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32-month-old male with Coffin Siris syndrome, bilateral middle ear effusions, and velopharyngeal insufficiency who presents with a submucous cleft palate.
Furlow Double-Opposing Z-Palatoplasty
Submucous cleft palate, velopharyngeal insufficiency
Any medical conditions that preclude using general anesthesia. Patients with airway conditions or syndromes, such as Pierre Robin sequence, retrognathia, may need to be addressed before palatoplasty.
Dingman mouth gag, 6700 beaver blade, Dandy trigeminal scissor, Stevens tenotomy scissors
General otolaryngology set up including Dingman mouth gag, 6700 beaver blade, Dandy trigeminal scissor, Stevens tenotomy scissors, 0.25% Marcaine with epinephrine.
Feeding/speech therapy, medical clearance, consideration for concurrent myringotomy tubes, preoperative parental counseling
Variable severity of a bifid uvula, zona pellucida, and bony indentation in the posterior edge of the hard palate can be seen in patient with a submucous cleft palate. A submucous cleft palate may involve only the secondary palate or both the primary and secondary palate. The palate is divided into primary and secondary palates by the incisive foramen, where the primary palate is anterior to the foramen and the secondary palate is posterior to it.
Palatal fistula formation (rare), scarring, bleeding, infection
None
1. Ravishanker R. Furlow's Palatoplasty for Cleft Palate Repair. Med J Armed Forces India. 2006;62(3):239-242. doi:10.1016/S0377-1237(06)80010-9.
2. Puente Espel J, Hohman MH, Winters R. Cleft Palate Repair. [Updated 2023 Jul 4]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK570586/
3. Sie KC, Tampakopoulou DA, Sorom J, Gruss JS, Eblen LE. Results with Furlow palatoplasty in management of velopharyngeal insufficiency. Plast Reconstr Surg. 2001;108(1):17-29. doi:10.1097/00006534-200107000-00004.
4. Chen KT, Wu J, Noordhoff SM. Submucous cleft palate. Changgeng Yi Xue Za Zhi. 1994;17(2):131-137. https://pubmed.ncbi.nlm.nih.gov/8069735/
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