Evolving consensus in cleft care guidelines: Proceedings of the 13 th annual conference of the Indian society of cleft lip palate and craniofacial anomalies

AK Singh; Divya Narain Upadhyaya; V Kumar; B Mishra; V Prasad

doi:10.4103/2348-2125.137897

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ORIGINAL ARTICLE

Year : 2014 | Volume : 1 | Issue : 2 | Page : 88-92

Evolving consensus in cleft care guidelines: Proceedings of the 13 ^th annual conference of the Indian society of cleft lip palate and craniofacial anomalies

AK Singh, Divya Narain Upadhyaya, V Kumar, B Mishra, V Prasad
Department of Plastic Surgery, King George Medical University, Lucknow, Uttar Pradesh, India

Date of Web Publication

2-Aug-2014

Correspondence Address:
Dr. Divya Narain Upadhyaya
Department of Plastic Surgery, King George Medical University, B-2/128, Sector-F, Janakipuram, Lucknow - 226 021, Uttar Pradesh
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/2348-2125.137897

Abstract

Introduction: The multi-disciplinary approach to cleft care has been a reality since the beginning of the last century, but there is a paucity of literature discussing or recommending specific cleft protocols. This is understandable due to the significant difference in cleft protocols around the world and the controversies surrounding each of them. Material and Methods: The Indian Society of Cleft Lip Palate and Craniofacial Anomalies in its 13 ^th Annual Conference discussed threadbare the different protocols around the world and propose a guideline to Indian surgeons delivering cleft care. Results and Discussion: These guidelines though not binding, are supposed to be pointers to a generally accepted standard for cleft care, considering the unique circumstances and limitations of cleft care providers in developing countries like India. It is a "best practice" indicator which, if adhered to by all the cleft care providers, will soon bring about uniformity in cleft care deliverance and allow us to evaluate our results on a much larger scale than has hitherto been possible.

Keywords: Cleft surgery, protocol, recommendations

How to cite this article:
Singh A K, Upadhyaya DN, Kumar V, Mishra B, Prasad V. Evolving consensus in cleft care guidelines: Proceedings of the 13 ^th annual conference of the Indian society of cleft lip palate and craniofacial anomalies. J Cleft Lip Palate Craniofac Anomal 2014;1:88-92

How to cite this URL:
Singh A K, Upadhyaya DN, Kumar V, Mishra B, Prasad V. Evolving consensus in cleft care guidelines: Proceedings of the 13 ^th annual conference of the Indian society of cleft lip palate and craniofacial anomalies. J Cleft Lip Palate Craniofac Anomal [serial online] 2014 [cited 2023 Mar 30];1:88-92. Available from: https://www.jclpca.org/text.asp?2014/1/2/88/137897

Introduction

The multi-disciplinary approach to cleft care has been a reality since the beginning of the last century, but there is a paucity of literature discussing or recommending specific cleft protocols. ^[1] This is understandable due to the significant difference in cleft protocols around the world and the controversies surrounding each of them. Additionally there has been a singular lack of studies evaluating the long-term effects of specific protocols and thus either recommending or repudiating them. Several studies like the one from the Australian Craniofacial Unit in Adelaide are remarkable but suffer from lack of significant numbers. ^[2] A literature search on PubMed with the keywords "cleft, protocol, guidelines, timing of surgery" returned no studies, which would "recommend" a specific protocol.

A visit to the website of the American Cleft Palate Association www.acpa-cpf.org led us to the "parameters for evaluation and treatment of patients with cleft lip/palate or other craniofacial anomalies (CA)" ^[3] which laid out the guiding principles for deliverance of care for children afflicted with cleft of the lip and palate. It also has references to treatment of other CA, maxillofacial surgery, otolaryngologic care, pediatric care, nursing care etc., and serves as a reference guide to professionals and lay persons informing them of the scope and standard of care that is expected to be delivered by cleft care providers in America. The document provides indicative timelines for cleft lip and cleft palate repairs without dwelling into the details of either timing or technique. It also loosely mentions other related issues in cleft care like speech, hearing assessment, fistulae, rhinoplasty and orthognathic surgery and outlines the need for these and the interventions that maybe required for complete management of the patient.

A similar document is being prepared by the European Cleft Organization ^[4] (www.ecoonline.org) and is expected to "address the huge inequality in cleft care across Europe by the development of an informative document that can be used by those countries where national protocols need to be established."

The section for professionals on the British cleft lip and palate association's website (www.clapa.com) however revealed no such guidelines ^[5] or parameters for the professionals to follow thus bolstering our belief that protocol recommendations are scarce and more so in the developing countries across the world.

The Indian Society of Cleft Lip Palate (ISCLP) and CA is a unique organization which was formed a decade and a half ago to ensure deliverance of quality care to the patients afflicted with cleft lip, palate and other CA. The organization through its annual meetings also endeavors to push the frontiers of research in the area of cleft and CA. The organization is a unique coming together of specialists from various streams of medicine, viz., plastic surgery, maxillofacial surgery, otorhinolaryngology, orthodontia and speech pathology for the sole purpose of cleft care. In its 13 ^th Annual Conference held at Lucknow, the organization tried to find common ground among the different protocols being practiced throughout the country and to incorporate enough flexibility in these protocols to allow for a unique, demanding and often restricting conditions imposed on the cleft care providers in the developing world today.

Materials and Methods

True to the theme of the conference "evolving consensus in cleft care," the conference had organized two panel discussions on two successive days to discuss the different cleft protocols being followed by cleft surgeons in India and across the world. The advantages and disadvantages of all the protocols were discussed with relation to outcome as measured by correction of deformity with regards to facial growth, correct speech, unimpaired hearing and aesthetic and psychosocial well-being. The panelists also discussed the peculiar circumstances, needs, shortcomings and patient demands in this part of the world and how to design "ideal" protocols to suit these requirements without compromising on the overall deliverance of quality cleft care.

The topics of discussion for the two panels were laid out concisely to circumvent wayward discussion and maximize the output in clear, quantifiable terms. Cleft care as provided to the patient throughout his/her life was broken down in subsets to allow focused, material debate on specific issues. The role of the coordinator was to keep the debate from losing its way into personal opinions, subjective experiences and biased approaches and remind the panelists to debate the pros and cons of a certain approach keeping in mind the prevailing circumstances in the country and the targeted outcome. A list of topics allotted to the panels is furnished herewith:

Panel 1
1. Use of distal femoral osteotomy (DFO)/presurgical orthopedics (NAM/Latham/palatal plate/taping/elastic bonnet)
  1. Is it required or not?
  2. Which device is best?
2. Lip repair
  1. Timing in Indian context,
  2. Extent of surgery (lip only/lip + gingivoperiosteoplasty (GPP)/lip + anterior palate/lip + nose)
3. Palate
  1. Timing?
  2. Palate first in some patients?
  3. Whole in one?
  4. Radical muscle dissection?
  5. Type of palate repair vis-à-vis width of the cleft?
  6. Pharyngoplasty in late presenters?
4. Arterial blood gas
  1. Timing?
  2. Preparation of arch?
  3. Expansion?
  4. Bone graft to nasal floor/para-pyriform area?
Panel 2
1. Palatal fistula
  1. Address the arch first or fistula first?
2. Velopharyngeal insufficiency
  1. Diagnosis - role of investigations in diagnosis?
    1. Naso-endoscopy?
    2. Ceph?
    3. Videofluoroscopy?
    4. Barium?
    5. Nasal manometry?
  2. Decision regarding type of surgery vis-à-vis type of velopharyngeal insufficiency (VPI)?
3. Early rhinoplasty in adolescents?
4. Orthognathic surgery
  1. Timing vis-à-vis skeletal maturity?
  2. Role of preoperative arch correction/orthodontia? Is it necessary?
  3. Surgical advancement versus distraction?
  4. VPI first or orthognathia first?

The list of topics was furnished to the coordinator and was supposed to act as a guidance to help the coordinator formulate a roadmap for steering the discussions. As is evident from the list, the topics covered were exhaustive and tailored for certain peculiarities of cleft care practice in the Indian context.

Results

Two panel discussions and several private conversations later the society succeeded in fleshing out a common minimum guideline, which can be used as a reference point on the path towards inclusive and comprehensive cleft care in the Indian context. The guidelines are not binding and do not claim to cover all the finer points of cleft care, of which there are innumerable.

guidelines thus issued from the conference are as below:

Role of DFO/presurgical orthopedics
1. Not mandatory
2. Helpful in bilateral cleft lip and palate with extreme protrusion of the premaxilla
3. May be used if social mores and economic conditions permit
4. Least invasive measure is best
  1. NAM/palatal plate
  2. Taping/elastics have limited benefit.
Primary surgery
1. Lip repair
  1. 3-6 months of age provided baby is medically fit and postoperative care is good
  2. Else postpone till after palate or when baby is strong enough to withstand surgery
  3. Preferably repair anterior palate with lip
  4. No consensus on primary rhinoplasty
  5. Primary bone graft/GPP not recommended.
2. Palate repair
  1. 6-9 months
  2. Hard and soft palate together
  3. Intra-velar veloplasty is a must
  4. Radical muscle dissection - optional. Requires training and long learning curve, greater fistula rate
  5. Aim for fistula free closure before 12-18 months
  6. Complete cleft palate - two flap palatoplsty
  7. Cleft soft palate/submucous cleft palate - Furlow's can be done
  8. Primary pharyngoplasty not recommended.
Arterial blood gas
1. Before descent of the canine into the cleft, depending on the intra-oral X-rays and in consultation with an orthodontist, if possible in the prevailing scenario
2. After preparation of arch by orthodontist, if available
3. Confirm absence of fistula
4. Can be combined with lip revision, nasal floor and para-pyriform augmentation.
In presence of palatal fistula
1. Correct arch first, then close fistula.
Velopharyngeal insufficiency
1. Age of assessment/surgery 6-7 years
2. Nasendoscopy if available should be done preoperative/postoperative for evaluation and documentation
3. Type of surgery depends on nasendoscopy findings
  1. A-P discrepancy - Palatal lengthening/pharyngeal flap
  2. Lateral weakness - Sphincter pharyngoplasty.
Orthognathic surgery
1. Definitive at 16-18 years of age
2. Earlier surgery can be contemplated (6-10 years) in case of severe maxillary retrusion, causing significant deformity and functional and psychosocial disturbance
3. Distraction to be contemplated if
  1. Advancement needed is more than 10 mm
  2. If there is significant soft tissue scarring and
  3. If the bone stock is not good.
Othognathic surgery versus speech
1. Ideally orthognathic surgery should precede speech surgery
2. If VPI correction has already been done - contemplate anterior segment advancement alone to minimize speech disturbance.
Orthognathia in presence of palatal fistula
1. If orthodontia is needed - expand the arch, close the fistula then contemplate orthognathic surgery
2. If orthodontia not needed - close fistula then proceed with orthognathic surgery.
Rhinoplasty
1. Primary septal correction can be contemplated at the time of lip repair (surgeon's preference)
2. 5-6 years of age - limited rhinoplasty may be contemplated in presence of gross deformity/concerned patient/parent, peer pressure and psychological needs
3. Definitive rhinoplasty - at 16-18 years of age/after orthognathic surgery has been performed.

Discussion

The search for the ideal cleft protocol continues and while there have been several studies evaluating cleft treatment protocols ^{[6],[7],[8],[9],[10]} there have been few that have either recommended or refuted one. The first such remarkable study was the Clinical Standards Advisory Group Cleft Lip and Palate Study in the United Kingdom. ^[6] The study examined two cohorts of children (5 years olds and 12 years olds) treated by 57 active cleft teams at that time in the United Kingdom. Over a period of 15 months from March 1996 to May 1997. The results of the study were an eye-opener for many as it revealed many shocking findings regarding the status of cleft care at that time in the United Kingdom. Of the 57 cleft teams only 36 could provide basic data like patient's names and 37-39% of the two groups of children examined had poor dental arch relationships as measured by the GOSLON index or the 5 years old index. "Seventy percent of the 12 years old patients had a skeletal Class III relation and 42% of bone graft were either seriously deficient or failed." The results shocked the committee into making serious recommendations for re-organization of cleft services in the country and for reducing the cleft units from 57 to 8-15 and for adapting the "hub and spoke" services model where the core team and specialist equipment is located at the "hub" but services may be provided for follow-up at the "spoke" thus reducing the inconvenience to the patient. The "hub" is also the coordinating center for record keeping and audit. This study, though it led to sweeping changes in the organization of cleft care in the United Kingdom, did not address the more "scientific" issues like a cleft protocol or timings and technique of various surgical interventions.

The Eurocleft Project 1996-2000 was a follow-up of the original Eurocleft study (1992). "The original Eurocleft project, a European inter-center comparison study, revealed dramatic differences in outcome, which were a powerful stimulus for improvement in the services of respective teams. ^[7]" The follow-up project known as "standards of care for cleft lip and palate in Europe: Eurocleft" ran between 1996 and 2000 and found that of the 201 centers that registered with the network, 194 followed differing protocols in treating the unilateral complete cleft of the lip and palate. The project report admitted that "attainment of even minimum standards of care remains a major challenge in some communities, and both the will to reform and basic strategy to follow are overdue." The report concluded that cleft services, treatment and research had suffered from a lack of uniformity and haphazard development of cleft care across Europe. However, even the Eurocleft project could not recommend specific technical protocols and timing of specific interventions as it found that these choices were highly controversial, and cleft care practices varied to a great extent across various centers in Europe.

An example of the diversity in cleft practices can be had from the finding of the above study where it was found that the centers participating in the study practiced seventeen possible sequences of the operation to close the unilateral cleft of the lip and palate. However, the majority (42.8%) closed the lip at the first operation and the hard and soft palate together at the second operation. Majority of the teams (65%) also practiced presurgical orthopedics, and most of these (70%) were by passive palatal appliances. This is not unlike the recommendations of the Indian society, which have been enumerated above.

The study enumerated the difficulties in achieving optimal standards of care across Europe and mentioned personal egotism of individuals and competition between different specialties as some of the reasons why standardization of cleft care was such a difficult job.

Changes in the cleft care scenario in the United Kingdom could be possible only due to the intervention of the government, but even the Clinical Standards Advisory Group, or the Eurocleft could not get the surgeons to agree to a specific protocol for treating cleft patients in their respective countries/regions. Such is the diversity of the protocols and difficulty in generating consensus in cleft care, which the consensus committees (Panel Discussion Committees) of the ISCLP and CA had to face.

Several centers (or countries) have taken cognizance of the changing scenario in cleft care and have woken up to evaluate their own protocols or subject them to trials to try and find the best suited protocol for cleft care. Notable among these are the Americleft study ^[9] and the study from the cleft group in Switzerland. ^[10]

The cleft group in Basel, Switzerland, after having evaluated the results of their cleft protocol and compared it with the results of the Eurocleft project decided to abandon primary alveolar bone grafting as they found that it led to inconsistent alveolar ossification and interfered with anterior maxillary growth. They also concluded that minimal (as opposed to maximal) stabilization of alveolar segments leads to a favorable occlusion and hence they have abandoned the use of palatal obturators to keep the palatal shelves in place. This is an example of self-evaluation and course correction that is being seen increasingly across the world. As a result of such studies cleft groups, associations and countries are taking note of their protocols and trying to evaluate and change the protocols accordingly. Cleft registries are being created in Belgium, Romania, Ukraine, Switzerland; cleft associations are being formed and the unification of services in Hungary, Netherlands and the Slovak Republic has seen a reduction in the number of centers dealing with clefts. ^[7]

In India a national survey on the management of cleft lip and palate was done from May 2006 to September 2007. ^[11] The study, the first if its kind in India, revealed that most of the cleft surgeons in India were repairing the cleft of the lip between 3 and 6 months of age and the palate at 6 months-1 year. Seventy-three percent of cleft surgeons in India did not use presurgical orthopedics and around as many did not advocate lip adhesion as a preliminary procedure. The respondents to this survey claimed to perform approximately 3200-34,700 cleft surgeries annually amongst themselves, which is a far greater number than reported anywhere in the literature till the publication of this study. ^[11]

Some Indian cleft surgeons have also advocated reversing this popular, unwritten rule of lip repair at 3 months and palate repair at 9 months ^[12] and have proposed a modified surgical schedule for managing clefts of the lip and palate in developing countries like India. The paper makes an interesting read but has, unfortunately, not found many takers among the cleft fraternity in India. These and many such studies referring to the peculiar circumstances facing the Indian cleft surgeons were discussed during the deliberations of the panel of the present study.

The ISCLP and CA is a nearly decade and half old organization and in its 13 ^th Annual conference had taken upon itself the onerous task of building a consensus draft for a cleft protocol. Such a cleft protocol which is now being published by the society is not binding but should serve as a guideline according to the consensus reached among the different specialists involved in cleft care in India. It is a "best practice" indicator which, if adhered to by all the cleft care providers, will soon bring about uniformity in cleft care deliverance and allow us to evaluate our results on a much larger scale than has hitherto been possible.

References

1.	Hurwitz DJ. Unilateral cleft lip and nose. In: Bardach J, Morris HL, editors. Multidisciplinary Management of Cleft Lip and Palate. Philadelphia: Saunders; 1990. p. 204-16.
2.	Schnitt DE, Agir H, David DJ. From birth to maturity: A group of patients who have completed their protocol management. Part I. Unilateral cleft lip and palate. Plast Reconstr Surg 2004;113: 805-17.
3.	Available from: http://www.acpa-cpf.org/uploads/site/Parameters_Rev_2009.pdf.
4.	Available from: http://www.ecoonline.org/en/european_standards/.
5.	Available from: http://www.clapa.com/pros/.
6.	Sandy J, Williams A, Mildinhall S, Murphy T, Bearn D, Shaw B, et al. The Clinical Standards Advisory Group (CSAG) cleft lip and palate study. Br J Orthod 1998;25:21-30.
7.	Shaw WC, Semb G, Nelson P, Brattström V, Mølsted K, Prahl-Andersen B, et al. The Eurocleft project 1996-2000: Overview. J Craniomaxillofac Surg 2001;29:131-40.
8.	Semb G, Brattström V, Mølsted K, Prahl-Andersen B, Shaw WC. The Eurocleft study: Intercenter study of treatment outcome in patients with complete cleft lip and palate. Part 1: Introduction and treatment experience. Cleft Palate Craniofac J 2005;42:64-8.
9.	Russell K, Long RE Jr, Hathaway R, Daskalogiannakis J, Mercado A, Cohen M, et al. The Americleft study: An inter-center study of treatment outcomes for patients with unilateral cleft lip and palate part 5. General discussion and conclusions. Cleft Palate Craniofac J 2011;48:265-70.
10.	Mueller AA, Zschokke I, Brand S, Hockenjos C, Zeilhofer HF, Schwenzer-Zimmerer K. One-stage cleft repair outcome at age 6- to 18-years - A comparison to the Eurocleft study data. Br J Oral Maxillofac Surg 2012;50:762-8.
11.	Gopalakrishna A, Agrawal K. A status report on management of cleft lip and palate in India. Indian J Plast Surg 2010;43:66-75. [PUBMED]
12.	Agrawal K, Panda K. A modified surgical schedule for primary management of cleft lip and palate in developing countries. Cleft Palate Craniofac J 2011;48:1-8. [PUBMED]

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