and acute blood loss. Further bone development also facilitates cranial remodeling. Encephaloceles with a compromised cutaneous envelope pose an imminent threat to life due to the risk of meningitis, necessitating surgical repair in the first days of life. This report presents a patient born with an open nasofrontal encephalocele that underwent encephalocele excision, dural repair, and cranial remodeling on the first day of life. Anterior encephaloceles represent a spectrum of uncommon craniofacial dysraphias. Given the rarity of these diseases, it is important to understand their many presentations and the treatment options that exist for each. When the cutaneous covering of the encephalocele is compromised, surgical correction must be undertaken urgently to mitigate the mortality associated with neonatal meningitis. This represents the first report of an open nasofrontal encephalocele managed in the first day of life. A multidisciplinary surgical approach involving the neurosurgical and craniofacial surgical teams is paramount to the treatment and survival of such complicated patients.

Possible Skull Base Erosion After Prolonged Frontal Sinus Stenting.

Frontal sinus stenting is widely used with the goal of maintaining nasofrontal duct patency after sinus surgery. The general recommendation is to leave stents in place for 6 months; however, prolonged stenting up to 6 years has been reported with no complication. We present the first reported case of frontal sinus posterior table and skull base erosion following prolonged frontal sinus stenting. A 57-year-old female presented with chronic sinusitis and nasal obstruction. Imaging revealed pansinusitis with retained stents in each frontal sinus that were placed 8 years prior. On the right, there was an area of skull base erosion at the tip of the stent. The patient underwent functional endoscopic sinus surgery with polypectomy. The stents were removed, revealing posterior table erosion on the right side but intact mucosa. Two months after surgery, there were no signs or symptoms of cerebrospinal fluid leak or other complications. Recent literature has suggested that prolonged stenting is safe; however, this case highlights a complication with potentially serious outcomes that can result from prolonged stenting. We recommend stent removal once stable nasofrontal duct patency has been achieved. If prolonged stenting is utilized, patients should be closely monitored and consideration should be given to periodic imaging to evaluate stent position.

Quiste dermoide nasofrontal: presentación de un caso y revisión narrativa / Nasofrontal dermoid cyst: case presentation

Las lesiones de la región nasofrontal en los niños son un reto diagnóstico debido a su rareza, y su potencial comunicación con el sistema nervioso central también aumenta su complicaciones. Dentro de las principales entidades de esta región se encuentran los quistes dermoides, los gliomas nasales y los encefaloceles. Un abordaje diagnóstico y terapéutico inapropiado podría generar desde simples recurrencias hasta fistulas e infecciones en el sistema nervioso central, que podrían contribuir a mayores complicaciones o incluso, poner en riesgo la vida de los pacientes.

Injuries to the naso-frontal region in children are a diagnostic challenge, associated with their rarity, their complexity also implies their potential communication with the central nervous system. Dermoid cysts, nasal gliomas, and encephaloceles are among the main entities in this region. An inappropriate diagnostic and therapeutic approach could generate from simple recurrences (as in our case), to fistulas and infections of the central nervous system that could contribute to greater complications or even put the lives of patients at risk.

Combined minimally invasive surgical management of a nasal dermoid sinus cyst affecting the frontal sinus: literature review and new classification.

INTRODUCTION: Nasal dermoid sinus cysts (NDSCs) are the most common lesions associated with midline craniofacial anomalies, in the majority of cases diagnosed during childhood. NDSCs affecting the frontal sinus are rare. AIM: To demonstrate the clinical, radiological and diagnostic pitfalls of NDSCs affecting the frontal sinus. MATERIAL AND METHODS: A retrospective analysis of NDSCs affecting the frontal sinus with a literature review and a novel classification is presented. RESULTS: We present a rare and complex case of an NDSC in an adult patient that affected the frontal sinus. Endoscopic-assisted open rhinoplasty with endoscopic sinus surgery - Draf type 2B approach - was performed as an effective removal method of choice. A literature review supports our report. CONCLUSIONS: NDSCs affecting the frontal sinus can mimic complications of sinusitis. A minimally invasive combined technique of endoscopic-assisted open rhinoplasty with endoscopic sinus surgery - Draf type 2B frontal sinus approach - is recommended for treatment.

Nasofrontal dermoid cyst: rare presentation of a philtrum sinus.

Congenital midline nasal lesions are extremely rare, and nasal dermoids are their most common presentation. To the best of our knowledge, only two cases of a philtrum sinus with skull base extension have been reported previously. A 3-year-old boy presented to the maxillofacial department with a discharging upper lip sinus that had been present from birth. No other congenital abnormalities were reported. Initially this sinus was assumed to be blind-ended and excised under general anaesthetic, but the area then failed to heal. Radiological work-up showed a patent dermal sinus that extended from the infranasal region through the nasal septum into the basal aspect of the anterior cranial fossa. The patient was referred for multidisciplinary management. A high index of suspicion of nasofrontal dermoid should be exercised when a patient presents with an upper lip sinus and recurrent discharge, until it is proven otherwise.

[Modern aspects of surgical treatment of nasal liquorrhea with localization of defect in frontal sinus]. / Sovremennye aspekty khirurgicheskogo lecheniia nazal'noi likvorei s lokalizatsiei defekta v lobnoi pazukhe.

Defects localized in the frontal sinus are difficult for surgical treatment, since there is a large number of anatomical variations in the structure of the naso-frontal canal and the sinus itself. With the development of endoscopic technology and paying attention to the modern tendency of minimal invasive surgery, new invasive approaches to reach frontal sinus, such as endoscopic approach according to Draf I-III and combined approaches were developed and put into practice. OBJECTIVE: To summarize and to analyze the results of treatment of nasal liquorrhea with localization of the defect in the frontal sinus using endoscopic endonasal and combined intra-extranasal approaches. MATERIAL AND METHODS: A retrospective analysis of a series of 43 cases of nasal liquorrhea with a frontal sinus defect being treated at the NMRCN Burdenko during the period from 2001 to 2017. To select access, a classification of frontal sinus defects according to their localization was developed. The analysis of demographic and clinical data of patients, as well as intraoperative and postoperative data analysis were done. RESULTS: In the series of 43 patients, endoscopic endonasal approach was performed in 28 (65%) cases. Combined approach was performed at 15 (35%) patients. The success rate of plastic surgery with endoscopic endonasal approach was 86% (24 of 28), with combined approach - 93% (14 of 15). CONCLUSION: Endoscopic endonasal and combined approaches are the methods of choice for plastic surgery of defects of the skull base in the frontal sinus as they are effective and safe. The choice of approach depends on anatomical features of the frontal sinus and on the localization of defect.

Nasofrontal surgical reconstruction by external table flap of frontal bone following removal of a dermoid cyst revealed by a fistula: A case report and review of the literature.

Nasofrontal fistulas correspond to the persistence of an abnormal communication of embryological origin between the deep layer of the skin and the central nervous system (CNS). They can rarely be associated with a dermoid cyst and be revealed by a locoregional infection, and especially neuromeningeal infections can be serious. The treatment is mainly surgical by performing a total excision of the cyst and the repair of defects. The authors report the case of an 18-month-old infant operated for a dermoid cyst revealed by a nasofrontal fistula. They insist on the characteristics of this pathology in order to establish a diagnosis and an early treatment to avoid the complications that can be heavy in certain cases. They describe the steps of nasofrontal reconstruction by a small flap taken from the outer table of the frontal bone with better esthetic results.

Frontal Bone Fractures and Frontal Sinus Injuries: Treatment Paradigms.

BACKGROUND: Timely, expeditious and appropriate management of Frontal bone fractures and associated Frontal Sinus (FS) injuries are both crucial as well as challenging. Treatment options vary considerably, depending upon the nature, extent and severity of these injuries as well as operator skill, expertise and experience. In cases of posterior table fractures of the Frontal Sinus, literature reports have in general, propounded direct visualization and exploration of the sinus via a bifrontal craniotomy, followed by sinus cranialization. AIMS AND OBJECTIVES: To review the standard protocols of management of Frontal bone fractures and Frontal Sinus injuries. To assess the efficacy of a more conservative approach in the management of outer and inner table fractures of the FS. MATERIALS AND METHODS: Contemporary and evolving management protocols and changing treatment paradigms of different types and severities of frontal bone fractures and frontal sinus injuries, have been presented in this case series. A useful Treatment Algorithm has been proposed to efficiently and effectively manage these injuries. RESULTS: In the present case series, effective and satisfactory results could be achieved in cases of significantly displaced inner and outer table fractures of the Frontal sinus by a more conservative protocol comprising of open reduction and internal fixation carried out via the existing scar of injury, without having to resort to the more radical intracranial approach and sinus cranialization. Nevertheless, presence of complicating factors such as cerebrospinal fluid rhinorrhea, evidence of meningitis or the development of encephalomeningocoeles necessitated the standard protocol of sinus exploration and its cranialization or obliteration. CONCLUSION: Management protocols of Frontal Sinus injuries vary, based on aspects such as the timing of presentation and intervention, degree of injury sustained, concomitant associated Craniomaxillofacial injuries present, presence of complicating factors or Secondary/Residual deformities & Functional debility, and need to be decided upon on a case to case basis.

A scanographic study of asymmetry of the frontal process of zygoma in unilateral coronal synostosis.

INTRODUCTION: Unilateral coronal synostosis (UCS) is a complex craniosynostosis, combining malformations of the upper (frontal bone, orbito-naso-frontal bandeau (ONFB) ) and the middle thirds of the face. In our centre, the surgical correction consists in the repositioning of the ONFB in front of the zygomaticofrontal suture on the affected side. Defects in the corrections have been observed post-operatively for some patients with a persistent asymmetry in the side walls of the orbits. The purposes of our study were to perform an analysis of the frontal process of zygoma (FPZ) in children affected by UCS using preoperative CT-scans and to consider the modification of surgical techniques in order to achieve a better ONFB repositioning and thus, better symmetry. MATERIAL AND METHOD: The preoperative CT-scans of 13 children with UCS who underwent corrective surgery in our department from 2005 to 2016, were analyzed. After the selection of 6 morphological points in 2 and 3 dimensions using planning software, the sutures constituting the coronal arch and the sagittal distances between the coronal plane and the zygomaticofrontal sutures were analyzed. We compared the measurements on the pathological side to the healthy side, the non-affected side being the reference side. The patients included were those for whom the lack of symmetry between the healthy side and the affected side was more than 2 mm. Statistical analyses were carried out using a Student t-test. RESULTS: Of the 13 children, 10 (1 day to 42 months old) met the inclusion criteria. The mean sagittal distance between the coronal plane and the healthy zygomaticofrontal suture was 33.8 mm ± 5.43 mm [range: 25.9: 40.9] [median: 34.3]. On the pathological side, the distance was 28.75 mm ± 4.76 mm [range: 20.8: 36.3] [median: 29.55], (p = 0.04). DISCUSSION: There is a significant asymmetry between the FPZ on the healthy and the pathological sides in children affected by UCS. This asymmetry is variable, therefore justifying a personalized surgical correction whichtakes into account not only the shape of the ONFB but also the degree of asymmetry of the FPZ. A prospective study with immediate preoperative CBCT image acquisition and long-term clinical and radiological follow-up, will be our next step.

Clinical delineation of a subtype of frontonasal dysplasia with creased nasal ridge and upper limb anomalies: Report of six unrelated patients.

Frontonasal dysplasias are rare congenital malformations of frontonasal process-derived structures, characterized by median cleft, nasal anomalies, widely spaced eyes, and cranium bifidum occultum. Several entities of syndromic frontonasal dysplasia have been described, among which, to date, only a few have identified molecular bases. We clinically ascertained a cohort of 124 individuals referred for frontonasal dysplasia. We identified six individuals with a similar phenotype, including one discordant monozygous twin. Facial features were remarkable by nasal deformity with creased ridge and depressed or absent tip, widely spaced eyes, almond-shaped palpebral fissures, and downturned corners of the mouth. All had apparently normal psychomotor development. In addition, upper limb anomalies, frontonasal encephalocele, corpus callosum agenesis, choanal atresia, and congenital heart defect were observed. We identified five reports in the literature of patients presenting with the same phenotype. Exome sequencing was performed on DNA extracted from blood of two individuals, no candidate gene was identified. In conclusion, we report six novel simplex individuals presenting with a specific frontonasal dysplasia entity associating recognizable facial features, limb and visceral malformations, and apparently normal development. The identification of discordant monozygotic twins supports the hypothesis of a mosaic disorder. Although previous patients have been reported, this is the first series, allowing delineation of a clinical subtype of frontonasal dysplasia, paving the way toward the identification of its molecular etiology.

Frontal Sinus Fractures.

The frontal bone serves a protective role, representing the transition between the facial skeleton and the cranium. Within its enclosure, the frontal sinus lies adjacent to many important intracranial structures, and injury to this region has severe complications if not properly managed. Historically, the goals of frontal sinus fracture management have been to best prevent intracranial complications with invasive procedures, but a recent shift favoring conservative management now aims to preserve the form and function of the frontal sinus and its outflow tract. With the advancement of endoscopic techniques and the development of new technologies, many alternatives to aggressive surgical management are available.

Brain Abscess and Unusual Nasal Pimple in a Child.

We report a child with misdiagnosed nasal dermal sinus and frontonasal dermoid cyst, that was complicated by brain abscess. Familiarizing physicians with this peculiar clinical picture may allow prompt recognition of this malformation, with subsequent imaging study, and surgical treatment, aimed to preventing fatal infectious complications.

Nasofrontal dermoid with sinus tract upto the tip of the nose: A case report with review of the literature.

Nasofrontal dermoid with sinus tract extending to the nasal tip is rare in adults. It is unique in its embryological origin, presentation, and management. Pathogenesis involves the incomplete obliteration of the developing neuroectoderm. A sinus opening with intermittent oily discharge is characteristic. Associated intracranial extension may lead to meningitis and brain abscess. We present a case of a 21-year-old adult having a nasofrontal sinus tract with intracranial extension who presented with recurrent episodes of meningitis and discuss the physical findings with relevance to embryology, elaborating on the importance and means of addressing the intracranial as well as extracranial components for complete treatment.