Treatment of patients with cleft lip, alveolus, and palate: an executive summary

Key points

• The Dutch guidelines contain 71 recommendations on genetic testing, feeding, lip and palate closure, hearing, hypernasality, bone grafting, orthodontics, psychosocial guidance, dentistry, osteotomy versus distraction, and rhinoplasty.
• Lip repair should be performed in the first 6 months after birth, and the soft palate should be closed during the first year of life.
• All patients with an orofacial cleft should be referred to a specialized centre for clinical genetics, preferably before their first operation.
• Oral feeding should start as soon as possible after birth and after surgery; tube feeding is not advised as the only feeding method.
• The quality of evidence was rated moderate to very low, so many recommendations are based on expert consensus.

Overview

This executive summary presents Dutch clinical practice guidelines for patients with cleft lip, alveolus and/or palate. They were developed because treatment varied significantly between cleft teams in the Netherlands. A multidisciplinary working group, together with patients and parents and assisted by two epidemiologists, screened 5157 articles and based its conclusions on 60 studies. The guidelines give recommendations for each stage of cleft care, but high-quality evidence is lacking and more good studies are needed.

Development of the guidelines

Clefts of the lip, alveolus and palate are among the most common congenital abnormalities, with a prevalence of approximately 1.7 per 1000 live births in Western Europe. Care by a multidisciplinary team can continue until about 22 years of age. The guidelines followed the Dutch 'Richtlijnen 2.0' standard and the AGREE II instrument. They were developed between 2013 and 2016, with additional modules added from 2017 to 2019. No systematic searches were performed for the chapters on genetic testing and dentistry.

Genetics, feeding and surgical repair

For an isolated cleft palate, a single nucleotide polymorphism (SNP) array is recommended before the first operation, and further testing such as whole-exome sequencing or a gene panel can be considered. Every cleft team should include an experienced speech therapist to support a normal drinking and eating pattern, and the feeding method is chosen individually with the parents. If optimal maxillary growth is the aim, the hard palate is repaired later. If optimal speech development is pursued, both the hard and soft palate should be closed in the first year. During palate repair, the palatal muscles should be moved to a more anatomically correct position, for example with a Furlow or Von Langenbeck technique. A Furlow double opposing Z-plasty should not be used for a wide cleft palate because of the increased fistula risk, and the Wardill-Kilner pushback technique is preferably avoided.

Hearing and speech

Neonatal hearing screening results should be checked, and audiology check-ups performed periodically up to 3 to 4 years of age. Grommets should be inserted only if indicated. Hearing aids give comparable audiological results with fewer complications, although grommets are the most cost-effective approach in the long term. Velopharyngeal dysfunction (VPD) should be diagnosed by a team including at least a cleft surgeon, a speech therapist and an ENT specialist. After primary palate repair, VPD should be diagnosed only when 6 months of specialized speech therapy has not given adequate results. Nasal endoscopy is usually possible from the age of 3.5 years, with videofluoroscopy as an alternative. Dynamic MRI is not recommended as a routine procedure.

Bone grafting, orthodontics and dental care

An early secondary bone graft is preferred to close the alveolar cleft. Its timing is based on the position and root formation stage (half to two-thirds) of the maxillary canine on the cleft side. Bone can be taken from the iliac crest if a large volume is needed, or from the chin supplemented by bone substitute. Nasoalveolar molding should be used only when preparing for or performing a clinical trial. A removable retainer that maintains the transverse dimensions of the upper dental arch should be worn at night for life and checked at least once every two years. A dentist with an interest in pediatric dentistry should see the child when the first deciduous teeth erupt (at 6 to 12 months) and again at 5 years. The general dentist should check the child at least every 6 months.

Frequently asked questions

When should psychosocial screening take place?

Both the patient and the parents should be screened after birth and when the child is 2-3, 5, 10-11 and 17 years old, using the same validated instrument across teams, such as the Strength and Difficulties Questionnaire.

When is secondary nasal surgery done?

To limit the total number of operations, secondary nasal surgery is preferably delayed until mid-face growth has ceased and any planned orthognathic surgery is completed.

Do the guidelines apply to syndromic clefts?

They were written for non-syndromic clefts. Many recommendations may also apply to clefts combined with other anomalies, with adjustments for the underlying condition.

Source

Mink van der Molen AB, van Breugel JMM, Janssen NG, Admiraal RJC, van Adrichem LNA, et al. Clinical Practice Guidelines on the Treatment of Patients with Cleft Lip, Alveolus, and Palate: An Executive Summary. Journal of Clinical Medicine 2021;10(21):4813. DOI: 10.3390/jcm10214813. Open access article. This page is a summary prepared by Medpresso from the original publication and is not a substitute for the full text or for medical advice.