11/61. Congenital salivary gland anlage tumor of the nasopharynx.OBJECTIVE: Nasal and upper respiratory tract obstruction in the neonatal period can result from a variety of conditions, and may present with variable symptoms. In the absence of dysmorphic features or other abnormalities, causes of nasal obstruction may be difficult to differentiate on initial examination. We report an unexpected and potentially life-threatening condition arising during the work-up of this common neonatal complaint. DESIGN: Case report with literature review. RESULTS: A male neonate presented with complaints of nasal obstruction and feeding difficulties. A common diagnostic approach to neonatal nasal obstruction was performed, resulting in an unexpected and potentially life-threatening, albeit curative, result. Cannulation of the nasal cavity to rule out choanal atresia resulted in a burst of bleeding from the nose and mouth. A finger sweep of the oropharynx produced a dislodged mass lesion. pathology revealed a salivary gland anlage tumor of the nasopharynx. CONCLUSIONS: The diagnosis of a nasopharyngeal mass lesion should be considered in neonates with nasal obstructive symptoms. It is wise to place an index finger in the oropharynx when passing catheters to rule out choanal atresia to feel a dislodged mass lesion before it can become an airway foreign body. Should passage of nasal catheters result in bleeding and/or respiratory distress, the possibility of a displaced mass lesion must be considered immediately to institute prompt intervention.- - - - - - - - - - ranking = 1keywords = nasal, nose (Clic here for more details about this article) |
12/61. Technique for fabrication of splint preventing postsurgical restenosis in choanal atresia.choanal atresia, a congenital narrowing or obstruction of the nasal airway caused by significant respiratory distress in neonates, may require emergency intervention. Although atresia can be repaired surgically, restenosis is a common complication with this procedure. To prevent this, tubes are inserted into the nasal cavity immediately after surgery. This article describes a technique for preparing individual surgical splints designed to prevent postsurgical obstruction of the nasal cavity.- - - - - - - - - - ranking = 0.49368908725581keywords = nasal (Clic here for more details about this article) |
13/61. Two brothers with Burn-McKeown syndrome.We report on two brothers with normal intelligence, bilateral choanal atresia, and a characteristic pattern of facial dysmorphic features consisting of hypertelorism, lower lid coloboma, narrow palpebral fissures, prominent nasal bridge, small mouth with thin lips, and protruding ears. These features show striking similarity to patients with Burn-McKeown syndrome [Burn et al., 1992 Clin. Dysmorphol 1: 137-144] and confirm the existence of this rare condition. These brothers show some additional features that were not previously reported in patients with this syndrome including median cleft palate with oronasal fistula, preauricular tag, hypomimic face, and hypoplastic unilateral kidney, thus indicating that the clinical spectrum of this entity is broader.- - - - - - - - - - ranking = 0.32912605817054keywords = nasal (Clic here for more details about this article) |
14/61. Hyper-IgM syndrome with CHARGE association.A girl with coloboma of the iris, sensorineural deafness, growth delay, distinctive face, and cranial nerve dysfunction was diagnosed of CHARGE association in the first year of life. She presented with repeated otitis. At 3 yr of age, the patient suffered a septicemia (streptococcus pneumoniae, corynebacterium sp.). The immunoglobulin g (IgG) and IgA serum levels were decreased, IgM increased and cellular immunity parameters were normal, supporting the diagnosis of hyper-IgM (HIM) syndrome. The sequence of cd40 ligand and cytidine deaminase genes were normal. From then on, she was receiving immunoglobulin intravenously with an excellent outcome. Here, we report the first case of CHARGE association and HIM syndrome in the same patient. Although the cause could not be identified, a non-random link is likely.- - - - - - - - - - ranking = 0.012621825488375keywords = nose (Clic here for more details about this article) |
15/61. choanal atresia and rare craniofacial clefts: report of three cases with a review of the literature.OBJECTIVE: This retrospective study reports three cases associating choanal atresia (CA) and rare craniofacial clefts (RCFCs) described in Tessier's classification. Although still discussed, these two malformations could derive from the same embryopathogenic context, with an anomaly of migration of the neural crest cells as a common factor. In two of the three cases presented, the RCFC was diagnosed during the neonatal period, and the third case (case 3) was diagnosed at the age of 12 years. The knowledge of this malformative association must permit the diagnosis of CA as early as the neonatal period or through the thorough and directed examination of the children as part of the follow-up.- - - - - - - - - - ranking = 0.025243650976749keywords = nose (Clic here for more details about this article) |
16/61. Bilateral choanal atresia in an adult: is it compatible with life?Bilateral choanal atresia is potentially life-threatening. The condition almost always presents in the new born, with alternating phases of respiratory distress and apnoea relieved by crying. We report a rare case of bilateral choanal atresia presenting for the first time at 22 years of age. The patient had no features of cyanosis or apnoea, presenting only with bilateral nasal obstruction, rhinorrhoea and anosmia. His neonatal history was unremarkable. No syndromic association was noted excepting for telecanthus. The presence of bony atresia was confirmed on computed tomography (CT) scan and transnasal endoscopic surgery was used to obtain a patent airway. This case is a rare report of bilateral choanal atresia presenting for the first time in adult life.- - - - - - - - - - ranking = 0.32912605817054keywords = nasal (Clic here for more details about this article) |
17/61. De novo interstitial duplication 4(q28.1q35) associated with choanal atresia.We report a case of direct interstitial duplication of chromosome 4 from 4q28.1 to 4q35 associated with bilateral choanal atresia. The child also had dysmorphic features including a broad nasal bridge, telecanthus, downward slanting palpebral fissures, prominent ears, mild bilateral clinodactyly of the 5th fingers and bilateral hypoplasia of the 2nd-5th toenails. There was also a slightly dilated renal collecting system. At the age of 2.5 years, he had moderate global developmental delay, short, wide and tapering fingers, and short toes with hypoplastic toenails. To our knowledge, this is the second report of choanal atresia in a patient with trisomy 4q involving this region.- - - - - - - - - - ranking = 0.16456302908527keywords = nasal (Clic here for more details about this article) |
18/61. Coexistence of choanal atresia and Tessier's facial cleft number 2.choanal atresia is one of the most commonly observed congenital abnormalities of the nose. This defect is frequently associated with other congenital anomalies. One of the anomalies, which is rarely encountered is a Tessier's facial cleft 2. In this report, we present a 6-year-old girl who was brought to our department with complaints of nasal obstruction and chronic nasal discharge. On examination, a scar was seen on the left ala of her nose due to the former repair of a congenital defect. We found hypoplastic nasal cavities and bilateral choanal atresia demonstrated by rigid and flexible endoscopy. A CT scan supported these findings. This coexistence of bilateral choanal atresia with a number 2 facial cleft is an addition to a formerly proposed classification of laterofacial clefts. We think that this classification is better for describing the extent and origin of these complex lesions.- - - - - - - - - - ranking = 0.51893273823256keywords = nasal, nose (Clic here for more details about this article) |
19/61. Correction of facial deformity using a Red III device in a patient with Antley-Bixler syndrome.Antley-Bixler syndrome was first described in 1975 and to date at least 44 cases have been reported. In addition to brachycephaly, this syndrome is associated with midface hypoplasia, bilateral radiohumeral synostosis, multiple joint contractures, long bone fractures, dysplastic ears, "pear shaped" nose, and occasionally urogenital or cardiac defects. The authors report a case in a 23-year-old man with severe midface deficiency treated using distraction osteogenesis. A modified Le Fort III osteotomy was performed, and nasal subunit and remaining Le Fort III segment was distracted separately. The classic RED II design was found to be insufficient, so the authors added an extra horizontal bar to the system and converted it to a RED III design. Midface advancement was 11 mm from the porion to the orbitale and 31 mm at point A. Nasal advancement at the nasal root level was 5 mm. There were no complications, and at 4 months after surgery, advancement was stable without any relapse.- - - - - - - - - - ranking = 0.34174788365892keywords = nasal, nose (Clic here for more details about this article) |
20/61. charge syndrome from birth to adulthood: an individual reported on from 0 to 33 years.charge syndrome was independently reported by Hall [(1979): J Pediatr 95:395-398] and Hittner et al. [(1979): J Pediatr Ophthalmol strabismus 16:122-128] and was initially considered to be a non-random association between distinct multiple congenital anomalies. It is now considered to be a recognizable syndrome with well-characterized diagnostic criteria and a genetic pathogenesis. We report on a 33-year-old adult male with charge syndrome, with emphasis on the unique medical, behavioral, and psychological issues faced in adulthood. Characteristic facial and ear abnormalities were obvious in early childhood, and bilateral retinal colobomata, left choanal atresia, right congenital hip dislocation, and hypogonadism were diagnosed during the first year. walking was delayed due to vestibular problems, speech was impaired due to moderately severe hearing loss, and use of sign communication was limited. choanal atresia was surgically corrected in infancy, and atrial septal defect, ventricular septal defect, and patent ductus arteriosus were surgically corrected in childhood. Undescended testes were removed in adolescence, and gallstones were removed in early adulthood. puberty was delayed until hormone replacement therapy began at 15 years. Behavioral disturbances and anxiety persisted throughout childhood, adolescence, and into adulthood, often resulting from communication challenges. At 33 years of age, he lives independently in a supervised group home, receives regular eye check-ups and is being monitored for severely reduced bone density.- - - - - - - - - - ranking = 0.012621825488375keywords = nose (Clic here for more details about this article) |
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