[Transethmoidal encephalomeningocele in neonate--report of two cases and review of literatures].
Transethmoidal meningoencephalocele is caused by protrusion into the nasal cavity of a part of brain and meninges through an ethmoidal defect being a subtype of basal meningoencephalocele. It is extremely rare as only a few cases have been reported so far. It gives characteristic symptoms and signs as follows: (i) mouth breathing and snoring due to intranasal tumor and obstruction from birth, (ii) pulsation of the tumor synchronous with pulse or respiration, (iii) complication of facial deformities such as hypertelorism, cleft lip and palate, and (iv) leakage of cerebrospinal fluid from the nose, meningitis, etc. Moreover, agenesis of corpus callosum and congenital hydrocephalus are identified, which indicate the origin of this disease as have been reported by various authors. Recently, we experienced two cases of newborn infants with this anomaly. Case 1: A 1-day-old male was born by Caesarean section at full term, body weight at birth being 3500 gm, cranial circumference 35.2 cm, and Apgar score at 5 minutes of full. Since he was dyspneic while nursing, he was transferred to our clinic. Hypertelorism, unequal anterior nares, and micrognathia were the facial abnormalities at admission. Both nasal cavities were completely obstructed by abnormal bony tissue at about 3 cm from the anterior border of the nares. No obvious abnormality was found by neurological and biochemical examination. A transcranial repair of the basal encephalocele was performed. The crista galli could not be seen, but the base of the frontal skull was found to be deeply depressed into the ethmoid sinus proper just as seen by CT scan.(ABSTRACT TRUNCATED AT 250 WORDS)
Hayashi
T
T
Department of Neurosurgery, St. Mary's Hospital, Kurume, Japan.
Hashimoto
T
T
Anegawa
S
S
Utsunomiya
H
H
D000015:Abnormalities, Multiple; D002533:Cerebral Angiography; D004677:Encephalocele / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D008588:Meningocele / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D011379:Prognosis; D014057:Tomography, X-Ray Computed