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ExperimentaL Oral Medicine
Published in Samuel Dreizen, Barnet M. Levy, Handbook of Experimental Stomatology, 2020
Samuel Dreizen, Barnet M. Levy
The classic constellation of findings in the Pierre Robin syndrome is micrognathia, glossoptosis, and cleft palate associated with respiratory obstruction. Along with these may be other minor malformations such as syndactyly or major anomalies such as achondroplasia, congenital amputations, and congenital heart disease. Although the exact etiology of the Pierre Robin syndrome remains obscure, Cooke133 has successfully reproduced the syndrome in chickens. Fertile white Leghorn eggs were incubated at 38°C. From 0.25 to 0.50 mg deoxyguanosine in a suspension of 0.5% carboxymethylcellulose was injected into the yolk sac on the 4th day of incubation by needle puncture into the blunt end of 40 eggs that were candled daily. From the 10th day on, dead embryos were inspected and gross abnormalities recorded. Embryos surviving the 18th day were also examined.
Head and neck
Published in Spencer W. Beasley, John Hutson, Mark Stringer, Sebastian K. King, Warwick J. Teague, Paediatric Surgical Diagnosis, 2018
Spencer W. Beasley, John Hutson, Mark Stringer, Sebastian K. King, Warwick J. Teague
Enlargement ofthe tongue may be congenital, as in Beckwith- Wiedemann syndrome, hypothyroidism (cretinism) or Down syndrome, or may be acquired, due to a space-occupying lesion such as a haemangioma or lymphangioma. Displacement of the tongue producing apparent enlargement is present in Pierre- Robin syndrome. Overgrowth of the tongue with Beckwith- Wiedmann syndrome may require surgical reduction, while lymphangioma, although producing an enlarged tongue, may be more difficult to treat surgically. A mass in the midline of the tongue posteriorly may be a lingual thyroid, caused by failure of the normal descent of the thyroid from the region of the foramen caecum; it may be the only functioning thyroid tissue present.
Transport of the surgical neonate
Published in Prem Puri, Newborn Surgery, 2017
Babies with Pierre Robin syndrome carry a high risk of tongue swallowing and asphyxiation. The baby should be nursed prone to prevent the tongue from falling back into the airway and an oropharyngeal airway inserted.6
Paraglossal straight blade intubation in syndromic children
Published in Southern African Journal of Anaesthesia and Analgesia, 2018
Michiel A du Toit, Rebecca M Gray
The basis for airway obstruction in Pierre Robin syndrome is micrognathia, retrognathia and glossoptosis. These abnormalities can also be accompanied by a cleft palate in up to 50% of patients. Because of this combination of facial abnormalities, conventional midline laryngoscopy is often unsuccessful and can cause soft tissue trauma. The efficacy of paraglossal straight blade laryngoscopy was shown in a series of neonates with severe Pierre Robin syndrome undergoing elective glossopexy.12 Further case reports have also demonstrated the efficacy of paraglossal laryngoscopy in this subset of patients.13