Presentation
OBJECTIVE: Nasal encephalocele may presents as a nasal mass, its treatment is surgical and it should be done early in life. [ncbi.nlm.nih.gov]
Nasal encephaloceles usually present at birth with symptoms of obstruction or other complications. It presents as an external swelling on the nose. [radiopaedia.org]
In a study including 114 patients, 59% have presented normal development, 18% have presented some degree of physical or mental deficiency, and 23% have a severe deficiency( 2). [rb.org.br]
Entire Body System
- Swelling
It presents as an external swelling on the nose. The swelling is usually soft, with normal overlying skin, and increases in size on coughing/straining. Symptomatic patients usually present with obstruction or rhinorrhea. [radiopaedia.org]
During preanaesthetic check-up, difficult mask ventilation was anticipated, as the base of nose was occupied by the irregular shaped, uneven swelling. There were doubts on achieving a perfect seal with face mask. [joacp.org]
The swelling increases in size in response to coughing. Most common site is occipital and then frontal. Bilateral compression of the internal jugular vein also leads to the increase in the size of mass called as Frustenberg Test. [medicowesome.com]
Case-4: A 12-year-old female presented to our clinic with complaint of a swelling over her nose since few years. The swelling was initially about the size of a lemon but progressively increased in size as she grew. [jpma.org.pk]
- Pathologist
[…] interdisciplinary approach to the diagnosis, treatment, and management of head and neck diseases, including the incidence, etiology, clinical presentation, pathology, differential diagnosis, and prognosis for each disorder-promoting clear communication between pathologists [books.google.de]
Perez-Atayde, MD, is Director of Special Techniques and Staff Pathologist at Boston Children's Hospital. [books.google.com]
“Nasal gliomas” and related brain heterotopias: a pathologist’s perspective. Pediatr Pathol 1986;5:353-62. 20 Fujioka M, Tasaki I, Nakayama R, Yakabe A, Baba H, Toda K, et al. Both nasal cerebral heterotopia and encephalocele in the same patient. [zdoc.site]
- Dentist
[…] general pathologists; otolaryngologists; oral, maxillofacial, plastic and reconstructive, general, head and neck, and orthopedic surgeons and neurosurgeons; oncologists; hematologists; ophthalmologists; radiologists; endocrinologists; dermatologists; dentists [books.google.de]
The book will appeal to clinical geneticists, pediatricians, neurologists, head and neck surgeons, otolarynologists, and dentists. The 4th edition, which published in 2001, has sold 2,600 copies. [books.google.it]
The ENT specialist must be aware of the fact that a dental origin is often overlooked by radiologists as well as by dentists so that a so-called inconspicuous dental or radiological examination cannot exclude dental genesis [691], [692], [693]. [egms.de]
- Surgical Procedure
Reposition (Medizin) – Reduction is a surgical procedure to restore a fracture or dislocation to the correct alignment. [wikivisually.com]
(b) Coronal CT showing transethmoid nasopharyngeal encephalocele Click here to view Surgical procedure Surgery was considered for repair of the encephalocele and correction of the deformity. [pediatricneurosciences.com]
In combination with the special growth behavior, the inverted papilloma requires a consequent surgical procedure to minimize the risk of recurrences. [egms.de]
- Developmental Disorder
Disorders associated with an encephalocele Other developmental disorders usually accompany encephalocele. [upmc.com]
Respiratoric
- Rhinorrhea
Key Words: Encephalocele · Cerebrospinal fluid rhinorrhea · Skull base · Encephalitis · Epilepsia. [jkns.or.kr]
Discussion The cause of spontaneous cerebrospinal fluid rhinorrhea in adults may be congenital or acquired [ 10, 12 ]. [jmedicalcasereports.biomedcentral.com]
Symptomatic patients usually present with obstruction or rhinorrhea. Nasal encephaloceles are typically identified in association with a discernible cranial bone defect. [radiopaedia.org]
- Aspiration
[…] distinguished physicians, Surgical Pathology of the Head and Neck, Second Edition now contains: over 1045 photographs, micrographs, drawings, and tables-nearly 200 more illustrations than the first edition five new chapters on molecular biology, fine-needle aspiration [books.google.de]
Consequently a surgeon might not consider an encephalocele among his differentials and proceed to carry out a diagnostic procedure such as fine needle aspiration (FNA), which could potentially be disastrous; leading to neurological deficits and intracranial [jpma.org.pk]
Ultrasound guided aspiration works with the inverse piezoelectric effect which allows bone removal realized by continuous rinsing and suction. It causes less heat than conventional drills. Soft tissue is protected [1176], [1211]. [egms.de]
- Hyposmia
The possibility of deterioration of olfaction (hyposmia, anosmia) in up to 10% should be mentioned preoperatively [1232], [1234], [1235], [1236]. [egms.de]
- Liquorrhoea
[…] nasal encephalocele: a review of 35 cases. ( 7699076 ) Turgut M....Erbengi A. 1995 9 Nasal endotracheal intubation in a premature infant with a nasal encephalocele. ( 8346963 ) Bannister C.M....Placzek M. 1993 10 A large convexity meningioma followed by liquorrhoea [malacards.org]
Cardiovascular
- Hypertension
In adults, encephaloceles are mainly due to traumatic or iatrogenic causes but are also increasingly recognised in idiopathic intracranial hypertension. Genetics Most cases tend to be sporadic. [radiopaedia.org]
Post-operatively, patient had no complications Case-2: A 50-year-old female, known case of hypertension, diabetes and deviated nasal septum, was referred to our clinic with complains of cerebrospinal fluid (CSF) rhinorroehoea for 6-7 years from her right [jpma.org.pk]
He was being treated for hypertension but presented no other significant pathological conditions. [zdoc.site]
If the infant fails to respond to medical treatment within 10–15 days, loses weight, has cyclical cyanosis, or develops pulmonary hypertension from obstruction, surgical repair is recommended. [shahfacialplastics.com]
Apparently it is even higher after accidents and resulting pneumatocephalus compared to spontaneous CSF with intracranial hypertension [704], [707]. [egms.de]
Face, Head & Neck
- Facial Mass
Frontoethmoidal encephaloceles can be recognized as a facial mass covered with normal skin, while basal encephaloceles may cause nasal obstruction or symptoms related to herniation of basal structures. [ncbi.nlm.nih.gov]
Neurologic
- Seizure
There were no further episodes of CSF-rhinorrhea and no more seizures reported at follow-up. The application of levetiracetam had been stopped without any further appearance of seizures. [jkns.or.kr]
[…] paralysis of the arms and legs), microcephaly (abnormally small head), ataxia (uncoordinated movement of the voluntary muscles, such as those involved in walking and reaching), developmental delay, vision problems, mental and growth retardation, and seizures [calsinus.com]
Cited by Diagnosis, treatment and postsurgical complications in a dog with epileptic seizures and a naso-ethmoidal meningoencephalocele. Mojarradi A, Van Meervenne S, Suarez-Bonnet A, De Decker S. Mojarradi A, et al. [pubmed.ncbi.nlm.nih.gov]
Complete loss of strength in the arms and legs, An unusually small head, Uncoordinated use of muscles needed for movement, such as those involved in walking and reaching, Developmental delay, Intellectual disability, Vision problems, Delayed growth, and Seizures [cdc.gov]
Those that live with occipital encephaloceles often have more problems, including delays in development, problems seeing, balance and coordination problems, hydrocephalus (fluid on the brain) and seizures Frontonasal (between the nose and forehead). [nationwidechildrens.org]
- Stroke
Smith, MD, is Director, Pediatric Cerebrovascular Surgery Co-Director, Neurosurgical Stroke Program Co-Director, Center for Head, Neck and Skull Base. Antonio R. [books.google.com]
Lakewood, CA 90715-1653 USA Phone: (562) 924-6666 Toll-free: (888) 857-3434 Email: [email protected] or [email protected] Website: http://www.nhfonline.org NIH/National Institute of Neurological Disorders and Stroke P.O. [rarediseases.org]
The complication rates regarding mortality, stroke, blindness, or blood transfusion are considered as being rather low equally [783]. [egms.de]
Workup
The preoperative workup is essentially based on imaging. [csurgeries.com]
Imaging studies in the diagnostic workup of neonatal nasal obstruction. J Comput Assist Tomogr. 2001;25(4):540. [shahfacialplastics.com]
Colonoscopy
- Polyps
These lesions may be mistaken for nasal polyps. A 36-year-old woman died after the removal of an intranasal encephalocele during a routine polypectomy. [jamanetwork.com]
Microscopically, these polyps differ very little from the common nasal polyps. Choanal polyps must, in fact, all be in the form of a constricted sac of which one half is in the antrum and the other in the nose and nasopharynx. [ncbi.nlm.nih.gov]
R. (1983): Intranasal encephalocele masking as a nasal polyp in an adult patient.Archives Otolaryngology 109: 420–421. [link.springer.com]
As CT & MRI are very useful in making a diagnosis and to differentiate it from other nasal masses like polyp, mucocele, thus avoids biopsy and helps in proper surgical management. [jemds.com]
Treatment
OBJECTIVE: Nasal encephalocele may presents as a nasal mass, its treatment is surgical and it should be done early in life. [ncbi.nlm.nih.gov]
Treatment Modalities 480 Conclusions 483 Conventional and Endoscopic Approaches to the Pituitary 485 Surgical Indications 486 Conventional Approaches to the Pituitary 487 Outcomes 489 References 490 Nasal and Paranasal Sinus Anatomy for the Endoscopic [books.google.com]
This is essential for determining the best treatment for an encephalocele. Encephalocele Treatment Surgery to remove the herniated sac and repair the opening in the skull is the only encephalocele treatment. [upmc.com]
Prognosis
[…] may be associated with brain malformations that can have an effect on the prognosis. [chw.org]
Treatment and prognosis The prognosis is variable depending on the presence of associated anomalies and presence of microcephaly (carries a much poorer prognosis). [radiopaedia.org]
What is the prognosis? The prognosis for individuals with encephaloceles varies depending on the type of brain tissue involved, the location of the sacs, and the accompanying brain malformations. [calsinus.com]
[…] this highly lauded three-volume reference provides an interdisciplinary approach to the diagnosis, treatment, and management of head and neck diseases, including the incidence, etiology, clinical presentation, pathology, differential diagnosis, and prognosis [books.google.de]
The prognosis appears to be better for patients with frontoethmoidal encephaloceles than for patients with occipital or parietal encephaloceles, and it depends largely on the presence of additional congenital anomalies of the brain. [ncbi.nlm.nih.gov]
Etiology
Updated, reorganized, and revised throughout, this highly lauded three-volume reference provides an interdisciplinary approach to the diagnosis, treatment, and management of head and neck diseases, including the incidence, etiology, clinical presentation [books.google.de]
Etiology and embryogenesis Theories abound to explain teratoma occurrence. [emedicine.medscape.com]
These lesions affect children from the low socioeconomic class, but its etiology remains poorly understood.[ 8 ] Encephaloceles can be congenital or acquired secondary to tumor, hydrocephalus or other cause.[ 9 ] Some suggest that its etiology could be [ncbi.nlm.nih.gov]
Reich, EW, Wishnick, MM: A clinical investigation into the etiology of frontonasal dysplasia. Am J Hum Genet 33: 88 A, 1981. Google Scholar 19. [journals.sagepub.com]
Epidemiology
The discussion covers the phenotype spectrum, epidemiology, mode of inheritance, pathogenesis, and clinical profile of each condition, all of which is accompanied by a wealth of illustrations. [books.google.it]
Each entity is extensively discussed with information on clinicopathological, epidemiological, immunophenotypic and genetic aspects of these diseases. This book is in the series commonly referred to as the "Blue Book" series. Contributors: :Dr. [books.google.de]
Epidemiology of frontoethmoidal encephalomeningocoele in Burma. J Epidemiol Community Health. 1984; 38 :89–98. [ PMC free article ] [ PubMed ] [ Google Scholar ] 12. Bhagwati S, Mahapatra A. Pediatric Neurosurgery. [ncbi.nlm.nih.gov]
Recurrences or more correctly persistence may be seen in up to 30% of patients if not completely excised. [1] Epidemiology [ edit ] Nasal glial heterotopia is rare, while an encephalocele is uncommon. [en.wikipedia.org]
Most registries and epidemiological studies classify encephaloceles using broad categories like frontal, parietal, occipital and sphenoidal. [rarediseases.org]
Pathophysiology
Nasal encephaloceles: a review of etiology, pathophysiology, clinical presentations, diagnosis, treatment, and complications. Tirumandas M, Sharma A, Gbenimacho I, Shoja MM, Tubbs RS, Oakes WJ, Loukas M. Tirumandas M, et al. [pubmed.ncbi.nlm.nih.gov]
Fig. 1 Midline nasal mass pathophysiology. ( A ) Encephalocele: neural contents herniate through the foramen cecum. ( B ) Glioma: trapped neural contents with an intact skull base. ( C ) Dermoid: invagination of skin elements toward the skull base. [pocketdentistry.com]
Currently it is unclear if it is a pathogenetic factor of the disease or mere a consequence [19], [1294], [1299] and which exact pathophysiological correlations exist [1291]. [egms.de]
Prevention
These must be prevented by careful planning of the procedure after having studied the patient���¢s anatomy on the CT-scan. [csurgeries.com]
Suwanwela Medicine British journal of preventive & social medicine 1972 In western countries, spinal and occipital meningoceles are common, but in Thailand the authors have found an exceptionally high incidence of encephalomeningocele in the anterior [semanticscholar.org]
Wang Y, Liu G, Canfield MA, Mai CT, Gilboa SM, Meyer RE, Anderka M, Copeland GE, Kucik JE, Nembhard WN, Kirby RS; National Birth Defects Prevention Network. [cdc.gov]
Surgery is performed early in life to prevent progression of the process and prevent damage to the herniated tissue. [chop.edu]
If brain tissue is present, it may be referred to as a meningoencephalocele. [2] When the head size or occipitofrontal circumference is smaller than the herniating sac, then it is termed as giant encephalocele. [3] Prevention [ edit ] It is recommended [en.wikipedia.org]