The reconstruction of skull base defects in infants using pedicled nasoseptal flap-a review of four cases.

INTRODUCTION: Benign lesions of the skull base are common in the paediatric population, and are usually congenital in aetiology. Majority of these lesions are treated transcranially exposing the patients to a number of risks. Although endoscopic endonasal surgery (EES) helps avoid many of these potential morbidities, CSF leak with its attendant complication remains a big concern. This study reports the use of the Hadad flap in the reconstruction of skull base defects in infants to prevent this problem. The study was conducted on four infants with a mean age of 7 months, who underwent repairs for CSF leaks associated with congenital lesions like meningocele or meningoencephalocele, using the Hadad flap. Of the four patients, three cases were revision cases and one was primary where the patients presented with complaints such as nasal obstruction and watery nasal discharge. Post surgery, the infants were monitored for a mean period of 23 months and no major complications or recurrent CSF drainage were observed. Minor complications that were observed include vestibulitis and crusting in the nose. The utility of the Hadad flap in the reconstruction of skull base defects in the paediatric age group has been controversial while its utility in infants has not been studied in literature so far. We report here in our series four infant patients in whom we believe that the nasal septum and the skull base will develop proportionally to each other, hence lowering the chances of a short flap and eliminating the occurrence of future complications. CONCLUSION: The nasoseptal flap is an effective and safe technique for reconstructing skull base defects in infancy. It can be concluded that this technique does not have any potential effect on septal or craniofacial growth as the flap is harvested only on one side with normal mucosal cover on the other side. There is no posterior septectomy or any form of bony or cartilaginous resection that is performed, hence avoiding any effects on bony growth. No studies have been published in literature so far and to the best of our knowledge, this is the first report describing the efficacy of the nasoseptal flap in infants.

Value of systematic analysis of the olfactory cleft in case of cerebrospinal rhinorrhea: incidence of olfactory arachnoid dilatation.

To report on the presence of olfactory arachnoid dilatations (OAD), a previously undescribed radiologic feature of spontaneous cerebrospinal fluid (CSF) rhinorrhea originating from the cribriform plate of the ethmoid bone. The medical records of all patients treated between 2001 and 2011 at a tertiary care center for a spontaneous CSF rhinorrhea originating from the cribriform plate were retrospectively reviewed. The radiological work-up included high-resolution computed tomography and magnetic resonance imaging with at least the following sequences: T1, T2, and T2 with fast imaging employing steady state acquisition (FIESTA). Thirty cases were identified. The mean age at diagnosis was 49. Fourteen patients (47 %) had a body mass index (BMI) of 30 or more and 3 patients (10 %) had a BMI between 25 and 29.9. Five patients had a history of meningitis. The imaging work-up revealed a bone defect of the cribriform plate in 6 cases (20 %), associated to a typical meningocele in 14 cases (47 %). In ten patients (33 %), there was no defect of the cribriform plate, but ultrathin coronal T2-FIESTA sequences revealed an OAD, i.e. a dilatation of the arachnoid sheath of the olfactory fibers, in nine cases (30 %), or a "pseudo-polyp" outlined by a thin layer of arachnoid (1 patient, 3 %). Preoperative imaging should be carefully analyzed for the presence of OAD or "pseudo-polyp" in patients presenting with a CSF rhinorrhea without bony defect of the cribriform plate.

Characteristics of Dehiscence of Lamina Papyracea Found on Computed Tomography Before Orbital and Endoscopic Endonasal Surgeries.

The aim of this study was to identify characteristics of dehiscence of the lamina papyracea found on computed tomography (CT) before orbital and endonasal endoscopic surgeries.The authors retrospectively reviewed the medical records of all patients who underwent orbital tumor removal, orbital decompression, and dacryocystorhinostomy from January 2012 to December 2015. The diagnosis of dehiscence of the lamina papyracea was made based on a bone defect with protrusion of orbital fat into the ethmoid sinus on CT, with no history of orbital trauma and/or eye movement disturbance on the same side.Overall, 6 patients (1.4%) were identified from among 315 patients (90 orbital tumors, 150 orbital decompressions, and 75 endoscopic endonasal dacryocystorhinostomies). All patients were asymptomatic. All dehiscence was limited to the anterior ethmoid sinus, with fat prolapse of <1 cm. An opacified ethmoid sinus was found in 1 of the 6 patients (17%) with dehiscence of the lamina papyracea. Operative area included the dehiscence site in 1 patient. Intraoperatively, the periorbita was found to be defected at the dehiscence site with intact connective tissue septa. The dehiscence site served as a landmark for the operation.Dehiscence of the lamina papyracea is a rare anomaly, but occasionally encountered in orbital and endoscopic endonasal surgeries.

Is there a relationship between cribriform plate dimensions and septal deviation angle?

The aim of this retrospective study was to investigate the relationship between cribriform plate (CP) dimensions and septal deviation degree. Coronal paranasal CT scans of 99 patients were reviewed. We measured depth and width of cribriform plate on both sides and compared with septal deviation side and septal deviation degree. Deviation angles were 6.85 ± 1.47° for right deviations; and 7.11 ± 1.63° for the left deviations. The mean depth of CP was 5.08 ± 1.57 mm at the right side and 5.06 ± 1.59 mm at the left side; and the mean width of CP was found 4.71 ± 1.36 mm at the right side and 4.56 ± 1.51 mm at the left side. When CP dimensions were evaluated according to the septal deviation side, mean width of CP was 4.69 ± 1.36 mm at ipsilateral side (deviated side); and 4.58 ± 1.51 mm at the contralateral side. The mean depth of CP was 4.9 ± 1.56 mm at the ipsilateral side (deviated side); and 5.22 ± 1.58 mm at the contralateral side. The CP depth at the contralateral side was significantly higher than that of the ipsilateral side (deviated side). In right SD, ipsilateral and contralateral CP depth increased. As deviation angle increased, ipsilateral and contralateral CP width, right and left CP width increased. Ipsilateral and contralateral CP width; and additionally ipsilateral and contralateral CP depth increased together. In other words, right and left CP width; and CP depth increased simultaneously. It is well known that the higher incidence of intracranial penetration is on the side where the position of the ethmoid roof (ER) is lower. The presence of septal deviation, the possibility of the deeper CP at the contralateral side should be taken into consideration to avoid iatrogenic injury.

Ethmoidal encephalocele associated with cerebrospinal fluid fistula: indications and results of mini-invasive transnasal approach.

Anterior skull base defects with encephalocele in adults are quite rare and can be a cause of spontaneous rhinoliquorrhea; however, cerebrospinal fluid (CSF) fistula can be not rarely misdiagnosed for several months or years. Five adult patients affected by ethmoidal encephalocele with CSF fistula were treated in our institute from 2006 through to 2011. Onset of clinical history was represented by rhinoliquorrhea, which was precociously recognized in only 1 patient; in the other 4, it was misdiagnosed for a period ranging from 11 months to 5 years. After clinical diagnosis of CSF fistula and after brain magnetic resonance imaging, ethmoidal encephalocele was evident in all patients; preoperative study was completed by spiral computed tomography scan, to clearly identify the skull base bone defect. All patients were operated on by transsphenoidal endonasal endoscope-assisted microsurgical approach through 1 nostril. The herniated brain was coagulated and removed, and reconstruction of cranial base was performed. Postoperative rhinoliquorrhea or other complications did not occur in any patient at short and late follow-up. All patients were discharged after a few days. Endonasal endoscope-assisted microsurgical approach was effective in exposing and repairing the ethmoidal bone defect; tridimensional vision and wide lateral and superior exposition of the operative field were possible in each patient, thanks to the use of microscope and angulated endoscope.

Case report: intracranial placement of a nasotracheal tube in a patient with Goldenhar syndrome associated with cribriform plate agenesis.

We describe a case of inadvertent intracranial placement of a nasotracheal tube in a patient with an undiagnosed major congenital cranial anomaly (a variant of Goldenhar syndrome, which included absence of the cribriform plate). We believe that this is the first reported case in which this complication arose as a result of a congenital abnormality rather than traumatic or iatrogenic disruption of the skull base. We conclude that patients with known craniofacial abnormalities or associated syndromes scheduled for procedures involving planned nasotracheal intubation or nasogastric tube placement should undergo preoperative cranial imaging studies to verify an intact skull base.

Nasal and ethmoidal alterations in anterior synostotic plagiocephaly.

BACKGROUND: Nasal deviation plays an important role in plagiocephaly nosography, and it is frequent in plagiocephaly and objectivable both in early treated patients and in those patients who did not undergo any surgical correction. PATIENTS AND METHODS: The group analyzed consisted of 12 patients affected by anterior synostotic plagiocephaly. All of them had reached the end of growth. A morphological analysis was made by using anthropometry. All patients were asked to undergo computed tomography, and all the scans were then reconstructed into three-dimensional models using a dedicated software. We made two-dimensional assessments on coronal and axial slices, and three-dimensional reconstruction was used to evaluate the volumetry of ethmoidal cells. RESULTS AND DISCUSSION: From anthropometry, we realized that, in all patients, not only that the tip of the nose is deviated to the not affected side but also that the root itself does not lie on the midline and it is tilted to the synostotic side. Analysis of the computed tomographic scan results shows that the ethmoid bone, as much as the vomer and the upper maxilla, is deeply involved in these nasal abnormalities, presenting a deviated position and an altered development. CONCLUSIONS: We concluded that nasal morphology in plagiocephaly is strictly related to basicranium dysmorphism.

Anatomical variation of the nasal septum: Correlation among septal components.

The nasal septum consists of multiple components with various developmental origins and is considered a mosaic structure. Few studies have focused on anatomical relationships among the components of the nasal septum, even though they are essential for clinical assessments of morphological abnormalities in the nasal septum. This study was performed to evaluate anatomical correlations among components of the nasal septum using computed tomography (CT) of the paranasal sinus. We studied images from CT scans of 168 paranasal sinuses collected at a secondary referral hospital between July 2008 and February 2009. The area of each component of the nasal septum was measured using median sagittal images of CT scans after three-dimensional reconstruction using computer software. The area of the cartilaginous septum decreased with age, whereas the area of the total nasal septum remained constant. The area of the perpendicular plate of ethmoid increased with age at the expense of the area of the septal cartilage.

Fronto-ethmoidal encephalocele in a historical skull with artificial deformation and no signs of chronic elevated intracranial pressure.

The intentional deformation of human skulls in the living being was one of the most curious rituals performed in historical and ancient times. It is thought that these practices cause chronic elevated intracranial pressure and subsequent symptoms of cognitive impairment. In this report, we examine such an artificially deformed skull dating from the sixteenth century that in addition shows a fronto-ethmoidal encephalocele. However, although the mild encephalocele was already manifest at birth and deformation practices were performed over years, the encephalocele did not progress into a more severe status. We conclude that the intentional deformation of skulls does not lead to chronic elevated intracranial pressure and mental retardation.

Persistent right aortic arch and cribiform plate aplasia in a northern elephant seal (Mirounga angustirostris).

A female weanling northern elephant seal (Mirounga angustirostris) presented to The Marine Mammal Center in Sausalito, California, USA, in poor body condition. An esophageal obstruction was diagnosed by contrast radiography and esophagoscopy, but despite extensive diagnostics and supportive care, the seal died 6 days later. On postmortem examination, the right aortic arch was persistent, forming a vascular ring anomaly with a patent ductus arteriosus that compressed the distal esophagus. Aplasia of the right cribiform plate and hypoplasia of the right olfactory nerve was also identified. A review of necropsy reports from January 1988 to December 2003 revealed 16 severe congenital anomalies in 454 juvenile northern elephant seals that stranded in northern California.

[Recurrent meningitis in inner ear malformations]. / Nawracajace zapalenia opon mózgowo-rdzeniowych w wadach wrodzonych ucha wewnetrznego.

Authors present two cases of children with reccurent meningitis and unilateral deafness. Implemented diagnostics (CT, NMR, ABR) revealed one side inner ear congenital malformation in one case and anterior fossa bony defect accompanied by labirynthine deformation in the other case. The presence of perilymphatic fistulae in oval and round windows and cerebrospinal fluid leakage has been confirmed in both cases during surgery. Carefull obliteration of the Eustachian tube and both windows has been performed. Non- complicated postoperative course (2 months and 6 years - respectively) has prooved the effectiveness of applied treatment.

Osteologic analysis of ethnic differences in supernumerary ethmoidal foramina: implications for endoscopic sinus and orbit surgery.

BACKGROUND: Knowledge of the position of the ethmoidal arteries is critical to enable safe endoscopic sinus and orbital surgery. The presence of a third or "middle" ethmoid variant has recently become more relevant as endoscopic intraconal surgery continues to advance. The purpose of this study was to quantify the presence of supernumerary (ie, over 2) ethmoid foramina in different ethnicities and genders. METHODS: Morphometric osteologic measurements were performed in 273 orbits. Prevalence of supernumerary ethmoid foramina and orbital length data were obtained from human skulls of Asian (n = 54), Caucasian (n = 70), African (n = 39), Hispanic (n = 49), and Middle Eastern (n = 61) derivation. Correlations between gender, ethnicity, symmetry, orbital floor, and lamina papyracea length were assessed by analysis of variance, paired t test, and χ2 test. RESULTS: Supernumerary foramina were identified in 95 of 273 orbits (34.79%). A significantly higher prevalence was seen in Asian (42.59%), African (41.02%), and Hispanic (41.00%) skulls as compared with Caucasian (25.71%) and Middle Eastern (22.95%) skulls (p < 0.05 for all). The length of the orbital floor was significantly shorter in the Asian (3.35 ± 1.52 cm) specimens (p < 0.01). Asians were found to have the highest risk of ethmoid artery injury compared with the other ethnic groups (ratio of number of supernumerary foramina to orbital floor length = 0.72). CONCLUSION: Supernumerary ethmoidal foramina were common among all orbits studied. Orbits of Asian and African derivation had significantly greater numbers of ethmoidal foramina, both unilaterally and symmetrically and within a shorter orbital length, suggesting a greater proximity between the ethmoidal vessels. Surgeons should be alert to the possible presence of middle ethmoidal vessels during endoscopic sinus and orbital approaches.

Treatment of frontoethmoidal meningoencephalocele in Cambodia: a low-cost procedure for developing countries.

OBJECT: Frontoethmoidal meningoencephaloceles (MECs) are a relatively common abnormality in southeast Asia, with disastrous consequences for the sufferer. In Cambodia, a lack of skilled neuro- and craniofacial surgeons, and the cost of surgery limit the possibilities for appropriate treatment of patients with these lesions. The authors developed a low-cost humanitarian program with the goals of treating frontoethmoidal MECs, ensuring careful postoperative follow-up, and teaching Khmer surgeons how to treat these malformations. METHODS: This program was facilitated by two nongovernmental organizations: Rose Charities Cambodia provided the facilities, patients, and local staff, and "Médecins du Monde" provided visiting surgeons and anesthesiologists. All operations were free of charge for all patients. A strict follow-up program was organized to evaluate the surgical results, the social impact of the surgery, and the satisfaction levels of the children and their parents. RESULTS: Forty-five children and seven young adults with MECs were treated using a rather simple surgical technique. Of the three types of MECs encountered, the most frequent was the nasoethmoidal type (43 cases). The most common postoperative issue was a temporary CSF leak (in 16 patients). Cosmetic results were considered excellent or good in 40 patients, average in nine, and poor in one; two patients were lost to follow-up. The overall cost of each operation was estimated to be $380 (US dollars), far less than a standard MEC operation would cost in a more developed country. At the end of this humanitarian program, Khmer surgeons were able to treat standard cases of frontoethmoidal MECs without the help of foreigners. CONCLUSIONS: Patients in developing nations who have limited access to standard neurosurgical care can be treated for frontoethmoidal MECs with few complications and a satisfactory cost-to-benefit ratio.

The prevalence of the Onodi cell - Most suitable method of CT evaluation in its detection.

BACKGROUND: The Onodi cell (sphenoethmoidal air cell) is an anatomic variant of the most posterior ethmoid cell that pneumatised superiorly and laterally to the sphenoid sinus, and is in close relation to the optic nerve. The proper identification of the Onodi cell is essential during a pre-operative computer tomography (CT) examination, as the presence of that ethmoid cell variant makes sphenoid sinus surgery more risky. The bulging of the optic nerve to the ethmoid cell wall is well visualized during endoscopic examination, but there are no clearly defined criteria for a potentially dangerous Onodi cell type in CT examinations. OBJECTIVE: To determine the prevalence and types of Onodi cell in CT examination and find the most suitable CT scanning planes to identify it. MATERIAL AND METHODS: Three plane (axial, coronal and sagittal) reconstructions of 196 paranasal sinuses were analyzed. The most posterior ethmoid cell was classified into four types, according to its position in relation to the sphenoid sinus and the optic nerve canal bulging into the lumen of the sphenoethmoid cell. RESULTS: The Onodi cell was detected in 39.8% of cases, although in 55.6% of cases a direct contact between the most posterior ethmoid cell and the optic nerve was present. Bulging of the optic nerve canal was seen in 25% of cases. In two cases two posterior ethmoid cells were in direct contact with the optic nerve canal. CONCLUSION: The prevalence of the Onodi cell was higher than previously reported. Pre-operative paranasal sinuses CT examination should be evaluated in all three planes (axial, coronal and sagittal) to avoid missing or over-detection of the Onodi cell. Axial and sagittal planes are preferable for the detection of the Onodi cell.

A complex therapy for treatment of frontoethmoidal meningoencephalocele in a developing third world country: neurosurgical aspects.

OBJECT: Meningoencephaloceles are congenital malformations that have a high incidence in the population of Southeast Asia. Frontoethmoidal meningoencephaloceles, the most common variety, require surgical treatment. The authors combined neurosurgical and craniofacial approaches for the development of a simple technique that corrects this type of meningoencephalocele in a one-step procedure that has not been discussed in the literature previously. METHODS: In three visits of approximately 1 week each, 30 patients suffering from a frontoethmoidal meningoencephalocele underwent surgery successfully at the Rose Charities Surgical Rehabilitation Center, Kien Khleang, Phnom Penh, Cambodia. To the authors' knowledge, this is the first reported series of operations in this geographical region to treat meningoencephaloceles at a relatively primitive surgical center. Difficulties faced in this series included tropical conditions, problems ensuring sterility, and limited technical support. CONCLUSIONS: The authors present the neurosurgical highlights and the outcomes in this series of patients. The single approach, via a bicoronal skin incision and small frontobasal trepanation, facilitates closure of the frontal skull defect and resection of the meningoencephalocele (including its extension into the facial area), as well as a satisfactory, one-step correction of the nasal skeleton and telecanthus.

Anterior encephaloceles: a series of 103 cases over 32 years.

Anterior encephalocele is a rarely reported CNS malformation with a geographical predilection for South-East Asia. The paucity of literature prompted us to analyse our results with hemiorbital advancement and classical Tessier's operation in 103 cases over 32 years (1971-2002). In our series, the frontoethmoidal subtype was the most common (80%), followed by the orbital (8%), transethmoidal (8%), transsellar (3%) and interfrontal types (1%). All patients with classical frontoethmoidal encephalocele had swelling over the nose or inner canthus since birth, with varying degrees of hypertelorism; and those with the nasopharyngeal subtype commonly presented with nasal obstruction and cerebrospinal fluid (CSF) rhinorrhoea. Neurofibromatosis was present in seven patients, all of whom had an orbital encephalocele. The diagnostic workup included a computerised tomography scan of the head in 96 patients and magnetic resonance imaging of the brain in 16 patients. The associated findings on imaging were hydrocephalus (15%), corpus callosum agenesis (7%), arachnoid cyst (3%), porencephalic cyst (3%) and single ventricle (3%). A classical Tessier's operation was performed in the initial 30 cases. However, since 1988, we have been performing a single stage hemiorbital advancement and repair of the encephalocele. There were three deaths, one due to pneumonia and two due to fulminant meningitis. Twenty-two patients (22%) had postoperative CSF leak, of which five required lumboperitoneal shunt placement. We believe that hemiorbital advancement offers satisfactory results with less morbidity than the Tessier's operation.

[Iatrogenic nasal meningocele: a cause of recurrent meningitis]. / Iyatrojenik nazal meningosel: Bir reküren menenjit nedeni.

We present a 35-year-old male patient with an eight-year history of endoscopic nasal polypectomy followed by five episodes of meningitis (4 episodes in the past 3 years). His major complaints were right-sided nasal obstruction, frequent watery nasal discharge, headache, and fever. Physical examination revealed a mass in the right nasal cavity. Computed tomography and magnetic resonance imaging demonstrated a defect in the cribriform plate and an ethmoidal meningocele. The patient was referred to the neurosurgery department for surgical reparation.