The aglossia-adactylia syndrome

Álvarez, G.

doi:10.1016/0007-1226(76)90046-1

Cited by 20 publications

(9 citation statements)

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“…Although bony syngnathia has been reported as an isolated occurrence, most cases have been associated with other malformations. Most of the reported syndromal cases belong to the aglossia-adactylia syndrome (Peterson, 1961; Hoggins, 1969; Bernard et al, 1971; Alvarez, 1976; Arshad and Goh, 1994; Dawson et al, 1997; Knoll et al, 2000) in the spectrum of oromandibular limb defect, or to the facioauriculovertebral spectrum/hemifacial microsomia (Burket, 1936; Dawson et al, 1997). Isolated syngnathia (Hochstetter, 1948; Salleh, 1965; Mishkinyar, 1979; Sternberg, 1983; Nwoku and Kekere-Ekun, 1986; Goodacre and Wallace, 1990; Agrawal et al, 1993; Kamata et al, 1996; Yazdi and Fakhraee, 2000; Daniels, 2004; Ugurlu et al, 2005; Nikolic et al, 2006; Shams et al, 2006; Mir et al, 2007; Trigg et al, 2007; Vahidi et al, 2007; Gupta et al, 2008) with no other anomalies thus far has been reported in only 17 instances of congenital syngnathia.…”

Section: Discussionmentioning

confidence: 99%

“…The extent of fusion between the alveolar processes is variable, including complete fusion, unilateral fusion leaving a slitlike opening on the opposite side of the mouth, and bilateral fusion with a small anterior slit. Review of existent literature reveals 26 cases (Burket, 1936; Hochstetter, 1948; Alvarez, 1976; Shah, 1977; Dobrow, 1983; Kittur et al, 1983; Sternberg, 1983; Nwoku and Kekere-Ekun, 1986; Brown and Marsh, 1990; Goodacre and Wallace, 1990; Agrawal et al, 1993; Kamata et al, 1996; Dawson et al, 1997; Ugurlu et al, 1999; Knoll et al, 2000; Yazdi and Fakhraee, 2000; Laster et al, 2001; Daniels, 2004; Ugurlu et al, 2005; Shams et al, 2006; Mir et al, 2007; Trigg et al, 2007; Vahidi et al, 2007; Gupta et al, 2008) of bilateral or complete bony syngnathia, while unilateral cases have been reported in seven instances (Salleh, 1965; Mishkinyar, 1979; Behnia and Shamse, 1996; Dawson et al, 1997; Verloesa et al, 2004; Nikolic et al, 2006) and anterior fusion (Peterson, 1961; Hoggins, 1969; Bernard et al, 1971; Arshad and Goh, 1994; Rao et al, 1997) in six instances. Our case is an example of unilateral fusion of the left side with a slitlike opening on the right, which diminished posteriorly because of the presence of alveolar soft tissue synechiae.…”

Section: Discussionmentioning

confidence: 99%

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Congenital Syngnathia: Case Report and Review of Literature

Naikmasur

Sattur

Joshi

et al. 2010

The Cleft Palate-Craniofacial Journal

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Congenital bony fusion of the maxilla and mandible, especially as an isolated occurrence, is a very rare condition. The very few cases reported in the literature are mostly inadequate in description and confusing in nomenclature. An isolated case of syngnathia in a 60-year-old female patient with unilateral bony fusion (synostosis) of the maxilla and mandible associated with fibrous adhesions (synechiae) of the opposite site is reported. The existent literature is also reviewed.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Congenital Syngnathia: Case Report and Review of Literature

Naikmasur

Sattur

Joshi

et al. 2010

The Cleft Palate-Craniofacial Journal

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“…Bony maxillomandibular fusion can be subdivided on the basis of the location of the bony fusion. Of the 27 previously reported cases, 19 involved fusion of the alveolar ridges of the mandible and maxilla, whereas only 8 cases 8,15,16,20,21,[23][24][25] involved fusion of the ascending rami of the mandible to the zygomatic processes and to the posterior aspects of the maxilla. The extent of the fusion between the alveolar processes is variable, including complete fusion, 14 unilateral fusion leaving a slitlike opening on the opposite side of the mouth, 1,5,11 and bilateral fusion with a small anterior slit.…”

Section: Discussionmentioning

confidence: 99%

Prenatal Imaging of Congenital Maxillomandibular Fusion

Vahidi

Joe

Glenn

et al. 2007

Journal of Ultrasound in Medicine

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“…Early multidisciplinary assessment and management of such cases are advisable. Surgery under local anesthesia 4 carries the risk of local anesthetic overdose. Also, as the airway is not protected, the risk of inhalation during the procedure cannot be ruled out.…”

Section: Discussionmentioning

confidence: 99%

Anesthetic Management of Aglossia-Adactylia Syndrome: Report of a Case

1988

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M Hegazy, H Farag, M Naguib, Anesthetic Management of Aglossia-Adactylia Syndrome: Report of a Case. 1988; 8(3): [221][222][223] Aglossia-adactylia syndrome is a rare congenital malformation first reported by Rosenthal in 1932. 1 The main features of the syndrome are hypoglossia, hypognathia, microstomia, syngnathia, hypertrophy of the submandibular salivary glands, total or partial absence of distal parts of the limbs, and in some cases, a man-dibulomaxillary fusion. Associated anomalies in the reported cases were transposed viscera and dextrocardia, imperforate anus, and mental retardation.2 In cases of aglossia-adactylia with man-dibulomaxillary fusion, the most serious problem is upper airway management. We describe the anesthetic management of a patient with aglossiaadactylia syndrome. Case ReportA 10-week-old boy weighing 3 kg was referred to the plastic surgery unit for separation of congenital mandibulomaxillary fusion. The mother, a 42-year-old, gravida 11, para 11, had a normal full-term delivery. She was asthmatic and took dexamethasone, 2 mg, and dexachlorpheniramine maleate, 5 mg twice daily, during the pregnancy. The father was a healthy 63-year-old man. The parents were first cousins, but without family history of congenital anomalies.The baby had micrognathia, microstomia, and complete mandibulomaxillary fusion and both lips were adherent to the alveolar margins. In addition, he had a lobster-claw deformity of both hands, absent toes, and bilateral inguinal hernias. The baby had a nasogastric tube for feeding since birth. He was nursed in the prone position with frequent pharyngeal suction through the nose to avoid choking from secretions. X-ray films disclosed a cartilagenous mandibulomaxillary fusion with an osseous part at the mandibular symphysis. Chest X-ray film and abdominal CT scan were normal.Before separation of the mandibulomaxillary fusion, the infant was premedieated with 10 mg of vitamin K 12 hours before surgery and 0.1 mg of atropine 30 minutes before surgery, both administered intramuscularly. In the operating room the nasogastric tube was removed, and nasal decongestant drops were instilled. A 5% dextrose in 0.2% saline infusion was started through a 22-gauge cannula at a rate of 20 ml/hour. The ECG and rectal temperature were monitored continuously, and blood pressure was monitored every five minutes using a neonatal electronic oscillotonometer. An awake blind nasal intubation was attempted under X-ray visualization with a No. 3.5 tube and wire stylet, but the larynx was located too far anteriorly for successful intubation. A tracheostomy was then performed under local anesthesia, and a No. 3.5 tracheostomy tube inserted. The baby was anesthetized with 70% nitrous oxide in oxygen via a Jackson Rees modification of Ayer's T-piece circuit, and ventilation was controlled with 2 mg of atracurium.The cartilagenous and bony fusion was divided surgically. A blind bilateral osteotomy was performed at the ascending mandibular rami for adequate movement of the mandible. A cleft soft pal...

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The aglossia-adactylia syndrome

Cited by 20 publications

References 0 publications

Congenital Syngnathia: Case Report and Review of Literature

Congenital Syngnathia: Case Report and Review of Literature

Prenatal Imaging of Congenital Maxillomandibular Fusion

Anesthetic Management of Aglossia-Adactylia Syndrome: Report of a Case

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