A protocol of situation-dependent transfusion, erythropoietin and tranexamic acid reduces transfusion in fronto-orbital advancement for metopic and coronal craniosynostosis.

PURPOSE: Assess the effect of a protocol of preoperative erythropoietin (EPO) and ferrous sulfate in addition to perioperative tranexamic acid (TXA) on blood transfusions in patients with coronal or metopic craniosynostosis undergoing cranial vault remodeling (CVR) with fronto-orbital advancement (FOA). METHODS: Retrospective review of all coronal and metopic craniosynostosis patients undergoing CVR and FOA from March 2010 to June 2019 was performed. Before 2014 ("Control group"), all patients received blood transfusion at the start of surgery. In 2014, a protocol of preoperative EPO and ferrous sulfate with perioperative TXA and non-automatic transfusion was instituted ("Study group"). Patient demographics and anthropometrics, perioperative hemoglobin (Hb) levels, and transfusion details were collected and compared. RESULTS: Thirty-six patients met inclusion criteria. Twenty-one patients were in the control group, and 15 in the Study group. Nineteen patients had metopic synostosis, 11 had unicoronal synostosis, and 6 had bicoronal synostosis. There were no significant differences between groups in demographics, operative time, intraoperative crystalloid volume, craniofacial syndromes, or sutures affected. The Study group had higher preoperative Hb (13.9 ± 1.0 vs. 12.6 ± 0.8 g/dL, p < 0.001), lower intraoperative Hb nadir (7.4 ± 1.8 vs. 9.2 ± 1.2 g/dL) lower intraoperative transfusion rate (66.7% vs. 100%, p = 0.008), lower postoperative transfusion rate (0% vs 28.6%, p = 0.03), and exposure to fewer unique units of packed red blood cells (0.7 ± 0.6 vs. 1.5 ± 0.9 units). CONCLUSION: Our protocol resulted in decreased transfusion needs. These results add valuable information to the growing body of work on transfusion reduction in craniosynostosis surgery.

Minimizing transfusion in sagittal craniosynostosis surgery: the Children's Hospital of Minnesota Protocol.

PURPOSE: To assess the success of a protocol using preoperative erythropoietin (EPO) and iron with perioperative tranexamic acid (TXA) in reducing blood transfusion in sagittal craniosynostosis surgery. METHODS: A retrospective chart review of all sagittal craniosynostosis patients undergoing open repair at our institution since 2010 was conducted. A novel protocol of preoperative EPO with iron and perioperative TXA, along with a shift away from automatic transfusion, was initiated in 2014. Perioperative hemoglobin levels, length of stay, and transfusion rates were compared between the historical control and the study group receiving the protocol. RESULTS: A total of 36 patients met inclusion criteria. Twenty-eight patients were male and 8 were female. Twenty-two patients were in the control group receiving neither TXA nor EPO and automatically received a transfusion, while 14 were in the study group and received the full protocol. There were no significant demographic differences between groups. Within the control group, 100% of patients were transfused compared with 14.3% of the study group (p < 0.0001). The study group also had a shorter postoperative length of stay in the hospital (mean, 3.4 days; range, 3-6) than the control (mean, 4 days; range, 2-5.5, p = 0.038). The study group had a higher preoperative hemoglobin than the control (13.6 vs. 11.8 g/dL, p = 0.0001). CONCLUSION: Our protocol of preoperative EPO and iron with perioperative TXA increased the preoperative hemoglobin and was associated with a low transfusion rate without negatively impacting postoperative course.

PD0332991 (palbociclib) for treatment of pediatric intracranial growing teratoma syndrome.

Growing teratoma syndrome is characterized by growth of mature teratoma elements of a mixed germ cell tumor despite resolution of immature/malignant elements with administration of chemotherapy. Surgical resection is the only known cure for growing teratoma syndrome but in the brain, complete resection may be impossible. In these instances, mature teratoma, although histologically benign, may be fatal. In this report, we present the case of a child with a large, rapidly growing, unresectable pineal region growing teratoma. PD0332991 was administered with stabilization of the solid, enhancing components of the mass. Minimal adverse effects were noted.

New resorbable plate and screw system in pediatric craniofacial surgery.

Early forms of resorbable fixation induced foreign body reactions requiring surgical removal of the fixation material. Most current plating systems attempt to circumvent this phenomenon by altering the composition of the α esters involved. ResorbX (KLS Martin, Jacksonville, FL), a plating system derived from 50:50 poly(D,L)lactide, boasts short resorption times, minimal foreign body reaction, and adequate strength for bony fixation. We present our experience with 134 patients undergoing correction of primary craniosynostosis, using ResorbX. One hundred thirty-four consecutive craniosynostosis patients underwent correction with calvarial remodeling by the senior author between April 2002 and March 2008. Five patients had 2-stage repairs. Postoperative analysis included plate visibility or palpability, head contour, and the incidence of complications. The mean age at repair was 17.0 months, with the median age being 9.0 months (range, 2.5-137 months). Postoperative follow-up visits were recorded at 3-month intervals from 0 to 24 months. Synostosis diagnoses were as follows: 43 metopic, 37 sagittal, 27 coronal, 5 lambdoid, and 27 multisutural synostosis. There were 3 complications requiring operative intervention. Three plates became exposed through the coronal excision, requiring removal, for an overall complication rate of 2%. Forty-four patients (31.7%) had a visible mass at the site of fixation at some point postoperatively; all of these resolved through observation only. Overall postoperative head aesthetics were deemed satisfactory to excellent, and no instances of contour regression occurred. This study documents the safety and efficacy of ResorbX in pediatric cranial vault remodeling. The system is easy to use, and with the advent of an ultrasonic pin, more abbreviated operating and anesthesia times are achievable.

Parental satisfaction with the CranioCap: a new cranial orthosis for deformational plagiocephaly.

OBJECTIVE: This study evaluates the safety and parent satisfaction of a new cranial orthosis designed to treat deformational plagiocephaly. DESIGN: A retrospective phone survey of parental satisfaction of a new cranial orthosis that has Food and Drug Administration (FDA) clearance for treatment of deformational plagiocephaly. SETTING: A tertiary referral center for children with craniofacial anomalies. PATIENTS: The first 232 consecutive infants enrolled in this program were retrospectively reviewed. INTERVENTIONS: Infants were entered in the cranial orthosis program if they had been diagnosed with deformational plagiocephaly and were less than 1 year of age, and if the infant's parents were interested in enrolling in a monitored cranial orthosis program. MAIN OUTCOME MEASURE: After completing therapy, a random sample of 81 parents (half completing treatment) was surveyed. Parents rated the improvement in their infant's head shape from 1 (least) to 5 (most). They were asked if they would repeat the program with a subsequent child. To date, 70% (162/ 232) have completed treatment. RESULTS: The orthosis was worn an average of 89 days. On average, parents rated head shape improvement 4.06 out of 5 (range, 3 to 5). Of parents surveyed, 81 (100%) would repeat the program with a subsequent child. There were three (3/232 or 1.3%) minor complications: one child with a persistent rash that resolved with termination of orthosis use, one child with a skin irritation that resolved with orthosis recontouring, and one child who could not be successfully fit. CONCLUSIONS: The CranioCap, a new cranial orthosis with clearance for treatment of deformational plagiocephaly, appears to be safe, well tolerated, and has excellent parent satisfaction.

Epilepsy surgery in infancy. A review of four cases.

It has been demonstrated that intractable seizures in children can be eliminated or become more responsive to anticonvulsant medication after surgical resection of the epileptogenic focus or dysfunctional hemisphere. We describe our surgical experience with 4 infants treated at Children's Healthcare of Atlanta Egleston Hospital between 1994 and 2002. All infants, ranging in age from 5 to 9 months, presented with severe seizure disorders and failed trials of anticonvulsants. All had preoperative EEG monitoring and MRI studies; 2 had PET functional imaging. One infant underwent a temporal resection for a low-grade glioma. The rest had cortical resections for malformations and dysplasia. All had improvement, with 2 infants free of seizures off medication. We argue for early intervention in severe cases, as the potential for recovery can be dramatic.