11/113. Unilateral choanal atresia. A possible indication for computer aided surgery? "A report of two cases".choanal atresia (CA) is a congenital obstruction of the posterior nose. Bilateral CA is a paediatric emergency and must be treated surgically in the first few weeks after birth. This is in contrast to unilateral CA, because surgical management can be planned more selectively. Transpalatinal surgery for CA is a safe procedure. The endonasal technique is minimally invasive and less traumatic; however, a major disadvantage is a limited field of vision. Computer aided surgery using images acquired by computer tomography (CT) or magnetic resonance imaging (MRI) is an application that has emerged in the last few years. This application provides an additional safety factor for endoscopic surgery to resolve choanal atresia, especially in cases of another malformation. We report results from two cases of unilateral CA who were surgically managed with Computer aided surgery.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
12/113. Congenital choanal atresia in siblings.choanal atresia is a relatively rare anomaly of the upper airway, which can result in significant respiratory distress in the newborn. It is often associated with other anomalies and has only rarely been reported in siblings. We report its isolated occurrence in two siblings.- - - - - - - - - - ranking = 0.83333333333333keywords = atresia (Clic here for more details about this article) |
13/113. Familial choanal atresia with maxillary hypoplasia, prognathism, and hypodontia.We report on two sibs and a cousin with bilateral choanal atresia. At 2 months, one sib died of complications following surgical correction of her defects. We evaluated her brother and cousin at age 7 and 9 years, respectively. Both had a tall forehead, maxillary hypoplasia, prognathism, and absence of certain deciduous and permanent teeth. Psychomotor development was appropriate for age. Roentgenocephalometric analyses of several relatives showed that one grandfather of these children and two of the five uncles and aunts also had maxillary hypoplasia and/or prognathism. To our knowledge, this condition has not been described previously and may represent a newly recognized autosomal dominant condition with incomplete penetrance and variable expressivity caused by a defect of neural crest development.- - - - - - - - - - ranking = 0.83333333333333keywords = atresia (Clic here for more details about this article) |
14/113. choanal atresia in premature dizygotic twins--a transnasal approach with holmium:YAG-laser.twins born prematurely presented with choanal atresia and were successfully treated using a transnasal, endoscopically and microscopically controlled Ho:YAG-Laser assisted technique. One twin, who had bilateral choanal atresia was operated immediately, the other one, with unilateral choanal atresia, received surgery 8 months after birth. The rare feature of twins both suffering from choanal atresia and a technique for definitive treatment of this disease in premature neonates are presented and discussed. This report of dizygotic twins with nonsyndromal choanal atresia suggests the possibility of an autosomal recessive inheritance with various penetration or an undefined teratogenic etiologic factor.- - - - - - - - - - ranking = 1.5keywords = atresia (Clic here for more details about this article) |
15/113. Treatment of a recurrent choanal atresia by balloon dilatation.A 16-year-old female patient with recurrent stenosis after unilateral choanal atresia that had been treated by surgery and laser therapy presented with restenosis 3 weeks after laser resection. As an alternative to repeat surgery, she was treated by balloon dilatation. Four balloon dilatations were performed (three at 4-week intervals and the last after an 8-week interval) over a 4-month period on an outpatient basis. She tolerated the treatment well without any complications. Over a follow-up period of 2 years the choana has remained completely open. The balloon dilatation described in this context here for the first time may be a good alternative to repeated operations in the treatment of recurrent choanal atresia, with minimal stress to the patient.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
16/113. temporal bone anomaly proposed as a major criteria for diagnosis of charge syndrome.The acronym CHARGE defines a non-random clustering of congenital malformations of unknown origin. Classical diagnostic criteria include: 1) one major feature namely coloboma/microphthalmia or choanal atresia, and 2) four of the six features designated in the CHARGE acronym. Interestingly, all CHARGE patients hitherto reported had partial or complete semicircular canal hypoplasia on temporal bone CT-scan. We report on semicircular canal agenesis/hypoplasia in three patients with three to four features of the charge syndrome and neither coloboma nor choanal atresia and we propose to include temporal bone malformations as a major criteria for diagnosis of charge syndrome.- - - - - - - - - - ranking = 0.33333333333333keywords = atresia (Clic here for more details about this article) |
17/113. Bilateral choanal atresia associated with nasal dermoid cyst and sinus: a case report and review of the literature.The relationship of choanal atresia to various systemic malformations is well acknowledged by the CHARGE acronym: colobomas, heart defects, atresia choanae, retarded growth and development, genito-urinary defects, and ear defects. In the past, we have reported the finding of an isolated skull base malformation associated with choanal atresia. We report here a unique case of choanal atresia associated with a patent foramen cecum and intracranial extension of a dermoid sinus. The embryogenesis of this condition is accounted for by the mesodermal flow theory of choanal atresia formation, and implies a need for thorough imaging of the anterior skull base in cases of bilateral atresia.- - - - - - - - - - ranking = 1.6666666666667keywords = atresia (Clic here for more details about this article) |
18/113. Complete congenital bony syngnathia in a case of oromandibular limb hypogenesis syndrome.Congenital fusion of the maxilla and mandible (syngnathia) is rare and can present in a wide range of severity from single mucosal bands (synechiae) to complete bony fusion (synostosis). Congenital synostosis of the mandible and maxilla is even less common than synechiae, with only 19 cases reported in the literature. Most of them have presented as an incomplete, unilateral fusion. Only three of the reported cases showed more extensive but still incomplete, intermaxillary bony fusion. We present a case of complete bony fusion of the maxilla and mandible associated with a cleft palate, hypoglossia, micrognathia, unilateral choanal atresia, and limb abnormalities as a feature of oromandibular limb hypogenesis syndrome. Details of operative management are presented.- - - - - - - - - - ranking = 0.16666666666667keywords = atresia (Clic here for more details about this article) |
19/113. costello syndrome: report of a new case with choanal atresia and fatal outcome.We describe a girl with motor and mental retardation, macrocephaly, a "coarse" face, choanal atresia, postnatal feeding difficulty, redundant skin with deep palmar and plantar creases, and histopathological evidence of altered elastic fibers, who died at the age of 11 months. We believe this represents another case of costello syndrome. Lacking papillomata, she had choanal atresia and underwent a fatal outcome at an early age. The differential diagnosis of cutis laxa in association with postnatal growth retardation and developmental delay and with cardio-facio-cutaneous and Noonan syndromes is discussed.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
20/113. Diagnostic and therapeutic problems in a case of prenatally detected fetal hydrocolpos.We report on a female fetus with prenatally suspected hydrometrocolpos. Postnatal evaluation additionally revealed ambiguous genitalia, anorectal atresia, vertebral segmentation anomalies and congenital intestinal aganglionosis. colostomy was performed, but postoperative recovery was complicated by pulmonary hypertension and renal failure, resulting in death at day 18. Postmortem examination furthermore revealed a small ventricular septal defect, as well as rectovaginal and urethrovaginal fistulae, causing massive dilatation of the septated vagina (hydrocolpos). The possibility of an overlapping VACTERL and MURCS association is discussed.- - - - - - - - - - ranking = 0.16666666666667keywords = atresia (Clic here for more details about this article) |
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