Global Cleft Care in Low-Resource Settings

By David W. Low

Cleft lip and palate represents the most common congenital facial anomaly. Congenital anomalies are one of the top 10 causes of disability and suffering in many low and middle income countries (LMICs.) Further, an estimated 30% of the global burden of disease is treated primarily through surgery, and among surgical conditions, treating clefts conveys disproportionately large benefit relative to cost because suffering can be halted at a young age and deliver a lifetime of health. Over the last 50 years, cleft care has advanced in low-resource settings through international surgical missions and the efforts of sparse local providers. While exciting anecdotes of impact abound from these efforts, recent studies have identified a complication rate that often exceeds 50% and there is concern about the sustainability of foreign volunteer missions.

This book serves as a thorough resource for providing medical and surgical care of cleft lip and palate deformities in low-resource settings, geared to the context of those settings. The content is consensus-based and evidence-based, with emphasis on treatment that is appropriate to a low-resource context and how this differs from that in higher-resource environments. It is authored by recognized cleft experts in close partnership with providers who practice in low-resource settings. It is heavily pictorially based through illustrative figures, photos and diagrams, to be facile for those who speak English as a second language.  

Following the introduction, Complete Cleft Care in Low-Resource Settings has six main sections: patient workup, surgical treatment, comprehensive care, educational innovation, care delivery models, and finally a series of illustrative case studies. Uniquely, the book’s breadth encompasses cleft care comprehensively, but does so through a practical “how to” format with many “from the field” examples of what has worked. 

• Language: English
• Publisher: Springer
• Release date: Apr 5, 2021
• ISBN: 9783030591052

Book preview

Part IIntroduction to Global Cleft Care

© Springer Nature Switzerland AG 2021

J. W. Swanson (ed.)Global Cleft Care in Low-Resource Settingshttps://doi.org/10.1007/978-3-030-59105-2_1

1. Introduction: A Paradigm Shift in Global Cleft Care

Jordan W. Swanson¹  

(1)

Division of Plastic Surgery, Children’s Hospital of Philadelphia and University of Pennsylvania, Philadelphia, PA, USA

Jordan W. Swanson

Email: [e-mail]

Keywords

Cleft careCleft surgeryLow-resource settings (LRS)Low- and middle-income countries (LMICs)SustainabilityField innovationSurgical educationBarriers to care

Key Points

Major evolution in global cleft care is being driven by five exciting—but challenging—forces.

1.

Surgery is increasingly seen as a critical but underdeveloped component of public health delivery in low-resource settings, and cleft surgery is an important potential bellwether to mediate this shift.

2.

Locally delivered cleft care offers many advantages, but creates a systemic transition from the international missions that were prominent in early outreach efforts.

3.

Patient-perceived outcomes of good function and appearance are the ultimate goals of cleft care, and employing outcome measures in these settings is critically important.

4.

Innovation borne out of low-resource settings can be nurtured by bringing voice and tools to emerging leaders in these settings.

5.

The evidence base for cleft surgery needs to be inclusive of low-resource settings, which often yield different findings than high-resource settings.

Lizmarlin was 4 years old when we first met, in the stale, humid air of a clinic room attached to the Esteban Serrano Hospital, in the Caribbean jungle of eastern Nicaragua. Cowering behind a door in the room, tears streamed down her cheeks and onto her pink dress, her nicest one, which had become dusty during the 8-hour walk and bus ride to our clinic. Each half of her full upper lip was splayed widely to the side, and mucous poured from the wide cleft in her lip and palate. Though this was the furthest that she had ever been from home, she was still another 10 hours by bus away from the capital city of Managua, where her cleft could be surgically repaired. The exhausted eyes of her young mother, sitting with us, revealed stoicism and love, but also divulged uncertainty about her daughter’s future.

Lizmarlin’s story is different from those of the children born with clefts whom I meet at the Children’s Hospital of Philadelphia. In Philadelphia, I often meet them for the first time while they are still in the womb, parents tipped off to the pending cleft by a prenatal ultrasound. These parents can count on successful lip and palate repairs to be completed by a year of age, with their child likely to begin to smile, speak, and sing shortly thereafter.

In truth, Lizmarlin’s story was graced with some good fortune. She was spotted by a coffee buyer, one of many who logs regular travel through rural agricultural regions and are trained up through an inspired effort of the Nicaraguan government and Operation Smile to identify children with clefts and inform their families of available treatment. And her family surmounted a number of barriers common to low-resource settings; Lizmarlin’s mother sought out and managed to reach care for her daughter. In high-resource settings, an orofacial cleft is not thought to be a fatal disease, but Smile Train data from low-resource settings suggest that fewer than half of children with clefts in some regions survive to their fifth birthday—due to a combination of malnutrition, aspiration pneumonia, and purposeful neglect [1]. In these ways, a cleft lip and palate exemplify health inequality in the modern world.

As a young surgeon living and working in Nicaragua, I struggled to understand the pathophysiology of clefts and their optimal care in the setting of poverty and a constrained health system. Was it appropriate to perform a palate repair in an older patient? How had infection led to dehiscence of a bilateral lip repair, something I had not seen before? Despite being trained by outstanding cleft surgeons at leading programs in the United States, the answers to these questions and others—the widely ranging cleft patterns and severity, and patient ages and comorbidities—exceeded my clinical experience and training.

Most challenging was caring for patients who had been left with poor outcomes from previous surgery. Specifics of the surgery were often unknown to the family or health system. Facing children stigmatized by appearance and function, their faces and mouths severely scarred, often elicited a sense of duty to bring mercy, even as we vowed not to repeat past mistakes that were not always evident. I also saw the consequences of well-intentioned efforts by well-trained surgeons from afar: for instance, the teaching of a Furlow palatoplasty under loupe magnification to young surgeons who did not have loupes, and who then struggled in subsequently emulating the technique. I know there were similar consequences resulting from my own efforts, and even others which I did not fully appreciate.

Fortunately, a growing cadre of specialists who care for children with clefts in these settings are marshaling keen observation, intuition, and problem-solving to significantly improve paradigms of care. Doctor Armando Siu, my senior partner in Nicaragua, has honed a remarkable surgical instinct over three decades of practice, deploying adroit maneuvers so that surgical repairs better stand up to the realities of poor hygiene, sugary diets, and irregular follow-up. Nurse Tatiana Morales, a fierce patient advocate, set up my visit with Lizmarlin in the rural hospital and as a patient coordinator meticulously tracks patients throughout the country and devises creative ways of reaching out to them. Tatiana and other similarly committed doctors and nurses are among a passionate and inspiring band of cleft providers in low-resource settings who are quick to improvise to bring practical help to people in need (Fig. 1.1). They seem to share three common attributes: (1) they have sought out excellent clinical training, (2) they have committed themselves to serving vulnerable and marginalized cleft patient populations, and (3) they work to be data-driven in their decision-making, collecting data where possible and studying outcomes in settings in that are simply not addressed in the vast majority of existing academic literature.

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Fig. 1.1

Infant with a cleft lip and nasal deformity is treated with an improvised naso-alveolar molding device by an unknown dentist in a region without known trained cleft providers

These unsung problem-solvers are forming a growing fraternity of cleft providers around the world. Accessible technologies such as WhatsApp give voice to surgeons in remote settings, and cleft organizations and societies help to mobilize disparate providers. These innovators are now becoming thought leaders in their own right, and whose ideas are worth spreading. This book was both inspired and, importantly, authored by these scholar-pragmatists—practitioners who have made it their mission to deliver the best possible cleft care to all people of the world using methods appropriate to their realities and circumstances.

Just as low-resource settings vary dramatically in their unique and specific economic, technological, and cultural constraints and attributes, perspectives on traditional and emerging approaches to cleft treatment at times diverge. The approximately 100 authors who have contributed to this textbook hail from 29 countries, most of them constituting low-resource settings (Fig. 1.2). Great efforts have been taken to present multiple perspectives where they exist, and nurture evolving surgical techniques and approaches by practitioners in low-resource settings even as they contrast with traditional surgical techniques originating in quite dissimilar clinical settings. In identifying, curating, and reviewing these contributions to the academic literature on cleft repair, much credit is due to this book’s talented section editors, each of whom possesses a unique depth of experience as medical directors and leaders of cleft centers and outreach programs in these challenging settings.

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Fig. 1.2

Authorship of Global Cleft Care for Low-Resource Settings . (Map courtesy of Chris Kalmar, MD)

Five Forces Shaping Global Cleft Care

We find ourselves amidst an exciting but challenging time of major evolution in the field of global cleft care. This book was conceived and inspired in the hopes of better understanding of how we should address these changes, and it aims to squarely take on each one.

Surgery as Public Health

First, the potential of surgery to contribute to dramatic improvements in global health looms bright. Not so long ago, surgery was thought to be too expensive or complex to scale effectively in poorer countries—a service peripheral to public health. This viewpoint is being challenged by recent findings from the World Health Organization and The Lancet that more people die each year from surgically treatable conditions than HIV/AIDS, malaria, and tuberculosis combined [2, 3]. Surgically treatable diseases are among the top causes of disability in the world, particularly in low- and middle-income countries (LMICs) .

In fact, surgical treatment of many conditions, including cleft lip and palate, can be highly effective relative to investment costs; it is, for example, more cost-effective than anti-hypertensive medicines for cardiovascular disease or antiretrovirals for HIV/AIDS [2]. Especially in poorer settings, surgical treatment of a laborer’s injured hand or a child’s congenital deformity is a form of Surgical Justice , the concept that surgery imparts freedom even beyond the receiving patient to their family, community, and society [4]. Cleft surgery is well-positioned to be a global bellwether for this shift in thinking about surgery. Cleft care represents the largest sector of philanthropically funded global surgery [5], and a compelling argument can be made for diagonal development of a health system by scaling up cleft care [6]. It is a field with leadership potential to strengthen surgical systems in low-resource settings.

Locally Delivered Cleft Care

Second, a major transition is occurring in how cleft care is delivered in low-resource settings—and by whom. Many of the most visible early cleft outreach efforts were conducted by international volunteer teams who traveled from higher to lower income countries for 1–2-week surgical missions to places that had no cleft care providers. The power of reconstructing major visible congenital deformities through relatively quick, safe procedures is remarkable, bringing hope to communities unfamiliar with cleft treatment. Surgical missions also provide a rare forum for doctors, nurses, and therapists to work side by side, sharing ideas, and learning from each other.

Perhaps inspired by these outreach missions, a growing corpus of local providers have begun to develop local cleft teams and programs. Home-grown teams have important advantages over visiting clinicians, including the possibility of offering year-round care continuity to patients and an intimate familiarity with the nuances of local health system dynamics. Unfortunately, these programs are often challenged by limited financing, and surgeons and clinical teams frequently serve in unpaid volunteer capacities. Moreover, in places where such local efforts have taken root, they have also served to expose some of the downsides of the traditional international surgical mission model: difficulty in offering patient continuity and following outcomes, undermining the reputation of local surgeons and of local cultural and social practices, outsized travel and cargo transport expenses (often exceeding the cost of a local surgeon’s full-time salary for up to a year), and limited ability to offer ongoing dental, speech, and other multi-disciplinary care.

In response, many cleft organizations that started out solely as surgical mission providers are working to adapt their programs to this changing landscape. Building and investing in local cleft centers staffed by local teams, embracing an educational focus to clinical efforts, financing local teams, and drawing volunteer providers from other low-resource settings, are several tactics that groups such as Interplast (now ReSurge ), Operation Smile, and Smile Train have adopted in recent years. And there still remains an important role for international cleft missions, particularly in locations where properly trained staff is altogether scarce, such as to provide care in Madagascar (population 29 million, with 2 plastic surgeons) even as efforts to train local surgeons are underway.

Focus on Achieving Great Patient Outcomes

Third, the effectiveness of cleft care is increasingly being judged not by what is done to patients, but by what it enables in them. A simple count of total cleft surgeries performed cannot be the primary yardstick for success. Success might be better defined as a patient who feels socially and functionally integrated into society. In settings where patient follow-up is challenging and provider presence is intermittent, it is particularly important to ensure that impactful outcomes are achieved consistently. Several recent studies have reported that post-surgical complications such as dehiscence and fistula, which typically occur in fewer than 5% of cases in high-income settings, occur in an astonishing 25–56% of cases in LMICs [7–9]. Thus we have to be cautious not to proliferate care that is ineffective, or worse, harmful, in settings where factors of poverty, medical care, nutrition, environment, and culture may influence healing differently.

Moreover, publicizing a high number of procedures or care episodes that an organization performs or a patient receives can be problematic. Increased numbers can indicate reaching more patients with better care, but it can also signify the repetition of care episodes or surgical revisions that reflect inadequate solutions to the underlying problem. Patients and families seek the best possible function and appearance delivered through the fewest possible episodes of treatment. Therefore, quality of care is paramount. Refining and tracking robust outcome metrics—reflecting the breadth of speech, oral health, appearance, and self-confidence—are critical to these efforts, and this is an exciting area of rapidly evolving research discussed in Chap. 37.

Innovation Borne of Low-Resource Settings

Fourth, innovations in cleft care are increasingly being developed in low-resource settings. Poverty devises skills , proposed the Greek poet Theocritus. If necessity is the mother of invention, as his contemporary Plato wrote, it seems logical that new and practical solutions would arise in constrained settings. Further, in many cases, these innovations and improvisations may be well-suited to other low-resource settings.

Historically, factors such as low densities of providers, a lack of research infrastructure, and the high costs of prototyping were thought to pose structural barriers to innovation and sharing in these settings. More recently, however, technological advances in communication, online collaboration, and 3-D printing, among others, are enabling a more conducive ecosystem to emerge. Compared to high-income countries, less complex regulatory environments and health care delivery systems may actually reduce barriers to entry for these innovations, even as the rights and vulnerability of patients involved in these efforts must be respected. In paradoxical and exciting ways, some innovations originating in low-resource settings are spreading to high-resource settings, including nerve blocks, obturator techniques, and cleft severity and outcome tools. Innovations derived from low-resource settings may be particularly timely as healthcare costs soar in high-resource settings, and efficacious lower-cost alternatives are increasingly being sought.

To be sure, there remain many challenges in identifying new innovations and vetting their efficacy, which is particularly important when resources to scale up new innovations are limited. Good ideas and good intentions do not always lead to better outcomes. But when they do, they can dramatically alter our patients’ lives for the better, and the desire to find these innovations—regardless of their place of origin—unites us all.

The Conundrum of Evidence-Based Medicine

Finally, while we all seek to practice evidence-based medicine, how that applies to low-resource settings is often far from clear. Much of the evidence, if defined as scientific literature published in high impact journals, originates from high-income countries. Mounting examples suggest that when these techniques or protocols are re-deployed in low-resource settings, the results can be very different [10]. Where higher quality studies do exist, they can lead to different recommendations than in high-resource settings. For instance, the best US evidence supports no postoperative antibiotic use after palatoplasty [11]. However, a double-blinded, randomized controlled trial in India showed superior outcomes when a 5-day prophylactic oral antibiotic was used postoperatively [12].

In the absence of clear data, practice patterns around the world vary widely; decision-making is often guided by intuition and personal experience, and multiple approaches often seem reasonable. Throughout this textbook, we strive to seek out and present these multiple approaches. With time, it is quite possible that some aspects of cleft care will converge toward a single most optimal technique or protocol, whereas others will continue to see evidence supporting multiple approaches. Further, higher fidelity studies may show us how and why certain approaches are better in certain places, and this may depend on broad socioeconomic factors or specific epigenetic factors in ways that we are now only beginning to understand.

Dr. Bill Magee, the founder of Operation Smile and an early advocate for global cleft surgery, often remarks that the surgeon plays at most 10% of the part in helping to heal a child with a cleft. Indeed, a few moments before I repaired Lizmarlin’s cleft lip , a photographer took this photo (Fig. 1.3). Although she had not undergone any surgical care at this point, a transformation was already well underway. A pediatrician found that she was otherwise healthy, with good growth potential; a speech therapist had counseled her family that her speech would improve after surgery; a child life specialist was helping her self-confidence grow through interactive games; and a team of caring specialists was already at her side.

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Fig. 1.3

Lizmarlin, a 4-year-old girl in Nicaragua with unrepaired cleft lip and palate , 1 month after her family’s initial visit to a cleft surgeon and shortly before her cleft lip repair. (Photo courtesy of Inti Ocon)

Ingenuity and problem-solving are not unique to cleft repair nor to surgery in general—medical practitioners have always sought out the best possible solutions with the resources at hand. This textbook first seeks to honor that tradition by presenting the techniques of leading experts from around the world, and by doing so and illustrating them in such a way to be accessible by all global providers. But it further seeks to recognize that ingenuity is an innate part of the practice of medicine, and that improvisation and practical adaptation are not necessarily less than ideal or second-rate care. They are often the right care for the setting in which they are being performed, and this textbook seeks to elevate these practices to their rightful place in the literature—or at the very least, validate the ingenuity and pragmatism that their originators were facing in developing them. It finally seeks to pay tribute to the many talented people dedicated to caring for children with clefts in low-resource settings, as we endeavor to learn from each other to do better for our patients.

References

1.

Mossey PA, Modell B. Epidemiology of oral clefts 2012: an international perspective. Front Oral Biol. 2012;16:1–18.Crossref

2.

Meara JG, Leather AJ, Hagander L, Alkire BC, Alonso N, Ameh EA, Bickler SW, Conteh L, Dare AJ, Davies J, Mérisier ED. Global surgery 2030: evidence and solutions for achieving health, welfare, and economic development. Lancet. 2015;386(9993):569–624.Crossref

3.

World Health Organization. World health assembly resolution 68.15: strengthening emergency and essential surgical care and anaesthesia as a component of universal health coverage. 68th World Health Assembly. 2015; 27. Available at http://​apps.​who.​int/​gb/​ebwha/​pdf_​files/​WHA68/​A68_​R15-en.​pdf?​ua=​1.

4.

Morrison SD, Swanson JW. Surgical justice. Plast Reconstr Surg. 2015;136(2):291e–2e.Crossref

5.

Gutnik L, Dieleman J, Dare AJ, Ramos MS, Riviello R, Meara JG, et al. Funding allocation to surgery in low and middle-income countries: a retrospective analysis of contributions from the USA. BMJ Open. 2015;5(11):e008780. https://​doi.​org/​10.​1136/​bmjopen-2015-008780.CrossrefPubMedPubMedCentral

6.

Patel PB, Hoyler M, Maine R, Hughes CD, Hagander L, Meara JG. An opportunity for diagonal development in global surgery: cleft lip and palate care in resource-limited settings. Plast Surg Int. 2012;2012:892437.PubMedPubMedCentral

7.

Maine RG, Hoffman WY, Palacios-Martinez JH, Corlew DS, Gregory GA. Comparison of fistula rates after palatoplasty for international and local surgeons on surgical missions in Ecuador with rates at a craniofacial center in the United States. Plast Reconstr Surg. 2012;129:2. https://​doi.​org/​10.​1097/​prs.​0b013e31823aea7e​.Crossref

8.

Daniels KM, Yu EY, Maine RG, et al. Palatal fistula risk after primary palatoplasty: a retrospective comparison of a humanitarian organization and tertiary hospitals. Cleft Palate Craniofac J. 2016;55:807. https://​doi.​org/​10.​1597/​16-007.Crossref

9.

Rossell-Perry P, Segura E, Salas-Bustinza L, Cotrina-Rabanal O. Comparison of two models of surgical care for patients with cleft lip and palate in resource-challenged settings. World J Surg. 2015;39:47–53. https://​doi.​org/​10.​1007/​s00268-013-2395-9.CrossrefPubMed

10.

Deshpande G, Wendby L, Jagtap R, Schönmeyr B. The efficacy of vomer flap for closure of hard palate during primary lip repair. J Plast Reconstr Aesthet Surg. 2015;68(7):940–5.Crossref

11.

Rottgers SA, Camison L, Mai R, Shakir S, Grunwaldt L, Nowalk AJ, Natali M, Losee JE. Antibiotic use in primary palatoplasty: a survey of practice patterns, assessment of efficacy, and proposed guidelines for use. Plast Reconstr Surg. 2016;137(2):574–82.Crossref

12.

Aznar ML, Schönmeyr B, Echaniz G, Nebeker L, Wendby L, Campbell A. Role of postoperative antimicrobials in cleft palate surgery: prospective, double-blind, randomized, placebo-controlled clinical study in India. Plast Reconstr Surg. 2015;136:59e–66e.Crossref

© Springer Nature Switzerland AG 2021

J. W. Swanson (ed.)Global Cleft Care in Low-Resource Settingshttps://doi.org/10.1007/978-3-030-59105-2_2

2. Burden of Cleft Disease and Opportunity for Impact

Naikhoba C. O. Munabi¹   and Nivaldo Alonso²  

(1)

Division of Plastic and Reconstructive Surgery, Keck School of Medicine of the University of Southern California, Los Angeles, CA, USA

(2)

Faculty of Medicine of the University of São Paulo, Hospital das Clinicas at the University of São Paulo, São Paulo, Brazil

Naikhoba C. O. Munabi (Corresponding author)

Email: [e-mail]

Nivaldo Alonso

Email: [e-mail]

Keywords

Cleft definitions and epidemiologyCleft disease burdenSocioeconomic impact of cleft care

Key Points

Clefts cause both functional limitations on feeding and speech, and aesthetic consequences with profound psychosocial impact due to social stigma, which together lead to poor educational and employment opportunities as well as inability to achieve major life milestones.

The long-term effects of untreated cleft lip and palate lead to a large social and economic impact, thus cleft repair is considered an essential surgery that should be available to all people everywhere by the 2015 Lancet Commission on Global Surgery.

Cleft lip and palate can be caused by a genetic mutation, either inherited or spontaneous. Its frequency is also increased by environmental exposures often experienced before a young mother knows that she is pregnant.

Even in regions where primarily lip and palate repair can be accessed, there is often a large backlog of patients who suffer from untreated or inadequately treated sequelae of cleft lip/palate.

Cleft conditions disproportionately impact low-resource countries, where many additional social determinants and barriers may impact the ability of individuals to seek and receive appropriate care.

Increased focus on comprehensive delivery of cleft care in austere settings, and assuring adequate outcomes of care, has the potential to dramatically improve social and economic well-being, and demonstrate the transformative impact of surgical care in these settings.

Introduction

Cleft lip and/or palate is the most common congenital malformation of the head and neck worldwide with a prevalence of 0.5–1.6 in 1000 live births [1]. Cleft conditions can vary in appearance and severity from small incomplete clefts of the lip to wide bilateral combined cleft lip and palate. Clefts present both functional and aesthetic challenges. Functionally, clefts particularly of the palate can result in challenges with feeding and speech. Aesthetically, an atypical appearance due to cleft can have a profound psychosocial impact due to social stigma and lack of community acceptance, leading to poor educational and employment opportunities as well as inability to achieve major life milestones [2]. These long-term effects of cleft lip and palate can lead to a large economic impact, and it is this combined individual and economic impact that makes cleft repair one of the essential surgeries listed by the 2015 Lancet Commission on Global Surgery that should be available from either in country or visiting skilled providers [3].

In order to create systems that care for cleft conditions globally, we must understand their epidemiology and the impact such conditions have on communities, domestic and international society. Cleft conditions disproportionately impact low-resource countries, where many additional social determinants and barriers may impact the ability of individuals to seek and receive appropriate care. In this chapter, we review the nature and burden of cleft disease, and introduce options for cleft care delivery and their potential impact.

Defining Cleft Conditions

Orofacial clefts originate from a failure of fusion of the frontonasal and lateral processes during development [4, 5] (Fig. 2.1). This failure occurs early in the development process between weeks six and eight of gestation [6]. Therefore, in cases where environmental exposures are the suspected cause of cleft conditions, these exposures may occur prior to awareness of pregnancy, highlighting the need for more preventive global policies and interventions rather than those just targeting expectant mothers.

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Fig. 2.1

Embryonic development of the lip and palate in humans at the end of the (a) fourth, (b) fifth, and (c), sixth weeks of gestation. Disruption in movement and fusion of the frontonasal and maxillary processes during this time period can lead to cleft lip development. Primary and secondary palate fusion occur between weeks six and eight of gestation (d–f), with failures in fusion during this time period contributing to the development of cleft palate. (Reproduced from Dixon and colleagues [6] by permission of Nature Reviews)

The degree of disruption of the embryologic fusion process leads to the severity of the cleft condition, which can range from an incomplete unilateral cleft of just the lip to full bilateral cleft of the lip and palate. Studies by Yao et al. and Campbell et al. have graded the severity of cleft lip conditions on a 4-point scale, allowing for more universal language describing cleft lip presentations [7, 8] (Fig. 2.2). Similarly, classifications such as Veau and Kernahan aim to stratify clefts of the palate by either severity or component of the palate involved [9]; classifications are discussed further in Chap. 6.

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Fig. 2.2

The Cleft Severity Index . Criteria for each of the 4 grades of the cleft severity and associated examples. (Reproduced from Campbell and colleagues [7] by permission of Plastic and Reconstructive Surgery Global Open)

In addition to clefts occurring as a result of intrinsic impairment in facial process fusion, clefts can also occur when the palatal shelves are otherwise obstructed from fusion by an intervening structure. Such a phenomenon is the case in Pierre Robin Sequence (PRS) , where stunted mandibular growth leads to inability of the tongue to descend to the floor of the mouth resulting in the tongue physically obstructing palate fusion [10]. Despite being associated with multiple structural abnormalities, PRS can occur independently of a genetic cause, however is often seen in Stickler syndrome [10].

The Burden of Cleft Disease

Epidemiology

Cleft lip and/or palate (CL/P) is one of the four most common congenital defects in children, and is the most common congenital anomaly of the head and neck. It affects between 1 in 500 and 1 in 2500 children born worldwide [11]. In general, Asian and Amerindian populations have the highest reported birth prevalence rates, often as high as 1/500, European-derived populations have intermediate prevalence rates at about 1/1000, and African-derived populations have the lowest prevalence rates at about 1/2500 [6].

Males are affected twice as often as females and for every bilateral cleft, there are three times as many patients affected with a cleft on the right, and six times as many with a cleft on the left [12].

The variability in incidence is due to a combination of genetic predisposition and environmental factors. Approximately 30% of cleft patients have a diagnosed syndrome, suggesting a genetic cause of the cleft. For the 70% of patients who are identified as non-syndromic, factors such as family history, rarer genetic mutations, and environmental exposures likely each play a role in the frequency of cleft development [13, 14]. For example, in Brazil, the familial recurrence rate for CL/P is dependent on the region of the country, with some areas demonstrating a recurrence risk as low as 0.6–0.7% compared to other regions with a three- to four-fold higher risk (2.2–2.8%) [15]. In this population, IRF6 may be one such gene associated with non-syndromic CL/P, as has been seen in other ethnic groups [14, 16]. However, environmental factors may also account for the geographic discrepancy observed.

Folate deficiency is perhaps the most well-known environmental factor associated with CL/P. Due to the effect of folate deficiency on neural tube formation, fortification of grains with folic acid has been mandated in many countries. In the United States, where fortification of grains was mandated in 1998, a 6–12% reduction in cleft palate prevalence was observed over the course of 12 years [16]. Convincing evidence also exists for other environmental factors influencing the rate of CL/P in a population. These factors all relate to maternal health and exposures during pregnancy such as gestational hypertension, seizures, smoking, and indoor cooking over an open fire stove [6, 13]. These factors can also show a geographic distribution due to ethnicity, culture, and socioeconomic status. Altogether, genetic predisposition, in addition to differential regulations and environmental exposures, may account for why some populations appear to be more afflicted by CL/P than others worldwide. Understanding the true nature of this gene by environment interaction may allow for improved pre-natal diagnosis and prevention of cleft conditions.

Outcomes for Cleft Patients

The effectiveness of cleft care influences the burden of cleft disease, and this depends on the availability and accessibility of cleft care, its timeliness, and quality of outcomes. Ideal timing of primary cleft repair is between 4 and 6 months of age for cleft lip, and 8 and 12 months of age for cleft palate [17]. Delayed repair can be defined as repair occurring at an age over twice the ideal: 1 year for cleft lip and 2 years for cleft palate [18]. These recommendations stem from studies demonstrating the negative psychosocial impact of the response individuals can have to a person with a cleft, as well as the need for a functionally intact palate during the development of speech [2, 17]. The collective of patients who do not have their CL/P repaired, or who have an unsatisfactory outcome of the repair, by the appropriate time points constitute the backlog of cases [11, 18]. This backlog also represents the number of patients who need to be cared for in order to arrive at the lowest rate of cleft surgery required—sustainability for the birth rate.

Studies have estimated the backlog, or unmet need of cleft surgery, is highest in central Africa and southern Asia [19] (Fig. 2.3). However, these estimates focus on the unmet need of primary cleft repair only. Primary cleft lip or palate repair is only a portion of cleft care which can be defined as either complete or comprehensive. Complete cleft care includes all the necessary surgical procedures that should occur over the course of childhood to result in normal oral function, speech, and aesthetics. Complete care differs from comprehensive care which refers to the ability for a cleft patient to receive all necessary services surrounding their cleft condition. For example, in addition to cleft repair, patients with cleft palate may require nutrition consultation, speech therapy, tympanostomy tubes placed by an otolaryngologist, and audiology intervention among other services.

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Fig. 2.3

Map demonstrating the unmet need for cleft lip and cleft palate surgery worldwide. The highest burden of need is observed in low- and middle-income countries. (Reproduced from Carlson and colleagues [19] by permission of World Journal of Surgery)

In today’s current reality, many parts of the world are unable to deliver complete or comprehensive cleft care. Without access to such services, or even just primary cleft surgery, untreated individuals face considerable risk of morbidity and mortality. Morbidity can stem from functional limitations due to the cleft, including challenges with feeding and speech. However, the psychosocial impact of untreated clefts can also be tremendous due to stigma surrounding the condition in many countries. This stigma leads to limited engagement in educational opportunities, learning difficulties, poor self-esteem, depression, and anxiety, all of which ultimately impact individual potential to participate and achieve major milestones in life [2].

Patients with untreated clefts also experience a higher rate of mortality. A 2012 study by Mossey et al. found an exponential decrease in estimated survival to less than 10% by age 20 in cleft patients who did not receive surgery in India. By comparison, cleft patients who did receive surgery had long-term survival estimates similar to the UK population with survival only decreasing below 90% by age 50 [11]. Mortality burden was attributed to two broad categories: nutritional failures and infanticide. In rural areas with limited education and literacy, where lack of understanding exists for feeding options and techniques, mortality from dehydration and starvation can occur frequently in the neonatal period for patients with severe defects [11]. With regard to infanticide, the true incidence remains unknown. However, given the known stigma surrounding cleft conditions, an element of infanticide or neglect can be assumed to be present, especially in areas where the birth rate is either unreported or significantly lower than anticipated [11]. Regardless of mortality cause, an estimated 16.9% of preventable deaths due to lack of surgery could be averted by scaling up access to cleft lip and palate surgical care [20].

Economics of Cleft Care

The dichotomy of cleft conditions is that they are associated with a high economic burden and yet predominantly affect countries with the lowest income. The lack of subspecialty surgical care in low- and middle-income countries (LMICs) contributes to 38.9 million avertable disability-adjusted life years (DALYs) per year [20]. When looking at a cost-effective profile of surgical and public health interventions, the cost of providing cleft surgery is lower relative to life years (DALYs) saved than both certain vaccine programs and antiretroviral therapy for HIV [3] (Fig. 2.4). This economic burden of cleft conditions is further exacerbated in low-resource environments where limited or often no providers are available who can perform cleft repair. In such instances the backlog exponentially increases, compounding the detrimental economic effects of the condition [21] (Fig. 2.5).

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Fig. 2.4

Cost-effective comparison of cleft surgery to other surgeries and public health initiatives. Data points represent median disability-adjusted life years and error bars show range. Surgical interventions appear in red diamonds and solid lines. Public health interventions appear as blue circles with dashed lines. (Reproduced from Meara and colleagues [3] by permission of The Lancet)

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Fig. 2.5

Map of the incidence of cleft lip and/or palate (CLP) per surgeon, anesthesiologist, and obstetrician (SAO) available in each country. Of note, SAO density does not represent cleft specific providers but is used as a proxy for the density of CLP workforce. (Reproduced from Massenberg and colleagues [21] by permission of Springer Nature)

Approaches to Cleft Care

Defining Adequate Cleft Care

Ensuring adequate cleft care requires increasing the accessibility of cleft care services for those who receive no interventions, as well as recognizing the need and providing for more complete or comprehensive care for patients who may have received one or two surgical interventions. The prevalence and global distribution of patients who have not received any cleft care has been well studied and reported. However, identifying populations that have received incomplete or non-comprehensive cleft care is more challenging due to the need to perform qualitative and longitudinal assessments of patient care. A 1998 study by the Clinical Standards Advisory Group (CSAG) for cleft lip and palate found that only 63.1% of cleft teams in the UK longitudinally tracked their cleft lip patients and associated outcome [22]. For those that did follow their patients, 37–39% cited poor or very poor dental outcomes. A follow-up CSAG study found that fewer than 30% of these patients had an associated good cosmetic outcome [23]. Studies of overall cleft care quality have been performed in few countries, and these data are particularly sparse for low-resource settings. Even without granular data, given that the UK is a high-income country, the majority of the world likely lags behind the CSAG outcomes, which in themselves show major opportunity for improvement.

An additional challenge to the provision of adequate cleft care lies in the debate that has existed in the elements required for comprehensive and complete cleft care. The desire for improved quality in cleft outcomes led to the push for more extensive guidelines on comprehensive cleft care. The American Cleft-Palate Craniofacial Association (ACPA) guidelines for cleft palate and craniofacial teams states minimum mandates: the presence of a patient care coordinator; core team members from surgery, orthodontics, and speech-language pathology; and community resources to access psychology, social work, audiology, genetics, general and pediatric dentistry, otolaryngology, and pediatrics/primary care services [24]. Given this list of specialty requirements, comprehensive cleft care is currently only available in middle- and high-income countries.

Providing complete cleft care—the assurance that patients receive the full sequence of necessary procedures for their conditions—requires a varied set of surgical capabilities as well as the ability to longitudinally track a cleft patient throughout their childhood and into adulthood. Per McIntyre et al., patients with CL/P at a tertiary children’s hospital in the United States typically undergo an average of 8.6 surgical procedures prior to the age of 21 [25]. These surgeries would include combinations of lip repair, palate repair, alveolar bone grafting, surgery to address velopharyngeal insufficiency, orthognathic surgery, rhinoplasty, and revisions. The ability to provide all of these surgeries as needed over the course of two decades may be feasible with some but not all cleft care delivery models.

Methods of Reaching Untreated or Undertreated Patients

Multiple methods have been used to try and deliver cleft care to areas with high burden of disease and limited locally available resources. Initiatives for the delivery of cleft care to LMICs first began with the surgical mission, where human resources, equipment, and supplies provided by non-governmental organizations (NGOs) were temporarily transferred to an area of high burden in order to deliver surgical care to a large volume of patients in a condensed time frame. The introduction of the surgical mission was one of the first ways that many rural and low-resource environments learned of the potential for cleft treatment, the specialized needs of cleft patients, and the ability for cleft patients to lead normal lives when they have the chance to receive appropriate care.

Surgical missions, which have many benefits and performed regularly throughout the world, are a challenging method for delivering ongoing patient follow-up and complete cleft care. Recognizing the deficits in this model, multiple NGOs have created cleft centers with independent facilities or through hospital partnerships, where patients can access ongoing comprehensive care. However, in order to have center activity, countries must have baseline infrastructure to support a facility and enough trained providers for reliable accessibility of services. Therefore, while centers have been valuable in some circumstances, they are not universally applicable or appropriate for all environments.

For countries with a high burden of need, but limited infrastructure and/or providers, the challenge remains for how to deliver ongoing rather than episodic cleft care. In-country education initiatives aimed at increasing the number of skilled providers have been incorporated into the cleft care programs of multiple NGOs through independent and partnership programs [26]. In addition to being able to provide more cleft services on an ongoing basis, education initiatives have served as an economic builder for countries by allowing for expansion of employment opportunities through community health worker, patient advocate, and other outreach care positions.

Additional methods for reaching untreated or undertreated patients exist, which have been employed by various NGOs and governmental bodies. Upcoming chapters will discuss these methods of cleft care delivery in further detail, including history of their use, sustainability of care, their compatibility to run concurrently, and their relative efficacy in different environmental circumstances.

The Potential Impact of Cleft Care

Patients in LMICs struggle to obtain access to cleft care due to a number of barriers including structural, cultural, financial, and educational factors among other reasons. In a 2016 study of cleft patients in Vietnam, the most reported barriers to obtain cleft care included lack of care continuity (68%), equipment or medication shortage (67%), physician unavailability, unaffordability of food costs when receiving care (66% each), and lack of knowledge of where to obtain care or cost of living expenses while receiving care (64% each) [27]. Barriers to care however can be regionally different. In Brazil, patients reported the greatest barriers to care to be predominantly structural: lack of availability of hospitals able to perform cleft surgery (54%) and doctors capable of performing the surgery (51%) [28]. Provider opinions overlap with patient reports, with the greatest barriers felt to be patient travel costs (60.7%), lack of patient awareness (54.1%), and lack of financial support (52.8%). Similar to patient reports, provider opinions varied depending on country of origin, with over 1997 individuals surveyed worldwide [29].

Developing methods to safely and sustainably deliver cleft care will require minimizing patient barriers to care. These barriers, however, do not uniquely affect cleft patients, and can impair any individual from obtaining surgical services. Addressing cleft care, therefore, has the ability to decrease the overall burden of disease, increase the economic productivity of LMICs, and augment surgical and healthcare capacity in a sustainable way to improve the quality of surgical care for the whole population.

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© Springer Nature Switzerland AG 2021

J. W. Swanson (ed.)Global Cleft Care in Low-Resource Settingshttps://doi.org/10.1007/978-3-030-59105-2_3

3. Patient Barriers to Care

Caroline A. Yao¹, ²  , Desmond T. Jumbam¹, ² and D. Scott Corlew¹  

(1)

Program in Global Surgery and Social Change, Harvard Medical School, Boston, MA, USA

(2)

Department of Plastic and Oral Surgery, Boston Children’s Hospital, Boston, MA, USA

D. Scott Corlew

Email: [e-mail]

Keywords

Global surgeryBarriers to careBarriers to cleft lip and palate careLancet Commission on Global SurgeryHealth systems

Key Points

Cleft surgical care must be available, affordable, and acceptable in order for patients to receive it.

There are many supply and demand-side barriers to obtaining cleft care in the developing world, including those related to infrastructure, workforce, safety, financial risk protection, and patient and community perceptions, as well as social and cultural factors.

The Three Delays Framework can be useful to categorize barriers as those that delay the (1) seeking of care, such as knowing that a cleft palate is present and that it can be surgically treated, (2) reaching of care, including the costs of transportation and arranging childcare for other children at home, and (3) receiving care, which can be impeded by availability of ORs, anesthesiologists, or medications at the local hospital.

Affordability is better seen not as the absolute cost of care, but as the cost of surgical care relative to the patient or family’s ability to pay for it.

As patients live farther from hospitals offering cleft care, are less educated, and have fewer resources, misconceptions about the availability, benefits, and cost of surgery are easily propagated.

Introduction

Many children born in lower and middle-income countries (LMICs) with facial clefts lack access to the surgical and other care necessary to correct these deformities. Even for children who can receive care, this leads to delays such that children are often older than is optimal when they do undergo surgical correction. This chapter addresses the barriers that prevent children in low-resource settings from accessing and receiving the care needed at critical ages for optimal lifelong results.

Unlike in high-income countries where cleft lips are generally repaired by 3–4 months of age and palates by 12 months, in low-resource settings care is often delayed significantly, to the detriment of the patient. In one study, the median age at initial presentation ranged between 17–35 months among 8 LMICs studied and between 14–66 months in 3 low-income countries. For every US$1000 decrease in Gross Domestic Product (GDP) per capita, there was a 70% increase in the probability of palatal surgery being delayed beyond 24 months [1]. A study in Vietnam showed that only 48% of patients presenting for cleft care were less than 18 months old [2].

Understanding the barriers patients face to access and receive care is the first step toward strengthening surgical healthcare systems to ensure that patients receive care that is timely and appropriate. The three delays framework was originally developed in the obstetric literature [3]. It also applies well to cleft care: the first delay being delay in seeking care, the second in getting to care, and the third in the provision of that care. Figure 3.1 summarizes the relationship between supply and demand of surgical care .

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Fig. 3.1

The three delays framework applied to surgical care

In the cleft setting, the first delay is often most significant. A patient may not get the surgical care they need for three major reasons: availability, affordability, and acceptability [4, 5]. Surgical care is not available when a country’s healthcare system lacks the necessary infrastructure, workforce, supplies, and systems (Table 3.1) needed for service delivery. Surgical care is not affordable if paying to access and receive surgery pushes patients and families deeper into poverty or financial ruin. Acceptability represents the patient demand for surgery. People may not seek appropriate surgical care because of lack of awareness, or incorrect information, or lack of community or cultural acceptance of surgical treatment. Lack of trust in the quality or dependability of surgical services at a health facility could also lead to low acceptability among the community.

Table 3.1

Types of Cultural, Financial, and Structural Barriers Experienced by Patients with Clefts [4]

The overarching picture of global surgical care is described by the Lancet Commission on Global Surgery, and is very applicable to cleft care. Five billion people worldwide were found to lack access to safe, timely, and affordable surgical care; 94% of people in LMICs were in this group. The lack of financial risk protection plays a major role in this access problem: the Lancet Commission found that 33 million people per year face financial catastrophe because of medical costs (defined as using more than 10% of their yearly spending money on surgical care), and 48 million people have catastrophic expenditures from paying for non-medical costs of surgery (e.g., transportation to the hospital, food needed while getting surgery, and wages lost from time away from work). None of these numbers included people who did not access their needed surgical care. The Lancet Commission found that roughly half of the global population is at risk of catastrophic expenditure if they needed surgery. Over 30 million people become impoverished (pushed into poverty or further into poverty) when paying for surgical care. Table 3.2 summarizes the goals for increasing delivery of surgical care in LMICs as set forth in the Lancet Commission [6].

Table 3.2

Core indicators for monitoring of universal access to safe, affordable surgical and anaesthesia care when needed [6]

These indicators provide the most information when used and interpreted together; no single indicator provides an adequate representation of surgical and anaesthesia care when analysed independently

The World Health Organization (WHO) , World Bank, and national governments have taken notice that people in LMICs tend to have the most trouble accessing surgical care. A number of countries, thus far including Tanzania, Ethiopia, Zambia, Pakistan, and Rwanda, have taken major steps to address barriers to surgical care as recommended by the WHO by introducing National Surgical Obstetric, and Anesthesia Plans (NSOAPs) as part of their overall National Health Plans. These plans outline a strategy to develop the six major areas needed for surgical capacity building: infrastructure and supplies, workforce, service delivery, information management, governance, and financing [6].

NSOAPs, while aimed at the greater picture of all surgical care, are particularly applicable to the barriers faced by people needing cleft care. System barriers , defined as a lack of expertise (i.e., workforce), infrastructure, facilities, and equipment are the most significant cause of low availability of cleft surgery [4]. The WHO created a standard survey to help assess a healthcare facility’s capabilities. The survey can be repeated at different time intervals to assess a system’s progress and challenges [7].

Availability of Care (Supply)

Workforce

The supply of surgeons qualified to perform surgery in LMICs is limited. WHO data indicate that the African, Eastern Mediterranean, and Southeast Asian regions account for 47% of the global population but only 17% of the licensed surgeons. More disturbing is that there is only one licensed anesthesiologist per 1 million people in Southeast Asia and Africa [8].

Internationally, cleft lip surgery is performed by plastic surgeons, oral surgeons, ear/nose/throat surgeons, and general surgeons who require specialized training beyond typical surgical training programs. A survey in Vietnam showed that 66% of patients did not receive cleft surgery because they perceived a lack of trained surgeons [9]. Many children with cleft lips and palates continue to receive surgical care from NGOs that bring temporary increases in workforce and resource availability. A comprehensive review reported that NGOs delivering care through supporting permanent surgical hospitals are the most cost-effective, safe, and sustainable vehicles for care delivery. They suggest that temporary, short-term platforms are efficient when no other surgical delivery platform is available [10].

The surgical and anesthesia workforce are not the only barriers to care. Even when surgeons are available, they may be hindered from providing quality cleft surgery by lack of other necessary personnel, lack of supplies, or financial realities of their practices. For example, it has been shown that the availability of cleft surgery decreases when surgeons capable of performing cleft surgery (typically plastic surgeons) earn more money to perform other types of surgery (e.g., cosmetic surgery), or have patients with more life-threatening surgical needs. Inequitable distribution of surgeons and other practitioners also constitutes a barrier for many people; most are based in urban areas where there are more opportunities for practice and for families. Few surgeons choose to work in rural areas where the need is greatest [11].

Safe anesthesia is an additional limiting factor for cleft and other surgeries in LMICs. The limited supply of trained anesthesiologists decreases the availability of surgery, especially for infants and small children for whom more specialized anesthesiology training is helpful or sometimes imperative. Consequently, anesthesia care in developing countries is often provided by non-physician providers with widely varied training and experience and associated safety concerns [12].

The underlying etiology of the barrier to care of the lack of a trained workforce is a reflection of a lack of training capacity of the country. Training of specialized practitioners such as surgeons requires a strong educational network, including a university or medical school, expert surgeons to serve as instructors and a hospital system that integrates surgeons in training. Specialized surgeons, who can perform cleft lip and palate surgery, require the above resources and have the added challenges of fewer expert instructors available and the relative rarity of patients born with a cleft lip and/or palate.

It is also important to acknowledge the network of support staff other than the surgeons and anesthesiologists that are required for safe surgical care provision. These include nurses, biomedical engineers, sterilization staff, speech therapists, and administrative staff. Limited numbers of these providers further limit care provision in LMICs.

Infrastructure

Even when expertise to provide cleft surgery is available, inadequate infrastructure may be a barrier. Not only hospitals, but also basic public needs such as reliable roads, bridges, and public transport are major factors that decrease the availability of surgery [4]. Hospital buildings must have appropriately outfitted operating rooms, anesthesia capacity, hospital beds, laboratory facilities, and supply chains. Transportation infrastructure affects whether or not patients and families can safely and consistently travel to a hospital, and also whether or not supplies can be reliably delivered to hospitals.

Once patients arrive at a hospital facility, the facility must be functional, meaning it has space, durable equipment (medical machines) that is appropriate, available and functional, appropriate diagnostics (labs and imaging), and consumables (e.g., medicine, bandages, and other supplies). Inadequacies in any of these present further barriers to care and can lead to delays in care and adverse outcomes. Availability of basic utilities like water and electricity can determine the availability and even the quality of surgery provided. From cleaning and sterilizing surgical equipment to powering equipment such as anesthesia machines, water and electricity are essential for the provision of surgical care.

Most healthcare systems are designed such that basic and emergency curative and preventive