Squamosal Craniosynostosis Associated with Rickets.

Rickets results from defective bone mineralization, leading to skeletal deformities. Among those deformities, rickets has been associated with craniosynostosis, the premature closure of cranial sutures. Most of these patients have fusion of major sutures. Rarely, squamosal craniosynostosis in association with rickets has been described. Squamosal craniosynostosis is noted as lacking a definitive head abnormality and difficult visualization on standard imaging modalities, leading to poor recognition. Careful attention should be given to rickets patients to monitor for these unusual suture closures. Additionally, craniosynostosis could be a presenting feature of rickets, and further rickets evaluation of the patient is indicated.

Posterior cranial vault distraction osteogenesis in the immunocompromised patient.

PURPOSE: The treatment of patients with multisuture craniosynostosis is complex and patient-dependent. Cranial distraction osteogenesis is a relatively new procedure for treatment of these patients, with its use increasing in many centers. With this increased use comes an expanding range of indications. Surgical management of multisuture craniosynostosis in therapeutically immunosuppressed patients following a solid organ transplant presents unique challenges. We describe our experience with posterior cranial vault distraction in two patients with multisuture craniosynostosis that had previously undergone organ transplantation. METHODS: Two solid-organ transplant recipient patients with multisuture craniosynostosis were identified. A detailed examination of their medical/transplant history and perioperative details were recorded. RESULTS: The first patient was a 3-year-old girl who received a kidney transplantation in infancy and subsequently presented with a symptomatic Chiari malformation and papilledema. Imaging revealed pansynostosis. She underwent posterior cranial vault distraction extending into a Chiari decompression. Her postoperative course was complicated by distractor site infection at the beginning of consolidation, necessitating early removal of distractors. The second patient was a 2-year-old boy who received a heart transplantation at the age of 3 months and subsequently presented with head shape concerns. Imaging revealed bicoronal and sagittal craniosynostosis. He underwent a posterior cranial vault distraction without complication. Following removal of the distractors, he developed an infection at one of the distractor sites with associated fever and leukocytosis, necessitating washout and drain placement. Both patients achieved successful cranial vault expansion with distraction osteogenesis and at a 2-year follow-up do not have evidence of elevated intracranial pressure. CONCLUSIONS: Immunosuppressive therapy has the potential to inhibit wound healing and place patients at risk for wound infection. Although we have demonstrated successful cranial vault expansion with distraction in two immunosuppressed children, extra care must be taken with these patients when placing semi-buried hardware. Specifically, prompt identification and proactive management of potential infectious complications is critical to applying this technique safely in these patients.

Iatrogenic Intradiploic Epidermoid Cyst Following Strip Craniectomy for Sagittal Craniosynostosis.

INTRODUCTION: The increased incidence and success of strip craniectomy with postoperative helmet therapy in the treatment of sagittal craniosynostosis has been documented by multiple centers throughout the country and world. The authors report a child with a postoperative implantation intradiploic epidermoid cyst following a strip craniectomy, a complication, that to our knowledge, has not been reported. METHODS: This clinical report involves a 3-year-old boy with a scaphocephalic appearance who was transferred to our center following an interstate adoption. He underwent a strip craniectomy with helmet therapy in infancy. On presentation to our facility the chief complaint was a scaphocephalic appearance. Preop computed tomography scans showed areas of bone gaps along the sagittal suture. RESULTS: The child was brought to the operating room for a mid-vault expansion. At surgery, a large intradiploic epidermoid cyst was noted on the posterior aspect of the area of the sagittal suture, immediately beneath to posterior incision for the strip craniectomy. The cyst extended through the inner table of the skull and necessitated split cranial grafts to aid in the reconstruction. CONCLUSION: The authors present a patient with an iatrogenic intradiploic epidermoid cyst of the posterior skull following strip craniectomy, which has not been previously been described in association with strip craniectomy. This patient underlies the importance of a strong working relationship between craniofacial surgery and neurosurgery.