The Anesthetic Challenges of Caring for a Pediatric Patient With Incontinentia Pigmenti: A Case Report.

Incontinentia pigmenti (IP) is a rare X-linked dominant disorder. We present a case of an infant with IP who was brought to the operating room for panretinal diode photocoagulation under general anesthesia. The anesthesia team was unable to obtain intravenous access even with instruments such as a vein finder and ultrasound. Anesthesia for IP patients also poses challenges such as prevention of the oculocardiac reflex, obesity and airway management, and preemptive measures for intravenous access due to skin manifestations. Patients with IP may present with many challenges for the anesthesiologist during all phases of anesthetic management.

Spinal arteriovenous malformation in a pediatric patient with a history of congenital syphilis: a case report.

BACKGROUND: Spinal arteriovenous malformations in children are extremely rare and pose great risk for intraoperative hemorrhage. Congenital syphilis sometimes presents with vascular symptoms, however, there is little published on patients with a history of congenital syphilis presenting with spinal arteriovenous malformations. CASE PRESENTATION: A 15-month-old female with a history of congenital syphilis presented with urinary retention, fever, and subacute onset of paraplegia. MRI showed a lesion at T8-L1, angiogram was performed which confirmed the presence of a complex type IVc arteriovenous malformation and fistula from Artery of Adamkiewicz at L1-L2. It also showed peri medullary dilated veins and a pseudoaneurysm that compressed the spinal cord at T8-T10. Somatosensory evoked potentials and motor-evoked potentials were not recordable on the bilateral lower extremities prior to surgery. Once the patient was optimized for surgery, osteoplastic laminotomies from T6-T12 were performed. The dura was opened and the intradural, intramesenchymal hematoma was evacuated. There were two episodes of brisk arterial bleeding with hypotension during resection of the hematoma. The patient was taken to the angiography suite from the OR to successfully coil the large aneurysm. Intraoperative spinal cord monitoring remained undetectable in the bilateral lower extremities. The patient's paraplegia remained unchanged from preoperative presentation. CONCLUSION: Congenital syphilis may present with vascular changes that might impact surgical approaches and treatment outcomes in patients with spinal arteriovenous malformations. Preparation for massive transfusion and intraoperative monitoring are imperative in ensuring a safe perioperative experience.

Anesthesia and the Developing Brain: A Review of Sevoflurane-induced Neurotoxicity in Pediatric Populations.

PURPOSE: For over 150 years of anesthetic practice, it was believed that the effects of general anesthetics were temporary and not adverse. A growing number of studies over the past 2 decades, however, have identified structural and cognitive abnormalities, especially in the developing brain. Despite the growing evidence of anesthetic-induced neurotoxicity in animal studies, the evidence to date in humans has been inconsistent and unclear. Sevoflurane, a commonly used inhalational agent in pediatric anesthesia, is an agent of choice for inhalational induction due to its rapid activity and low blood-gas solubility. With evaluation of the current literature, improved considerations can be made regarding the widespread use of sevoflurane as an anesthetic. METHODS: PubMed database was searched for article published between 1969 through 2020. The reference lists of identified articles were searched manually for additional papers eligible for inclusion. This review addressed the tolerability of sevoflurane in specific populations, particularly pediatrics, and is divided into 3 parts: (1) the history of sevoflurane use in anesthetic practice and the pharmacokinetic properties that make it advantageous in pediatric populations; (2) proposed mechanisms of anesthesia-induced neurotoxicity; and (3) considerations due to potential adverse effects of sevoflurane in both short and long procedures. FINDINGS: There is reason for concern regarding the neurotoxic effects of sevoflurane in both the pediatric and elderly populations, as spatial memory loss, developmental deficits, and an enhanced risk for Alzheimer disease have been linked with the use of this popular inhalational agent. IMPLICATIONS: The duration and dose of sevoflurane may need to be altered, especially in longer procedures in pediatric populations. This may change how sevoflurane is administered, thus indicating a greater demand for an understanding of its limitations as an anesthetic agent.

Grisel's syndrome: a case report on this rare pediatric disease and its anesthetic challenges.

BACKGROUND: Grisel's syndrome is a non-traumatic atlantoaxial subluxation associated with inflammatory conditions of the head and neck, which occurs primarily in children. Increased flexibility of the ligaments during inflammation is implicated in the pathogenesis of the subluxation between the axis and atlas. The potential sequelae may be severe, and early diagnosis and treatment of Grisel's syndrome can prevent tragic outcomes. CASE PRESENTATION: We present a case of torticollis in an 8-year-old child. She had a two-week history of a streptococcal throat infection. The patient was treated with several different methods of conservative care, including muscle relaxation, cervical halter traction, and Halo application. However, the torticollis persisted. The patient then required surgical correction involving cervical spine fusion. She had no complications and experienced no reoccurrence of the torticollis to date. CONCLUSION: Grisel's syndrome is a pathology for which conservative management is successful in most cases. Cases requiring surgical intervention are rarely documented in the literature. Our case is significant, as in spite of aggressive conservative management, the patient required surgical correction. Patients requiring surgical management of Grisel's syndrome may require additional anesthetic exposure for diagnostic interventions like magnetic resonance imaging or neck manipulations for closed reduction. We discuss the features of Grisel's syndrome and specific anesthetic management considerations for procedures such as magnetic resonance imaging, application of cervical traction, and surgical correction of torticollis.