Multidisciplinary Team Care: Cleft and Craniofacial Surgery

Abstract

Cleft and craniofacial anomalies impact the functional, psychological, and social well-being of patients and their families. To properly address the manifestations of these conditions, coordinating a team comprised of a variety of specialists is the gold standard. In this chapter, we will discuss the structure of the multidisciplinary team and our experience caring for patients with cleft and craniofacial differences at a large academic pediatric hospital.

Cleft lip/palate and craniofacial disorders often present concurrently with a diverse range of symptoms, impacting structure, function, and psyche in ways that overlap multiple specialties. ¹ To provide optimal, comprehensive treatment for these patients, a multidisciplinary team is required. As such, the American Cleft Palate Craniofacial Association has set standards for the approval and requirements for the composition of a cleft palate and craniofacial team. ² In this chapter, we will review multidisciplinary team care in the setting of patients with cleft lip/palate and craniofacial diagnoses and provide our perspective from a large academic pediatric hospital.

Multidisciplinary Team Composition

An ideal cleft and craniofacial multidisciplinary team ( Table 1 ) is composed of audiologists, dentists/orthodontists, geneticists and genetic counselors, neurosurgeons, nurse coordinators, nutritionists, occupational and physical therapists, oral and maxillofacial surgeons, ophthalmologists, otolaryngologists, pediatricians, plastic and reconstructive surgeons, psychologists/psychiatrists, researchers, respiratory care specialists, social workers and speech and language pathologists. ³

Table 1. Members of the cleft and craniofacial multidisciplinary care team and their roles.

Specialty	Role and importance
Audiologists	Evaluate and manage hearing loss or impairment in infants and children with craniofacial anomalies
Dentists/orthodontists	Provide early oral health interventions, fabricate oral devices, and manage orodental issues in patients
Geneticists/genetic counselors	Share expertise on diagnosis, prognosis, and hereditary risks for cleft or craniofacial conditions
Neurosurgeons	Assess neurodevelopment of patients and perform necessary surgical interventions for craniofacial conditions such as craniosynostosis
Nurse coordinators	Coordinate patient care needs and ensure efficient communication between the patient and the multidisciplinary team
Nutritionists	Optimize dietary intake and nutritional goals for patients, to optimize wound healing and mitigate surgical complications
Occupational/physical therapists	Strengthen motor function and coordination by improving muscle strength pre- and postoperatively
Ophthalmologists	Identify and manage orbital deformities or ocular defects associated with craniofacial syndromes.
Oral and maxillofacial surgeons	Identify and manage dentofacial abnormalities, such as malocclusion
Orthotics	Create and adjust helmets to correct pathologic head shapes
Otolaryngologists	Provide complex airway and otologic care
Pediatricians	Manage the patient's overall health and issues related to growth and development in cleft or craniofacial disorders
Plastic and reconstructive surgeons	Central role in cleft and craniofacial multidisciplinary teams offering definitive surgical treatments and comprehensive guidance
Psychologists/psychiatrists	Monitor and manage potential cognitive, psychological, and developmental issues
Researchers	Conduct research to improve outcomes and expand knowledge in the field of craniofacial surgery
Respiratory care specialists	Optimize airway management to prevent hospital readmissions
Social workers	Ensure patients and families receive necessary services and support throughout the continuum of care
Speech and language pathologists	Evaluate and treat speech, language, and feeding issues in patients with cleft lip/palate and craniofacial anomalies

Craniofacial anomalies are among the most common causes for hearing loss and impairment in infants and children. ⁴ Audiologists are an exceedingly important component of the multidisciplinary team as untreated hearing deficiencies can complicate speech and language development in addition to their inherent psychosocial effects.

Patients with craniofacial conditions, especially those with cleft lip/palate may be affected by several orodental issues, requiring consistent involvement of a dentist and orthodontist. ^{5 6 7} Pediatric dentists and orthodontists are able to provide early interventions such as sealants and varnishes to help prevent the development of orodental issues such as dental caries. ⁵ Dentists and orthodontists also administer nasoalveolar molding (NAM) to prepare for cleft lip repair and enhance postoperative results. ⁵

Cleft or craniofacial disorders can be associated with a variety of environmental and genetic factors. A geneticist and genetic counselor provide clarity and guidance on diagnosis, prognosis, and the hereditary risk for future children.

Certain diagnoses, such as craniosynostosis, necessitate involvement of a neurosurgeon for neurodevelopmental assessment, management of elevated intracranial pressure, and surgical intervention.

By serving as a primary point of contact, a nurse coordinator promotes efficiency of the multidisciplinary team by liaising and consistently communicating with the patient and their family. Additionally, depending on the diagnosis and treatment stage, the coordinator ensures that each patient is seen by the appropriate clinician(s).

Dietary consistency may directly relate to human craniofacial growth and nutritional optimization is exceedingly important for patients who require surgical intervention for healing. ⁸ As such, a nutritionist can work with patients and their families to reach general, preoperative, and postoperative nutritional goals. Especially in the setting of cleft lip and palate, nutritional assistance is vital as many of these patients have feeding difficulties in the first few weeks of life.

Occupational/physical therapists assist in strengthening motor function and coordination in this patient population, as well as optimizing muscle strength and function pre- and postoperatively.

Ophthalmologists are experts in managing orbital deformities that are a byproduct of craniofacial anomalies. In conditions that affect the cranium such as craniosynostosis, ophthalmologists are integral in detecting abnormal intracranial pressures through fundoscopic examination.

Many craniofacial disorders, especially in regard to cleft palate and craniosynostosis, may present with dentofacial abnormalities, such as midface hypoplasia and malocclusion that may require the expertise of an oral and maxillofacial surgeon or craniofacial surgeon trained in orthognathic surgery. Oral surgeons may also perform dental extraction in preparation for orthognathic surgery.

Certain children with craniosynostosis may qualify for an endoscopic suturectomy, which then requires several months of helmeting to correct cephalic anomalies. An experienced orthotist is vital to not only fabricating an initial helmet but also performing routine three-dimensional laser head scans to finesse the shaping.

Any patient presenting with a cleft or craniofacial condition requires immediate assessment of the airway. Otolaryngologists are essential in evaluating and managing the airway in this population, such as in cases of Pierre Robin sequence. Furthermore, given the often aberrant anatomy of the Eustachian tube in cleft diagnoses, otolaryngologists provide surgical intervention with myringotomy and tympanostomy tube insertion. They can also monitor children for the development of airway obstruction due to tonsil and adenoid hypertrophy.

Pediatricians are the team members who fully understand the overall health of a patient with cleft or craniofacial differences and manage the comorbid medical conditions as they grow and progress through treatment.

A plastic surgeon trained in craniofacial surgery is often at the core of the multidisciplinary team. Their expertise and extensive knowledge on the pathophysiology of cleft and craniofacial disorders allows them to determine treatment options as well as diagnostic sequelae. In addition, they offer education and surgical planning, often including multiple staged procedures.

The psychological impact of cleft or craniofacial disorders can be immense. Psychologists and psychiatrists are essential in monitoring the cognitive, psychological, and developmental growth of these patients. Additionally, the incidence of suicidal ideation and behavior has been reported to be higher in patients with cleft lip/palate and other craniofacial conditions, when compared with the general population. ⁹ This highlights the importance of early psychological and, if needed, psychiatric involvement.

Compared with many other medical specialties and subspecialties, the field of craniofacial surgery is relatively new. There is much about the conditions, treatments, and outcomes that warrants investigation, and a research team is invaluable in optimizing knowledge in this patient population and advancing treatment in an evidence-based manner.

Respiratory care specialists optimize the airway perioperatively while also establishing healthy airway treatment plans, preventing unnecessary hospitalizations. ³

Social workers are in constant communication with patients and their families ensuring that they receive necessary services as they progress through their clinical course, which can include numerous appointments, procedures, and surgeries. ³

Speech and language pathologists are paramount for evaluating and treating issues in speech, language, resonance, and feeding in patients with cleft lip and/or palate and those with craniofacial anomalies such as velopharyngeal insufficiency. ¹⁰

The Texas Children's Hospital Experience: Cleft and Craniofacial Clinics

Over 1,300 patients are seen between our cleft and craniofacial clinics yearly, originating from local, state, national, and international contexts. Each week begins with an interdisciplinary craniofacial team conference, where admitted patients are discussed and upcoming weekly operative cases are reviewed. Each Monday and Wednesday are reserved for our multidisciplinary cleft and craniofacial clinics. Prior to the first patient's arrival, the nurse coordinator audits the schedule and marks on a centralized whiteboard the disciplines that will be needed for each patient. For cleft clinic, our team includes craniofacial surgeons, orthodontists, genetic counselors, advanced practice provider (APP), nurses, dietitians, nurse coordinators, psychologists, social workers, speech and language pathologists, and support staff. Each of these providers has a room flag, signaling that they are interacting with the patient. Our craniofacial clinic (i.e., craniosynostosis) includes all aforementioned members, with the addition of our neurosurgical and orthotic colleagues. As our clinic is located within a large pediatric hospital, readily available collaboration includes audiologists, dentists, otolaryngologists, ophthalmologists, geneticists, occupational/physical therapists, oral and maxillofacial surgeons, pediatricians, researchers, and respiratory care specialists. Every effort is made to provide same-day encounters with these services. Fig. 1 depicts our multidisciplinary cleft treatment timeline.

Fig. 1.

Multidisciplinary treatment timeline for patients with a cleft lip and/or palate. Reproduced with permission from Texas Children's Hospital.

In order to illustrate the nuances of our institution's approach, below is a case study of a prototypical patient navigating through a multidisciplinary visit.

Clinic Flow Case Study

A 2-week-old female with 22q11.2 deletion syndrome and bilateral complete cleft lip and palate presents to our Monday clinic. Her mother is a refugee and delivered at 37 weeks. The mother did not receive prenatal counseling, and the patient is small for gestational age. A language-specific interpreter is obtained for each encounter, either in person or remotely. Once the patient is roomed and vitals obtained, the medical assistant (MA) updates the electronic medical record with a green dot, indicating that the patient is ready for the provider. The MA also organizes door flags to reflect the various providers who will be involved in the patient's care. In this case, the plastic surgeon and APP initiate the history and physical exam and then communicate the surgical plan with the family. Given that the cleft lip is wide, the surgeon recommends NAM to prepare for primary cheiloplasty. This proposal is communicated to the orthodontist by the plastic surgeon. The orthodontist then visits the patient and explains the process, which includes serial follow-ups associated with NAM. The APP places a surgical order to schedule NAM impression within the week, and now, both the surgical and orthodontic teams remove their flags from the door. The surgical team provides a direct hand off to the APP who then addresses feeding, growth, and primary care needs of the infant. Next, the registered dietitian assesses the caloric intake of the infant and helps coordinate the Special Supplemental Nutrition Program for Women, Infants, and Children documentation so that the family can receive formula. The speech pathologist performs an initial evaluation and provides samples of slow-flow nipples to facilitate feeding. They also reinforce the plan of repairing the palate prior to development of meaningful speech, and they educate the family on expectations for future speech therapy sessions. The genetic counselor elicits a thorough family history and participates in shared medical decision-making to send a cheek swab for further genetic testing. Psychology visits the patient and focuses on ensuring the well-being of the mother in the context of postpartum depression. Counseling resources are given and follow-up with the family is scheduled. Finally, the social worker concludes the patient's visit by offering resources for Medicaid enrollment and transportation options. While not held within the same clinical space, the patient will be scheduled for an anesthesia preoperative appointment to ensure the patient is safe to proceed with general anesthesia from a cardiac and airway perspective. Additionally, a referral is made to otolaryngology for tympanostomy tube placement at the time of cleft palate repair, or earlier if the patient has a history of recurrent ear infections. In the case of a failed newborn hearing screen, otolaryngology may evaluate the patient concurrently with audiology. At the conclusion of cleft clinic, the nurse coordinator and plastic surgeon facilitate a multidisciplinary postcleft conference to ensure all services have a holistic understanding of every patient. We have found that this nurtures a collaborative environment.

Regarding craniofacial multidisciplinary clinic, the foundation is almost identical to our cleft clinic with the addition of a neurosurgeon and neurosurgery APP. They provide insights from a neurosurgical perspective regarding intracranial pressure, any potential Chiari malformation, and surgical planning for cranial vault remodeling, endoscopic suturectomy, fronto-orbital advancement, monobloc advancement, or spring-assisted cranioplasty. Patients who undergo endoscopic suturectomy will often follow up concomitantly at their postoperative visit with our helmet orthotist for head shape scans and helmet adjustments. The craniofacial Wednesday clinics include speech pathology and orthodontics on an as-needed basis. The multidisciplinary model functions not only to streamline patient's surgical needs but also to provide holistic and dynamic care for the patient with cleft ( Fig. 2 ) and craniofacial ( Fig. 3 ) differences.

Fig. 2.

An 8-year-old patient with complete right complete cleft lip and palate ( A ) underwent alveolar bone grafting, with the postoperative results in B and C . After being optimized by our dental and orthodontic colleagues, the patient returns at 18 years of age with class III malocclusion ( D ) requiring a LeFort I maxillary advancement. Postoperative results are depicted in E and F . This case highlights the necessity of multidisciplinary collaboration throughout the patient's treatment course to achieve favorable outcomes. Reproduced with permission from Texas Children's Hospital.

Fig. 3.

An 8-year-old Crouzon syndrome patient ( A and B ) without symptomatic exorbitism but residual midface hypoplasia, treated with LeFort III distraction osteogenesis ( C and D ). This patient required the multidisciplinary care at our craniofacial clinic involving plastic surgery, neurosurgery, and orthodontics, with the durable 20-month postoperative results demonstrating improved facial profile in E and F. Reproduced with permission from Texas Children's Hospital.

Conclusion

A multidisciplinary team is indispensable for the proper treatment and restoration of the craniofacial region's vital functions and aesthetics. While the various specialties involved are foundational, the manner in which the logistics are coordinated vary from center to center. With over 10 years of our multidisciplinary cleft and craniofacial clinics functioning in this manner, we have sought to share the highlights of the structure that has benefited our patient cohort. This has proven to not only serve our patients and families but also bolster a collaborative and innovative environment.

Funding Statement

Funding None.