Introduction

Cleft surgeons are united globally by a common desire to provide the highest quality care for their patients and yet there is a striking diversity of surgical pathways in common use with a paucity of scientific evidence to support them.1,2 Cleft surgeons have typically published their chosen surgical pathways towards the end of their career, when they are established and have earned their reputation. High-profile examples of this include Roberto Brusati3 and Jean Claude Talmant.4 While it is undoubtedly incredibly valuable to learn from experienced clinicians, publishing at the end of a career, in isolation, could potentially be problematic due to recall bias.

Centralised cleft services in the United Kingdom (UK) match their number of surgeons to their caseload, to ensure surgeons operate on at least 30 new babies born with cleft lip and/or palate per year, as stipulated in the national service specification.5 The Welsh Centre for Cleft Lip and Palate is relatively unusual in that it is a single-surgeon UK cleft service (Fig. 1). Having been appointed as the Welsh Cleft Surgeon September 2024, I had the opportunity to design bespoke cleft surgery pathways at the start of my cleft surgery career with the multidisciplinary cleft team.

Fig. 1
figure 1

A map to show the 12 cleft services in the United Kingdom and the estimated number of cleft surgeons performing infant surgery in each service. Created with mapchart.net

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The aim of this opinion article is to document my chosen paediatric cleft surgical pathways (for patients aged 0–16 years) in the hope that an open declaration of surgical intent and associated reasoning would set the scene for transparent and accurate outcome reporting in the future.

Training to be a cleft surgeon and designing cleft pathways

My surgical training was, for the most part, carried out within the UK National Health Service (see online Supplementary Information).6 I trained in the plastic and reconstructive surgery parent specialty and undertook two years of dedicated cleft fellowship training (Cleft and Craniofacial Fellowship at the Royal Children's Hospital in Melbourne, Australia and the Training Interface Group Fellowship in Cleft Lip and Palate at the Spires Cleft Centre in Oxford and Salisbury, UK), before being appointed as an independent consultant cleft surgeon. Cleft surgery pathway decisions were initially proposed by me, based on my personal training experiences and interpretation of evidence in the literature. Modifications were made following discussion with cleft care professionals within the Welsh cleft multidisciplinary team and presentation to global cleft professionals at a dedicated Circle of Cleft Professionals online forum.7

This article details the agreed surgical pathways by cleft phenotype (see the online Supplementary Information for accompanying peri-operative care, operative kardex and operative steps).

Unilateral and bilateral cleft lip +/- alveolar cleft

Single-stage reconstruction of the unilateral cleft lip will be scheduled at 4–5 months, aiming to improve maternal bonding in infancy.5,8,9

The markings for unilateral lip reconstruction are influenced by the anatomical subunit approximation technique described by David Fisher10 and modifications described by Marc Swan11 and David Chong.12 Subperiosteal dissection is chosen to reorientate the facial envelope4 because of limited evidence to suggest a detrimental impact on maxillary growth outcomes compared to supra-periosteal dissection.13 The primary rhinoplasty is a closed technique aiming to address the anatomical differences associated with the unilateral cleft nose, in order to reduce stigma during childhood, while minimising the risk for secondary deformity.14,15 Elements of the primary rhinoplasty include caudal septal dissection and repositioning without suture fixation, closed nasal tip dissection, internal nasal valve plication, and alar transfixion stitches. Silicone nasal stents, stitched in place for six weeks, aim to improve alar shape and reduce risk of nostril stenosis.16

The markings for bilateral cleft lip reconstruction follow the principle of recruiting white roll and vermillion tissue from the lateral lip elements, as described by David Chong17 (technique influenced by John Mulliken and David Fisher).18,19 The strategy for asymmetric bilateral cleft lip reconstruction is to favour a complete primary bilateral lip reconstruction in infancy, with the aim of achieving tissue balance, as influenced by David Fisher.20 The approach to primary rhinoplasty mirrors the principles described for the unilateral phenotype, with the exclusion of septal dissection/repositioning and with the addition of an alar cinch stitch to set the alar bases.

If an alveolar cleft is present, reconstruction with an autologous alveolar bone graft (ABG) will usually be performed at 8–11 years of age, based on dental development during mixed dentition.21 Patients will be jointly assessed at 7–8 years of age by the cleft surgeon, orthodontist and paediatric dentist to determine the ideal timing for the ABG. Pre-operative orthodontic expansion will be used if surgical access is required or to improve arch alignment.22,23 The extraction of erupted cleft-adjacent teeth will occur intra-operatively at the time of alveolar bone grafting due to weak evidence to suggest a clinical benefit for extraction in advance and to reduce the burden of additional anaesthetic episodes.24,25 Bilateral clefts of the alveolus will preferentially be reconstructed in a single stage; however, if palatal fistulae are present (typically Pittsburgh 5/6 fistulae), bilateral alveolar clefts will be reconstructed in two stages to increase the likelihood of definitive fistulae closure and reduce the risk of premaxillary necrosis.

Cleft palate only

Single-stage reconstruction of a cleft palate at eight months of age aims to achieve a balance of promoting optimal speech outcomes before the onset of babble,26 while minimising harm to maxillary growth.5,8 The intravelar veloplasty technique using the operating microscope, as described by Brian Sommerlad,27,28 will incorporate the palatal portion of tensor aponeurosis with the en bloc velar muscle dissection to provide fibrous tissue bulk for muscle approximation. Emphasis will be placed on radical muscle retropositioning, leaving the levator sheath in place on the nasal mucosal layer. There will be a low threshold for lateral relieving incisions where the cleft distance at the hard-soft junction is more than or equal to 10 mm, and buccal fat pads will be used if the nasal mucosa has been inadvertently perforated or lateral relieving incisions have been used.29 In wide clefts involving the hard palate, the hard palate will be closed in two layers, using vomer flaps anteriorly and sphenoid flaps posteriorly to assist with closure of the nasal layer.30

Submucous cleft palate

The decision to operate will be based on multidisciplinary assessment and the presence of velopharyngeal symptoms. The presence of an overt submucous cleft palate may warrant surgical reconstruction before the development of velopharyngeal symptoms, judged on a benefit-risk profile for each patient, carefully discussed with parents. The surgical technique will incorporate an intravelar veloplasty in association with double opposing Z-plasties in both the oral and nasal layers, with an aim of achieving both velar muscle repositioning and palatal lengthening.31

Unilateral cleft lip and palate

In line with the widely adopted surgical approach for unilateral cleft lip and palate in the UK, a sequence of two stages for primary closure of the cleft lip and palate in infancy will be used (see Table 1), followed by ABG in the mixed dentition.32,33 The first stage at four months of age will consist of unilateral cleft lip reconstruction with complete nasal floor closure and vomer flap.34,35 The aim of the vomer flap is to make the soft palate reconstruction easier, reducing the prevalence of symptomatic fistulae and to provide a good foundation for mixed dentation ABG.36,37 The second stage, at eight months of age, will consist of a completion palatoplasty with the intravelar veloplasty technique, as described for the cleft palate-only phenotype. The third planned stage of ABG will occur during mixed dentition at 8–11 years of age.

Table 1 Operative sequence for babies born with unilateral cleft lip and palate in the first year of life
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Bilateral cleft lip and palate

In the absence of a national consensus of surgical sequence for bilateral cleft lip and palate,38 a three-stage approach for the primary closure of cleft lip and palate will be used in the first year of life (see Table 2), followed by ABG in mixed dentition. The first stage at three months will be a lip adhesion and unilateral vomer flap.39 This stage will allow the dissection, release and repositioning of the orbicularis oris muscle to the edge of the prolabium without overstretching the myofibrils across the premaxilla. The unilateral vomer approach is safe and minimises vascular compromise to the premaxilla and the development of complex Pittsburgh 5 fistulae, which are common in BCLP.40,41 The second stage, at five months, will be a definitive bilateral lip reconstruction and contralateral vomer flap. The ‘delayed' orbicularis muscle can now be brought across the pre-maxilla with minimal tension. The third stage, at eight months, will consist of a completion palatoplasty, with the intravelar veloplasty technique, as described for the cleft palate-only phenotype. ABG will occur in one or two stages during mixed dentition at 8–11 years of age, dependent on the status of tissue healing following infant surgery.

Table 2 Operative sequence for babies born with bilateral cleft lip and palate in the first year of life
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Velopharyngeal dysfunction surgery

Patients with velopharyngeal dysfunction (VPD) will be assessed in a multidisciplinary setting and undergo both perceptual speech assessment and instrumental assessment (lateral videofluoroscopy and/or nasendoscopy). There will be an open acknowledgement of complexity and lack of consensus regarding optimal surgical options in VPD42 (both cleft and non-cleft VPD), with unreliable outcomes, potential to cause harm (i.e., sleep apnoea, mixed hypo/hypernasality, decreased clearance of secretions) and need to consider non-surgical options. There will be an overarching principle of first doing no harm (primum non nocere).

If surgical intervention is indicated, a palate-first strategy, incorporating radical muscle retropositioning and double opposing Z-plasties,31 will be advocated for mobile palates with an anterior velar muscle position and a small gap. A sphincter-type pharyngoplasty, using bilateral superiorly based myomucosal flaps inset side-to-side into the posterior pharyngeal wall, will be considered for mobile palates and a moderate gap.43,44 Bilateral buccinator myomucosal flaps will be considered for short mobile palates with a large gap.45,46,47 A posterior pharyngal flap48 will be considered as a first-line strategy for VPD in the setting of an immobile palate and for patients born with 22Q1.1 deletion.49 Pre- and post-operative sleep studies will be arranged for patients undergoing potentially obstructing pharyngeal surgery.

Peri-operative pathways

A single antibiotic dose on induction will be used as per the national guidance of cleft surgery being categorised a clean-contaminated procedure.50 Three post-operative intravenous doses of antibiotics will be given following ABG procedures, due to the most common risk of bone graft failure being wound dehiscence and infection.51 Post-operative antibiotic prophylaxis will not be planned for any other elective cleft procedure.52,53,54,55,56,57 A single airway dose of dexamethasone 250 micrograms/kg on induction will aim to reduce airway swelling. For palatal and pharyngeal procedures, three post-operative doses of dexamethasone will be administered.58,59,60 A single dose of tranexamic acid 10 mg/kg on induction will aim to reduce intra- and post-operative bleeding.61,62,63 As part of an enhanced recovery pathway strategy,64 pre-incisional regional nerve blocks will be used, including the infra-orbital nerve block for lip and alveolar procedures,65 and the suprazygomatic maxillary nerve block for palatal procedures.65,66

Discussion

The opportunity to design a cleft surgery pathway at the start of a surgical career is a privilege; however, the multitude of decisions to be made can be overwhelming, especially because the outcomes of the pathway will only be realised many years later. Early outcomes at five years of age are a useful indicator, but only at the end of facial growth at approximately 20 years of age will results be fully appreciated. It is incumbent upon cleft surgeons to have a clear rationale for the surgical pathway decisions they have decided upon and a strategy of how to assess the effectiveness of decisions made.

Working within a single-surgeon service, it will be imperative to adhere to a robust strategy of outcome evaluation and both internal and external validation (see online Supplementary Information). In the UK, the national Cleft Registry and Audit NEtwork (CRANE) provides quality assured benchmarking of outcomes at a cleft service level, but due to a limited number of multidisciplinary outcomes reported, the CRANE database is currently not sufficient alone for comprehensive outcome evaluation at an individual surgeon level. Consecutive evaluation of surgical outcomes over time is required, with notable examples in the literature acting as an inspiration to aspire to.67,68 For internal validation, I will use cumulative summation techniques for performance monitoring69 and will engage with audit and quality improvement processes. For external validation, I will seek structured, supportive peer review through engagement with the Surgical Clinical Excellence Network mentoring and coaching schemes, complex case discussion forums and annual visits to external (national and international) cleft units.

I am extremely grateful for the support I have received from colleagues, patients and my family to reach this long-standing dream position of becoming a cleft surgeon. I hope my decisions for the cleft surgery pathways described will serve my patients, their families and the Welsh Cleft Team well. Furthermore, it is the hope that this contribution will ignite debate and encourage open publishing of cleft surgery pathways and outcomes going forward.