First Total Posterior Craniopagus Twins in Central African Republic: Two Years Challenge of Management

Introduction: Countries with limited resources have real difficulties in separating craniopagus twins. Separation surgery cannot be considered there due to lack of technological conditions and appropriate means. Objective: To show the benefits of an Italian-Central African humanitarian collaboration, after two years follow-up, aiming to improve the first childhood years of craniopagus twins, born in the Central African Republic and separated in Rome. Case Report: The two female conjoined craniopagus twin, born at term on June 29, 2018 in Mbata located about 96 km from Bangui (CAR), were referred to the neonatology unit of the CHUPB on their 3rd day of life. The mother was 31-year-old, gravida 4 and para 5. Prenatal cares were quantitatively and qualitatively poorly followed. The diagnosis of posterior conjoined craniopagus twins associated with early neonatal infection was retained. After seven days of antibiotics and regression of infectious symptoms, the newborns were kept in the neonatology unit for a closer follow-up, pending the completion of administrative procedures for a medical evacuation as part of the international humanitarian activities of the Pediatric Hospital of the Holy Seat in collaboration with the Pediatric Teaching Hospital in Bangui. Finally, the twins were transferred to the Bambino Gesu Hospital in Rome on September 10, 2018 where the 3D images confirmed the total posterior fusion. Between May 2019 and June 2020 thirty hours of interventions allowed a step-by-step separation of the twins, with a gradual rerouting of the shared blood supply, remodeling and re-creating a skin to cover the skull bones. No postoperative complications were recorded;the twins recovered satisfactorily after 6 months of separation and are awaiting discharge at their origin country where the CHUPB team will continue monitoring. Conclusion: The successful separation of total posterior craniopagus twins remains historically scarce and the guarantee of success has been based on detailed and well planned surgical management.

Current role of surgery for tuberous sclerosis complex-associated epilepsy

Tuberous sclerosis complex(TSC)is a rare multisystem,autosomal dominant neurocutaneous syndrome in which epilepsy is the most common of several neurological and psychiatric manifestations.Around two thirds of patients develop drug-resistant epilepsy for whom surgical resection of epileptogenic foci is indicated when seizures remain inadequately controlled following trial of two antiseizure medications.The challenge with presurgical and surgical approaches with patients with TSC is overcoming the complexity from the number of tubers and the multiplex epileptogenic network forming the epileptogenic zone.Data suggest that seizure freedom is achieved by 55%-60%of patients,but predictive factors for success have remained elusive,which makes for unconfident selection of surgical candidates.This article presents three different cases as illustrations of the potential challenges faced when assessing the suitability of TSC patients for epilepsy surgery.