2025 Poster Presentations
P196: PEDIATRIC SINONASAL CHONDROMESENCHYMAL HAMARTOMAS: CASE REPORT AND OPERATIVE CONSIDERATIONS FOR CRANIOFACIAL GROWTH OPTIMIZATION IN EXPANDED ENDONASAL APPROACHES
Elaina J Wang, MD1; Natalie Amaral-Nieves, MD1; Mattthew Kovoor, BA1; Peter Steinwald, MD2; Maria Koenigs, MD1; Basit Jawad, MD1; Christine K Lee, MD, PhD1; 1Brown University; 2Tufts Medical Center
Introduction: Nasal chondromesenchymal hamartomas (NCMH) are rare, benign sinonasal lesions which are treated with complete surgical resection. For pediatric patients withNCMH or other skull base and sinonasal tumors, it is important to preserve structures critical for craniofacial developmentwherever possible. The pediatric skull base and cranial vault continues to undergo changes over the first decade of life, and such structural changes and sinus pneumatization patterns should be considered when planning for these surgeries. In this study, we report a case of NCMH in a pediatric patient, and we conduct a review of NCMH in pediatric patients as well as a review of studies on the effect of expanded endonasal surgeries on craniofacial growth, with the objective of understanding which structures are critical to preserve during surgery to minimize surgical morbidity while achieving maximal tumor resection.
Methods: A rare case of NCMH in a 3 year old pediatric patientand complete surgical resection of the tumor was described. The authors conducted a literature review to identify studies on NCMH in pediatric patients, as well as a review to identify studies detailing expanded endonasal surgeries of skull base tumors in relation to craniofacial development in children.
Results: A 3 year old girl with a history of bilateral eye discharge since infancy presented with intermittent fevers and right-sided epistaxis from a protruding sinonasal mass. On MRI, a large heterogeneously T2-hyperintense lesion was seen in the right nasal cavity with extension into right maxillary sinus (Figure 1). On exam, the patient had septal deviation to the left, right-sided proptosis, and right-left orbital height mismatch (Figure 2). Preliminary pathology from a biopsy revealed myxoid stellate to spindled cells interspersed with cartilage/bone suggestive of chondromesenchymal hamartoma versus sinonasalmyxoma. The patient subsequently underwent endoscopic endonasal resection. The tumor appeared to originate from theinferior medical maxilla and extended to the nasal septum medially and the floor of the anterior cranial fossa superiorly. No inferior turbinate was identified, likely due to erosion from the compressive effects of the tumor. Involved bone of the medial/posterior maxillary walls, medial pterygoid, and bone overlying the nasolacrimal duct were removed. Maxillary mucosa closely intertwined with the tumor itself was also removed. Following gross total resection of the tumor (Figure 3), which was confirmed to be a chondromesenchymal hamartoma, the patient demonstrated improvement in intraocular pressures and the position of the orbit. Based on our literature review of NCMH in pediatric patients, and a review ofaspects of craniofacial development specifically in relation to expanded endonasal approaches for pediatric skull base tumors, we identified key skull base and sinonasal structures to preserve during surgery and summarized the surgical nuances involved in these cases to balance the surgical goals of maximal tumor resection and minimal surgical morbidity.
Conclusion: This study provides an investigation of NCMH in pediatric patients and a discussion of the nuances regarding critical structures to preserve intraoperatively to preserve normal craniofacial development, which should be taken into consideration in the resection of skull-base tumors in the pediatric population.
