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Incomplete Cleft Palate Repair: Von Langenbeck Converted to Two-flap Palatoplasty with Furlow Double Opposing Z-Plasty

Title:

Incomplete Cleft Palate Repair: Von Langenbeck Converted to Two-flap Palatoplasty with Furlow Double Opposing Z-Plasty

Authors:

Nima Vahidi, MD1; Nilan Vaghjiani, BS1; Rajanya Petersson, MS, MD1,2

1Virginia Commonwealth University School of Medicine, Richmond, VA

2Children Hospital of Richmond at VCU, Richmond, VA

Overview:

10-month-old male with 18q deletion syndrome, Pierre Robin sequence (cleft palate, glossoptosis, and micrognathia), eustachian tube dysfunction, cardiac disease including ASD, VSD and WPW, pulmonary hypertension, as well as tracheostomy and G-tube dependence.

In preoperative evaluation he was noted to have an incomplete cleft palate involving the hard and soft palate. He was noted to have bilateral eustachian tube dysfunction with effusions present. After discussion with family decision was made to proceed with surgical intervention.

Cleft palate repair was performed. A Dingman mouth gag was placed in the patient’s mouth. Palate was palpated, there was a visible cleft palate involving the soft palate and a portion of the hard palate. We proceed to perform the cleft repair with a von Langenbeck repair anteriorly and Furlow double opposing Z-plasty posteriorly. The operative area was injected with 1% lidocaine with 1: 100,000 epinephrine, a total of 6cc of local used. The proposed incisions were marked on the palate. The palate was then split through the midline at the junction of the nasal and oral mucosa. Anteriorly we elevated our nasal and oral flaps in a von Langenbeck fashion to close in a straight-line repair. We decided to convert to a two-flap palatoplasty as the palate was very high-arched anteriorly, and visualization was not ideal. We then dissected posteriorly and planned our double opposing Z-plasty. A posteriorly based myomucosal flap was then incised and elevated on the left-hand side. An anteriorly based mucosal flap was then elevated on the right-hand side. These flaps were retracted laterally with 4-0 Vicryl stay sutures. The nasal midline was then split and that nasal flaps were then developed on the left and right. There was a nasal myomucosal flap based posteriorly on the right, and a nasal mucosal flap based anteriorly on the left. Once the flaps were developed, they were rotated, and the wound was closed. The nasal layer was closed first by rotating the left sided anteriorly based nasal mucosal flap and the posteriorly based right sided myomucosal flap. Flaps were closed with 4-0 Vicryl in a combination of simple interrupted and running sutures. We then transposed the oral flaps and closed them with 4-o Vicryl in a simple interrupted manner, ensuring there was no tension on the closure. The hard palate flaps were then closed in the midline with 4-0 Vicryl in a simple interrupted fashion, with intermittent vertical mattress sutures to evert the edges. The uvula was approximated with 4-0 Vicryl in a simple interrupted manner. Lastly, anterolateral tacking sutures were placed between the palatal mucosa and alveolar ridge. This concluded our procedure.
- Repair of physical deformity - Main objective of cleft palate repair is creation of adequately functioning velopharynx for production of intelligible speech and improvement in swallow function. - Improvement in eustachian tube function - Minimize negative effects on maxillary growth pattern.
- Excessively wide palatal defects where there isn’t enough palatal tissue for closure, may consider delaying surgery to allow for increased growth. - Patients with syndromic disorders, tracheostomies, or mental disabilities consideration should be made as to the benefit of surgery and appropriate timing.
The patient was placed supine and turned 90 degree away from anesthesia team. A head wrap was placed, and the patient was draped in a standard fashion for operating in the mouth. As the surgery is intraoral, sterilization and sterile draping is not required. An oral ray endotracheal tube is utilized as it allows for placement of the Dingman mouth gag. We recommend a preoperative dose of dexamethasone (0.5 mg/kg, max of 10mg) as well as perioperative antibiotic coverage. The mouth gag is suspended on a stack of rolled tows for our cases instead of the Mayo stand.
Scheduling of palatoplasty is an important consideration in the management of cleft palate patients, surgeries are typically schedule between 10 and 12 months of age, and possibly a little bit later if there is concern for growth, comorbid conditions, and/or airway obstruction. There is no specific preprocedural workup necessary, outside of institutional preoperative anesthesia evaluation. Hemoglobin and hematocrit can be obtained as part of the preoperative workup. Consideration should be placed to the ears as well, as often these children will need myringotomy tubes placed at the time of palatoplasty. Imaging is not necessary in the preoperative evaluation
Embryologically palatal development takes place between 5 and 12 weeks of development. Anteriorly the maxillary prominences grow and push the nasal prominences together in the midline. The fusion of these prominences creates the primary palate. The secondary palate is formed by the fusion of the maxillary prominences starting at the incisive foramen and ending at the uvula. The type of palatal defect is a result of interruption at a certain stage in development. The incidence of cleft palate alone is about 1:2000, with a female to male predominance. Approximately 40% of cleft palate patients will have an underlying syndrome. Common Syndromes that may present with cleft palate: - Van der Woude Syndrome - Stickler Syndrome - Pierre Robin Sequence - 22q11.2 deletion Syndrome - Treacher Collins Syndrome - Klippel-Feil Syndrome - Nager Syndrome
Advantages: Bernard von Langenbeck described the palatoplasty technique in 1861, which is the oldest procedure commonly used today. The technique involves bipedicled mucoperiosteal flaps based posteriorly with an anterior connection to the alveolus. Commonly utilized for repair of incomplete and narrow cleft palate defects. The Bardach Two-Flap Palatoplasty is a modification of the Von Langenbeck technique which involves an incision along the cleft margin and the alveolar margin. The cleft is then repaired in a straight line fashion. This technique provides versatility and simplicity, and can be utilized to repair complete cleft palate defects. Another commonly utilized cleft palate repair technique is the Furlow Double Opposing Z-Plasty. This technique utilizes a double reverse Z-plasty for the oral and nasal surfaces of the soft palate. The two muscle based flaps are rotated posteriorly and the two mucosal based flaps are rotated anteriorly. This technique lengthens the soft palate and is commonly utilized for narrow soft palate clefts, submucous cleft defects or as a combination technique with hard palate cleft techniques. Disadvantages: Von Langenbeck: use is limited to the repair of incomplete cleft palates and narrow defects. Anterior visualization can be difficult. Two-Flap Palatoplasty: can result in an excess area of exposed bone and result in delayed healing. Furlow Double Opposing Z-plasty: can lead to soft palate scarring.
Complications can be subdivided into immediate complication and delayed complications. Immediate complications include hemorrhage, respiratory obstruction, hanging palate, dehiscence of repair or oronasal fistula formation. Late complication typically includes bifid uvula formation, velopharyngeal insufficiency, abnormal speech, and dental malpositioning.
None
N/A
Agrawal K. (2009). Cleft palate repair and variations. Indian journal of plastic surgery: official publication of the Association of Plastic Surgeons of India, 42 Suppl(Suppl), S102–S109. https://doi.org/10.4103/0970-0358.57197 Furlow LT Jr: Cleft palate repair by double opposing z-plasty. Plast Reconstr Surg 1986; 78: 724–736 Koudoumnakis E, Vlastos IM, Parpounas K, Houlakis M. Two-flap palatoplasty: description of the surgical technique and reporting of results at a single center. Ear Nose Throat J. 2012 Mar;91(3):E33-7. doi: 10.1177/014556131209100320. PMID: 22430346. Ravishanker R. (2006). Furlow's Palatoplasty for Cleft Palate Repair. Medical journal, Armed Forces India, 62(3), 239–242. https://doi.org/10.1016/S0377-1237(06)80010-9

Review Incomplete Cleft Palate Repair: Von Langenbeck Converted to Two-flap Palatoplasty with Furlow Double Opposing Z-Plasty.

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