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.

Posterior cervical spinal fusion in a 3-week-old infant with a severe subaxial distraction injury.

Unstable spinal injuries in the neonate pose particular challenges in the clinical and radiographic assessment as well as the surgical stabilization of the spine. In this report, the authors present the unfortunate case of a 3-week-old infant who suffered a severe subaxial cervical fracture dislocation with spinal cord injury that occurred as a result of nonaccidental trauma. Imaging demonstrated severe distraction at C5-6 and near-complete spinal cord transection resulting in quadri-paresis. Open surgical reduction was performed with noninstrumented posterior fusion augmented with split rib autograft and recombinant human bone morphogenetic protein-2. Postoperative imaging demonstrated progressive bony fusion at 2 months, and clinical examination findings progressed to a motor examination classification of ASIA C. At 2 years, the fusion mass is stable and cervical alignment is maintained. The patient remains flaccid in the bilateral lower extremities, but has movement with some dexterity in both hands. Follow-up MRI shows severe spinal cord injury with evidence of bilateral C-5 nerve root avulsions. This case represents the first report of spinal fusion in an infant of less than 1 month of age. Given the extreme young age of the patient, the diagnostic challenges as well as the mechanical and technical considerations of surgical fusion are discussed.

Opioid overdose in a child: case report and discussion with emphasis on neurosurgical implications.

In environments in which opioids are increasingly abused for recreation, children are becoming more at risk for both accidental and nonaccidental intoxication. In toxic doses, opioids can cause potentially lethal acute leukoencephalopathy, which has a predilection for the cerebellum in young children. The authors present the case of a 2-year-old girl who suffered an accidental opioid overdose, presenting with altered mental status requiring cardiorespiratory support. She required emergency posterior fossa decompression, partial cerebellectomy, and CSF drainage due to cerebellar edema compressing the fourth ventricle. To the authors' knowledge, this is the first report of surgical decompression used to treat cerebellar edema associated with opioid overdose in a child.

Intracranial malignant triton tumor in a patient with neurofibromatosis type 1: case report and review of the literature.

We report the fourth case of an intracranial malignant triton tumor not associated with a cranial nerve in a 26-year-old male with a clinical history of neurofibromatosis type 1. The patient was found unresponsive and displayed confusion, lethargy, hyperreflexia, and dysconjugate eye movements upon arrival at the emergency room. MRI revealed a large bifrontal mass. Biopsy demonstrated a high-grade spindle cell tumor with focal areas of rhabdomyoblasts that stained positive for desmin, myogenin, and muscle-specific actin. Electron microscopy showed skeletal muscle differentiation. Based on the clinical history of NF1 and the pathologic results, a diagnosis of malignant triton tumor was made. The differential diagnosis, immunohistochemistry, molecular genetics, and treatment of malignant triton tumor are reviewed.

Shunt infection in the first year of life.

OBJECT: Children younger than 1 year of age are unique in their physiology and comorbidities. Reports in the literature suggest that the risk factors for shunt infection may be different in this population compared with older children. Importantly, these infants often have other congenital malformations requiring various surgical interventions, which impose an additional risk of infection. METHODS: In the 3-year period between 2008 and 2010, 270 patients underwent initial CSF shunt placement during the 1st year of life. Clinical characteristics, hospital course, and shunt infections were prospectively recorded in the practice and hospital electronic medical record. Special attention was given to types and timing of other invasive procedures and their relationship with shunt infection. RESULTS: The average gestational age was 33.6 weeks, and the average birth weight was 2333 g. The average weight at the time of shunt insertion was 4281 g. Prior to shunt insertion, 120 patients underwent 148 surgical procedures, including ventricular access device insertion (n = 63), myelomeningocele closure (n = 37), and cardiac procedures (n = 11), among others. In the 12-month period after shunt insertion, 121 of the 270 patients underwent 135 surgical procedures, which included 79 CSF shunt revisions. Shunt infection occurred in 22 patients, and organisms were identified in 20 cases. Univariate analysis showed that of the very prematurely born infants (gestational age < 30 weeks), those who underwent preshunt cardiac surgery and any surgical procedures within 30 days after the shunt insertion were at a greater risk of shunt infection. In multivariate analysis, preshunt cardiac surgery and surgical procedures within 30 days postshunt placement were significant risk factors independent of gestational age, birth weight, and history of shunt revisions. CONCLUSIONS: The results of this study suggest that surgical procedures within 30 days after shunt insertion and preshunt cardiac surgery are associated with a greater risk of shunt infection in children in whom these devices were inserted during the 1st year of life.