Internasal: A New Type of Frontoethmoidal Encephalomeningocele.

IMPORTANCE: Frontoethmoidal encephalomeningocele (FEEM) is a congenital anomaly with a skull base defect at the foramen caecum and a protrusion of meninges and brain tissue through an external defect on the face. The external defect was classified to nasofrontal, nasoethmoidal, naso-orbital, combined, and abortive type. OBJECTIVE: The aim of our study was to present a newly discovered internasal type (IN) of FEEM. DESIGN, SETTING, PARTICIPANTS: Ninety-six FEEM patients' preoperative 3-dimensional computer tomography scans were studied in the Department of Surgery, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand followed by treatment from 2006 to 2016. FINDINGS: Sixteen patients had an external bony defect between the nasal bone that could not be classified in any previous classification. We suggest a new type of FEEM called "IN." The bony defect of this IN can be located on the upper nasal bone (4 cases), middle nasal bone (5 cases), or combined with naso-orbital type (7 cases) counted in the combined type. CONCLUSIONS AND RELEVANCE: Our study presents a newly discovered IN of FEEM. This IN can cause hypertelorism when the nasofrontal type could not. Preoperative 3-dimensional computer tomography scans are very helpful for surgical planning.

The Onodi Cell: An Anatomic Illustration.

This anatomic image demonstrates the sphenoethmoidal (Onodi) cell (a variant of the paranasal sinuses), the identification of which is critical to prevent neurovascular injury during endoscopic approaches to the sella and adjacent regions of the skull base.

[The correlation between the types of the uncinate process superiorattachment and chronic frontal sinusitis without nasal polyps].

Objective:To investigate the correlation between the types of unicate process (UP) superior attachment and the chronic frontal sinusitis without nasal polyps(CFSsNP).Method:The images of 240 sides of frontal recess were evaluated. The types of UP superior attachment was compared between the CFSsNP group and the control group. The correlation between UP the superior attachment and CFSsNP were analyzed. Result:Seven types of UP superior attachment were identified. The most common type in the two study groups was type 1(insertion into the lamina papyracea). The occurrence rate of type 1 of UP superior attachment in the CFSsNP group was significantly higher than that in the control group(59.8% and 36.4%,respectively P<0.01),but type 5(insertion into both the lamina papyracea and the skull base) was significantly less frequent in the CFSsNP group than in the control group(9.8%，28.8%,P<0.01). Logistic regression analysis revealed that the type 1 of UP superior attachment was an independent risk factor for CFSsNP(OR=1.181,P<0.05),and that type 5 of UP superior attachment was a protective factor for CFSsNP(OR=0.391,P<0.05). Conclusion:UP superior attachment varied significantly,with the most common type being type 1; This type is intimately related to CFSsNP.

[Individual nasal endoscopic surgery for non-sinusitis-related rhinogenous headache: our experience in 68 cases].

OBJECTIVE: To investigate the role of anatomical abnormalities in non-sinusitis-related rhinogenous headache and to evaluate effects of nasal endoscopic surgery for non-sinusitis-related rhinogenous headache. METHOD: Sixty-eight patients diagnosed as non-sinusitis-related rhinogenous headache were selected in this study. They were treated with nasal endoscopic surgery after failed long-term medical treatment. Data from this group were analyzed retrospectively. RESULT: Multiple anatomical abnormalities were noted by endoscopy and sinus computed tomographic scans in the 66 patients. These included nasal septum deviation in 46 cases (67.6%), middle turbinate gasfication in 20 cases (29.4%), protruding ethmoid bulla or uncinate processor in 10 cases (14.7%) and abnormal middle turbinate in 8 case (11.8%). Fifty-six (82.4%) patients showed significant improvement after surgery. CONCLUSION: Non-sinusitis-related rhinogenous headache can be significantly minimized with individual nasal endoscopic surgery, as long as a precise identification of the etiologic anatomical factor can be made.

Frank-ter Haar syndrome--additional findings?

Frank-ter Haar syndrome is a genetic disease that is transmitted by autosomal recessive pattern with characteristic features such as megalocornea or glaucoma, a prominent coccyx, heart defects, developmental delays, brachycephaly, a wide anterior fontanel, finger flexion deformities, full cheeks and micrognathia. Dentomaxillofacial features of this syndrome are not well documented in the literature. We present of a 21-year-old male with Frank-ter Haar syndrome and some features that may be linked with this syndrome not reported before in the literature.

The role of the uncinate process in sinusitis aetiology: isolated agenesis versus maxillary sinus hypoplasia.

OBJECTIVE: This study aimed to evaluate the clinical significance of maxillary sinus hypoplasia and isolated agenesis of the uncinate process in sinusitis aetiology. METHODS: Three patients with isolated agenesis of the uncinate process and 27 patients with 43 maxillary sinus hypoplasia variations were recruited. The frequencies of sinusitis episodes and radiological findings were compared between patient subgroups. RESULTS: In all, 23 type I maxillary sinus hypoplasia, 13 type II maxillary sinus hypoplasia and 7 type III maxillary sinus hypoplasia variations were detected. Patients with isolated agenesis of the uncinate process underwent antibiotic treatment an average of 7 times per year, whereas those with types I, II and III maxillary sinus hypoplasia were treated 1.57, 3.22, and 5.75 times per year, respectively, over a 5-year period. The antibiotic treatment frequency for patients with isolated agenesis of the uncinate process was significantly higher than for those with types I and II maxillary sinus hypoplasia. CONCLUSION: Isolated agenesis of the uncinate process seems to play a stronger role than types I and II maxillary sinus hypoplasia in the pathophysiology of chronic sinusitis.

Combined aplasia of sphenoid, frontal, and maxillary sinuses accompanied by ethmoid sinus hypoplasia.

OBJECTIVE: To report the case of a woman who had combined aplasia of sphenoid, frontal, and maxillary sinuses accompanied by ethmoid sinus hypoplasia. METHODS AND RESULTS: A 47-year-old woman presented with complaints of headache, nasal obstruction, and postnasal drainage. She had no previous history of either facial trauma or systemic diseases affecting the skeletal system. She had a history of functional endoscopic sinus surgery twice in an other institution. However, the surgeries did not significantly alter her symptoms. Coronal and axial computed tomographic scans of the nose and the paranasal sinuses showed poorly developed frontal, sphenoid sinuses, ethmoid cells, and very severe and profound hypoplasia (aplasia) of bilateral maxillary sinuses. CONCLUSIONS: To our knowledge, this patient seems to be the first case having combined aplasias of the sphenoid, frontal, and maxillary sinuses with hypoplastic ethmoid cells without any systemic or skeletal disease.

Bilateral ethmoidal dural arteriovenous fistula: unexpected surgical diagnosis.

Dural arteriovenous fistulae (DAVFs) are infrequent lesions, the most common locations of which are the cavernous, sigmoid and transverse sinuses. The cribiform plate is one of the less frequent sites for DAVFs, where they entail a high hemorrhage risk. Feeding arteries for ethmoidal DAVFs can be uni- or bilateral. However, the draining fistulous system has classically been described as unilateral. The authors report the second case in literature of bilateral ethmoidal DAVF, which is defined as that with bilateral draining veins. The present case was diagnosed only after surgical exploration of both cribiform plates. No preoperative radiological test could detect the presence of a bilateral venous draining system from the ethmoidal DAVF. Possible reasons for that lack of presurgical diagnosis are discussed. Bilateral surgical exploration of the anterior cranial fossa is recommended when dealing with ethmoidal DAVFs, even when they seem to be unilateral on preoperative studies.

Transethmoidal meningoencephalocele involving the olfactory bulb with enlarged foramina of the lamina cribrosa--case report.

A 3-year-old girl presented with a transethmoidal meningoencephalocele manifesting as recurrent rhinorrhea. Initially, she developed meningitis, but after treatment she experienced rhinorrhea. Two months later, she again presented with rhinorrhea. Neuroimaging studies revealed a small protrusion (15 mm x 10 mm) at the roof of the ethmoidal sinus. Nasal endoscopy confirmed the diagnosis of meningoencephalocele. The operative findings revealed a small hole in the left olfactory bulb, which had descended into an enlarged foramen along with the arachnoid membrane. The left olfactory bulb was removed, and the enlarged foramina of the lamina cribrosa were covered with a frontal pericranial flap. The defect in the bone was very small, but contributed to the development of meningitis and leakage of the cerebrospinal fluid. Basal cephalocele should be considered in a patient with recurrent rhinorrhea and intracranial infections, even in the absence of any apparent anomaly.

Medialization of the lamina papyracea after endoscopic ethmoidectomy: comparison of preprocedure and postprocedure computed tomographic scans.

OBJECTIVE: The purpose of this article is to report computed tomography demonstration of medial bowing of the lamina papyracea in 5 patients after internal ethmoidectomy. METHODS: We identified 5 patients who had apparent medial bowing of the lamina papyracea after functional endoscopic sinus surgery (FESS) and who had preoperative scans available. Preoperative and postoperative scans were reviewed using a 3-dimensional workstation to ensure similar angulation of the slices before measurement. Measurements of the interorbital distance and also the position of the posterior margin of the globe relative to a line connecting the lateral orbital walls were performed. RESULTS: Each of the patients demonstrated a decrease in the interorbital distance on the postoperative scan. Measurement of globe position showed that 9 of the 10 globes lay in a more posterior position within the orbit on postoperative examination. CONCLUSIONS: Medial bowing of the lamina papyracea may occur as a result of FESS and may lead to relative enophthalmos in comparison. The incidence of this phenomenon is unknown because most patients are not reimaged after FESS.

Recurrent unilateral periorbital cellulitis in a pediatric patient--an anatomic abnormality.

A 2-year-old male, otherwise healthy, suffered a total of 7 episodes of recurrent right-sided periorbital celluitis (POC) which began at 11 months of age. Five of the 7 episodes of right eye swelling/erythema required hospital admission for intravenous antibiotics. Imaging studies demonstrated a well-defined dehiscence in the lamina papyracea. Endoscopic sinus surgery was performed and an abnormal uncinate process was visualized, fused to the lateral nasal wall and ethmoid bulla. Post-operatively, the patient had no further infections. Nasal endoscopy and high-resolution imaging in pediatric patients with recurrent POC could identify those who would benefit from early surgical intervention.

[Management of patients with recurrent purulent sinusitis caused by structural anomaly of the unciform process].

We have examined and treated 14 patients suffering from recurrent bacterial sinusitis (RBS) in abnormal unciform process (UP). The duration of the disease was 1 to 3 years, mean 1.9 years. The patients had UP hypoplasia (n=7), UP perforation (n=4), defects due to endonasal surgical intervention (n=3). We made surgical remodeling of the UP by two original techniques. After follow-up for 1-1.5 years the results of the treatment in patients with recurrent bacterial maxillary ethmoiditis were satisfactory in 12 (85.7%) patients and unsatisfactory in 2 (14.3%) patients.

Variations of maxillary sinus and accompanying anatomical and pathological structures.

Hypoplasia and aplasia of maxillary sinus, maxillary sinus septae, ethmomaxillary sinus, superior meatus-draining maxillary sinus, and over-pneumatization are the variations of maxillary sinuses. Findings such as uncinate process abnormality, orbital enlargement, sphenomaxillary plate, canine fossa elevation, infraorbital fissure enlargement, thickening of the sinus wall and mucosal pathologies can be seen together with these variations. The aim of this study is to determine the incidence and morphology of the anatomical variations of maxillary sinus and accompanying structures. A total of 330 consecutive paranasal sinus computed tomography scans of the patients presenting with sinonasal complaints at our ENT department were assessed for maxillary sinus anatomical variations and related structures. There were 134 (20.3%) anteriorly and 17 (2.5%) posteriorly localized maxillary sinus bony septa. The position of antral septa was frequently vertical at anterior, and horizontal at posterior. We found significant correlation between the anteriorly localized maxillary sinus septa and infraorbital fissure enlargement. Maxillary sinus hypoplasia was found in 31 (4.6%) maxillary sinuses. Seven (1.0%) ethmomaxillary sinuses were assessed. Significant correlation was found between maxillary sinus hypoplasia and orbital enlargement. Presence of mucosal pathology was significant in patients with maxillary sinus septa, but there was no difference when it was compared with the mucosal pathology in the nonseptated group. There was no significant correlation between other anatomical variations and mucosal pathologies. Determining the anatomical variations of maxillary sinus and accompanying structures in patients planning endoscopic sinus surgery will significantly help with providing surgical orientation and preventing possible complications.

Atypical synophthalmos with arhinencephaly unilateralis.

This article describes a rare case of a 4-month-old infant with atypical synophthalmos with ipsilateral arhinencephaly unilateralis, ethmoid sinus, and lacrimal apparatus and brain abnormalities--diagnosed on the basis of the clinical picture and imaging findings.

Bilateral maxillary sinus hypoplasia and aplasia: radiological and clinical findings.

Maxillary sinus hypoplasia (MSH) is classified into three types depending upon embryological development of the sinus and uncinate process. Type III MSH is characterized by a near-absence of the uncinate process and an almost absent cleft-like sinus. Bilateral maxillary sinus aplasia or severe hypoplasia with associated paranasal sinus abnormalities is extremely rare. Two cases with severe maxillary sinus hypoplasia/aplasia (one of them with other associated paranasal sinus abnormalities) are presented in this article. This extremely rare abnormality should be kept in mind to prevent misdiagnosis and possible complications during endoscopic sinus surgery.

Aicardi syndrome associated with anterior cephalocele in a female infant.

Aicardi syndrome is a triad of abnormalities that includes total or partial agenesis of the corpus callosum, chorioretinal lacunae, and infantile spasms. This syndrome was first described in 1965. A female infant with Aicardi syndrome associated with a nasoethmoidal cephalocele is described in this report. She presented with a history of unilateral nasal discharge since birth and seizures since age 1 week. She was microcephalic and there was visual impairment. A fleshy mass of the left nostril was noted. Ophthalmological evaluation revealed left exotropia, dysplastic optic discs and retina, 'morning glory' appearance of the left optic disc, and bilateral chorioretinal lacunae. Magnetic resonance imaging of the brain showed absence of the corpus callosum, dysmorphic changes of the lateral ventricles, a superiorly located third ventricle, heterotopic grey matter of the frontal lobes, a left nasoethmoidal cephalocele, and closed lip schizencephaly of the left frontal lobe. This female infant developed asymmetric infantile spasms at age 8 weeks. Surgical correction of the cephalocele was declined. She developed recurrent pneumonias secondary to aspiration of feeds and died at age 8 months during one of these events.