Haploidentical Transplant Achieves Long-Term Remission in Relapsed Refractory Leukemia With Fludarabine-Induced Neurotoxicity Complication.

Hematopoietic stem cell transplant is potentially curative for relapsed/refractory leukemia. However, neurotoxicity is common and has been reported in 11% to 59% of children following hematopoietic stem cell transplant. Most pediatric studies of the neurological effects of hematopoietic stem cell transplant have focused on acute neurotoxicity. Limited information is available for long-term neurotoxicity, particularly those cases that are severe and permanent and caused by conditioning chemotherapy. Here, we report 2 cases of relapsed acute lymphoblastic leukemia that achieved long-term remission by haploidentical hematopoietic stem cell transplant but remained complicated with severe and persistent fludarabine-induced neurotoxicity.

Recommendations on the Management of Interhospital Transport of Pediatric Patients With Mediastinal Mass.

PURPOSE: Children with mediastinal masses often present with insidious symptoms to nonspecialist centers and require interhospital transport to oncology centers for definitive care. We evaluated clinical characteristics and patient outcomes and proposed a management protocol. MATERIALS AND METHODS: This is a retrospective review of all children with mediastinal mass at the pediatric intensive care unit of the Hong Kong Children's Hospital between April 2019 and March 2020. RESULTS: Ten children with a median age of 14.5 years (interquartile range, 9.3-17.0 years) were included. Leukemia and lymphoma accounted for the majority of cases (n = 6, 60%). Nearly all patients (n = 9, 90%) required interhospital transport before definitive treatment could be instituted. There were no deaths, but 2 patients were transported with significant respiratory compromise. Among patients requiring more than 1 interhospital transport, there was a higher incidence of shortness of breath (100% vs 40%; odds ratio, 33; P = 0.048) and orthopnea (80% vs 0%; odds ratio, 33; P = 0.048), whereas none had a neck mass (0% vs 80%; odds ratio, 0.03; P = 0.048). CONCLUSIONS: Children with mediastinal mass are at risk of life-threatening cardiorespiratory compromise. Pretransport assessment, planning, and stabilization along with clear management plans for deterioration during transport are crucial especially for patients who are symptomatic at time of presentation, to reduce risks associated with delays in arriving at the specialist point of care for definitive treatment.

Neurometabolic Disorders of the Newborn.

There is an extensive and diverse set of medical conditions affecting the neonatal brain within the spectrum of neurometabolic disorders. As such, their clinical presentations can be rather nonspecific, and can often mimic acquired entities such as hypoxic-ischemic encephalopathy and sepsis. Similarly, the radiological findings in these entities can also be frequently nonspecific, but a more detailed analysis of imaging findings (especially magnetic resonance imaging) alongside the relevant clinical details can be a rewarding experience, thus enabling a timely and targeted diagnosis. Early diagnosis of an underlying neurometabolic disorder is vital, as some of these entities are potentially treatable, and laboratory and genetic testing can be precisely targeted. Further, their detection helps with counselling families for future pregnancies. We present a review of neurometabolic disorders specific to the newborns with a focus on how neuroimaging findings match their clinical presentation patterns.

Morphological variability of inferior alveolar nerve in low-grade craniofacial microsomia.

Craniofacial microsomia (CFM) involves asymmetric hypoplasia and dysmorphogenesis of the facial skeleton. Certain aspects of CFM may be treated by surgical osteotomy and distraction osteogenesis (DO). Mandibular osteotomy places the inferior alveolar nerve at risk. The aim of this study was to investigate radiological landmark relationships to the anatomy of the inferior alveolar nerve in CFM. Application of this understanding will aid intraoperative protection of the inferior alveolar nerve. Six subjects with similar presentations of hemifacial microsomia were selected. Three-dimensional reconstruction CT images was used to locate bony structures that held important relationships with the inferior alveolar nerve. Measurements (of the normal and microsomic sides) were made between fixed landmarks: mandibular notch, mandibular foramen, condyle, back of second and third molar tooth, and mental foramen. The unaffected sides acted as controls. The distance between the normal and the microsomic sides from condyle to mandibular foramen was significantly different. The sizes of condyles differed significantly between the normal and microsomic sides. Most of the remainder of the vertical distance of the ramus and most of the horizontal distance of the body were similar. In conclusion, the inferior alveolar nerve should be found in very similar locations on both normal and microsomic sides in low-grade hemifacial microsomic patients.