This is a case of an 8 month old with a midline nasal mass present since birth. Preoperative physical exam and imaging was consistent with a nasal dermoid cyst with no evidence of intracranial extension.
The most common of the rare craniofacial clefts, Tessier’s No. 7 cleft is represented by a deficiency of tissue that may span from the oral commissure to the ear. (1) The repair of the cleft of the lip must include especial attention to restoring continuity of the orbicularis oris muscle as well the vermillion. This case is presented as an example of the repair of the Tessier 7 cleft lip deformity.
DOI #: http://dx.doi.org/10.17797/4h2edlts5zz
Contributors: Larry Hartzell
Repair of the bilateral cleft lip deformity can be challenging to the cleft and craniofacial surgeon. The goals of an acceptable repair must include precise continuity of the cupid’s bow, maximizing philtral length, and establishing a mucosa lined sulcus. We present an example of a repair of the bilateral incomplete lip as described by Millard.
Patients with Pierre-Robin Sequence (PRS) suffer from micrognathia, glossoptosis, and upper airway obstruction, which is sometimes associated with cleft palate and feeding issues. To overcome these symptoms in our full-term male neonate patient with PRS, mandibular distraction osteogenesis was performed.
The patient was intubated after airway endoscopy. A submandibular incision was carried down to the mandible. A distractor was modified to fit the osteotomy site that we marked, and its pin was pulled through an infrauricular incision. Screws secured the plates and the osteotomy was performed. The mandible was distracted 1.8 mm daily for twelve days.
During distraction, the patient worked with speech therapy. Eventually, he adequately fed orally. He showed no further glossoptosis or obstruction after distraction was completed.
In our experience, mandibular distraction is a successful way to avoid a surgical airway and promote oral feeding in children with PRS and obstructive symptoms.
By: Ravi W Sun, BE
Megan M Gaffey, MD
Adam B Johnson, MD, PhD
Larry D Hartzell, MD
Department of Otolaryngology – Head and Neck Surgery, University of Arkansas for Medical Sciences, Little Rock, AR, USA
Arkansas Children’s Hospital, Little Rock, AR, USA
Recruited by: Gresham T Richter, MD
Velopharyngeal dysfunction (VPD) refers to the improper control of airflow through the nasopharynx. The term VPD denotes the clinical finding of incomplete velopharyngeal closure. Other terms used to describe VPD include velopharyngeal insufficiency, inadequacy and incompetence. However, the use of VPD has gained popularity over these terms as they may be used to infer a specific etiology of impaired velopharyngeal closure.1
Control of airflow through the nasopharynx is dependent on the simultaneous elevation of the soft palate and constriction of the lateral and posterior pharyngeal walls. Disruptions of this mechanism caused by structural, muscular or neurologic pathology of the palate or pharyngeal walls can result in VPD. VPD can result in a hypernasal voice with compensatory misarticulations, nasal emissions and aberrant facial movements during speech.2
The assessment of velopharyngeal function is best preformed by a multispecialty team evaluation including speech-language pathologists, prosthodontists, otolaryngologists and plastic surgeons. The initial diagnosis of VPD is typically made with voice and resonance evaluation conducted by a speech-language pathologist. To better characterize the patient’s VPD, video nasopharyngeal endoscopy or speech videofluoroscopy can be used to visualize the velopharyngeal mechanism during speech.
VPD may first be managed with speech-language therapy and removable prostheses. For those who are good surgical candidates and do not fully respond to speech-language therapy, surgical intervention may be pursued.
Surgical management of VPD is most commonly accomplished by pharyngeal flap procedures or sphincter pharyngoplasty. In this video, a superiorly based pharyngeal flap with a uvular mucosal lining flap was preformed for VPD in a five-year-old patient with 22q11 Deletion Syndrome and aberrantly medial internal carotid arteries.
This video shows a pediatric patient with Van der Woude syndrome. He has lip pits that are classic for this syndrome and his family desired surgical correction. This video outlines and shows the steps of the modified simple excision technique as well as discussing tips for a successful surgery.
This video outlines the steps taken for pre-operative markings that need to be made prior to performing unilateral cleft lip repair using the Fisher anatomic subunit approximation technique. The technique has been written about in detail by Dr. David Fisher in his article “Unilateral Cleft Lip Repair: An Anatomical Subunit Approximation Technique”. This video simply outlines the markings that are made prior to performing this technique, which are crucial for correctly carrying out the repair.