F. Fallahian1, M. Tracy1, A. Kaye1 1Children’s Mercy Hospital- University Of Missouri Kansas City,Department Of Plastic Surgery,Kansas City, MO, USA
Introduction: Direct causality of congenital cleft conditions is often not known and is currently considered to represent a combination of complex environmental and genetic factors and interactions. There are numerous syndromes which can be associated with cleft lip and/or palate as well as unique patients with multiple medical problems without an over-arching diagnosis. This provides us with a number of patients for whom highly individualized treatment plans must be devised. Safety and appropriateness for surgery or the need to prioritize other necessary surgeries are factors that may preclude following typical cleft treatment protocols. This study aims to determine the prevalence of children in our Cleft Team population who also carry concomitant complex medical diagnoses in order to assess the challenges of this population and the alterations in their ultimate cleft-related care.
Methods: This study is a retrospective review of patients presenting to the Cleft Team for cleft care with a history of multiple medical issues in addition to a diagnosis of cleft lip and/or palate.
Results: 133 patients were identified with a variety of cleft conditions: incomplete cleft lip (CL) = 7, cleft lip and palate (CL/P) = 51, isolated cleft palate (CP) = 53, and submucous cleft palate (SMCP) = 22. A numerous variety of concomitant diagnoses were seen including 37 named syndromes, 20 unique chromosomal abnormalities, and 17 unidentified constellations of anomalies. Diagnoses were made by a combination of clinical assessment (30.7%), specific gene studies (25.0%), high resolution chromosome (18.3%) or CGH microarray analysis (16.3%). 51.9% of patients have congenital heart disease, 40.6% of which required surgery to treat. 36.8% of patients have brain abnormalities. 23.3% have congenital hearing loss. 63.9% have developmental delays. 81.2% of patients are cared for by 3 or more subspecialty teams aside from the Cleft Team, most commonly: ENT, Cardiology, Ophthalmology and Orthopedic Surgery. 54.1% of these patients have surgical feeding tubes and 15.0% have tracheostomies. 6.7% of these patient have died prematurely related to their condition. Average age at cleft lip repair when performed in this group is 7.29 months (SD 2.65). Average age at primary palate repair in this group is 23.13 months (SD 20.56). One third of patients have delayed or missed cleft-related surgeries. 60.9% of these patients have global developmental delays. More than half have speech-language delays, 59.4% of which are severe or profound.
Conclusion: Patients with congenital cleft conditions and concomitant complex medical presentations present unique situations for coordinated cleft team care. These patient have frequent delays in the timing of their cleft-related surgeries resulting from poor health condition, need for other surgery, or significant speech/developmental delays.