OBJECTIVE This study examines a series of patients with hypophosphatemic rickets and craniosynostosis to characterize the clinical course and associated craniofacial anomalies. METHODS A 20-year retrospective review identified patients with hypophosphatemic rickets and secondary craniosynostosis at 3 major craniofacial centers. Parameters examined included sex, age at diagnosis of head shape anomaly, affected sutures, etiology of rickets, presenting symptoms, number and type of surgical interventions, and associated diagnoses. A review of the literature was performed to optimize treatment recommendations. RESULTS Ten patients were identified (8 males, 2 females). Age at presentation ranged from 1 to 9 years. The most commonly affected suture was the sagittal (6/10 patients). Etiologies included antacid-induced rickets, autosomal dominant hypophosphatemic rickets, and X-linked hypophosphatemic (XLH) rickets. Nine patients had undergone at least 1 cranial vault remodeling (CVR) surgery. Three patients underwent subsequent surgeries in later years. Four patients underwent formal intracranial pressure (ICP) monitoring, 3 of which revealed elevated ICP. Three patients were diagnosed with a Chiari Type I malformation. CONCLUSIONS Secondary craniosynostosis develops postnatally due to metabolic or mechanical factors. The most common metabolic cause is hypophosphatemic rickets, which has a variety of etiologies. Head shape changes occur later and with a more heterogeneous presentation compared with that of primary craniosynostosis. CVR may be required to prevent or relieve elevated ICP and abnormalities of the cranial vault. Children with hypophosphatemic rickets who develop head shape abnormalities should be promptly referred to a craniofacial specialist.

Total ear reconstruction has been approached by several techniques involving autologous graft, prosthetic implant, and alloplastic implant options. Recent studies have shown the superiority of porous polyethylene (Medpor, Porex Surgical) reconstruction over autologous reconstruction based on improved aesthetic results, earlier age of intervention, shorter surgery times, fewer number of required procedures, and a simpler postoperative recovery process. A durable and permanent option for total ear reconstruction, like Medpor, can help alleviate the cosmetic concerns that patients with auricular deformities may be burdened with on a daily basis. In this article, the authors discuss the advantages of Medpor-based ear reconstruction and discuss recent advances in the surgical techniques involved, such as harvesting a temporoparietal fascia flap and full-thickness skin graft to adequately cover the Medpor framework and decrease extrusion rates.

Attempts at reducing exposure to allogeneic transfusions, using blood conservation techniques such as controlled hypotension and normovolemic hemodilution, have met with mixed results and are not always practical in small infants. Recombinant human erythropoietin (RHE), a hormone that stimulates RBC production, increases the hematocrit when administered to infants. A retrospective chart review of all patients undergoing fronto-orbital advancement for craniosynostosis by the same plastic surgeon between January 2002 and December 2002 was conducted. A subgroup of patients (10/19) received RHE as a blood-conservation strategy. Transfusion requirements were lower in the RHE group (5/10) versus the control group (9/9). Total volume of blood products transfused was statistically lower in the RHE group (154 mL RHE group versus 421 mL control) (P < 0.03). RHE combined with blood-conservation techniques was associated with a decreased need for blood transfusion, thus exposing the patient to fewer risks associated with allogeneic transfusion.

Despite war wound contamination, massive cranial defects can be successfully reconstructed using custom alloplastic implants. However, reconstruction of frontal cranial defects in proximity to the airways and orbits was associated with infection and implant removal.