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Type I neurofibromatosis is characterised by altered skin pigmentation and the growth of benign tumours, particularly along the peripheral nerves and central nervous system. We report a 36-year-old woman in labour who was admitted to the obstetric suite of the Hospital Sant Joan de Déu, Barcelona, Spain, in 2007 with hypothyroidism, type I neurofibromatosis and a factor V Leiden mutation. Due to a lack of cranial and spinal imaging data, an epidural was not indicated; instead, continuous intravenous remifentanil analgaesia was administered. The remifentanil infusion was self-titrated by the patient using a visual analogue scale, with the dosage ranging from 0.01 to 0.25 μg/kg/minute. Due to rotational dystocia, Kjelland-type forceps were used during the delivery. After birth, the infant was found to have Apgar scores of 9 and 10, with no maternal or neonatal adverse effects observed. Although still controversial, remifentanil may be a successful alternative for analgaesia in similar cases; however, the specific risks and benefits for each patient should be considered prior to administration.
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http://dx.doi.org/10.18295/squmj.2017.17.04.016 | DOI Listing |
Cureus
August 2025
Thoracic Surgery, Tohoku Medical and Pharmaceutical University, Sendai, JPN.
Neurofibromatosis type 1 (NF-1) is an autosomal dominant disorder associated with vascular abnormalities, including spontaneous hemothorax and arterial aneurysms. We present a rare case of spontaneous hemothorax in which an apparently hemostatic sub-pleural hematoma began to bleed again after the patient was repositioned. A 47-year-old man with NF-1 presented with the sudden onset of left-sided chest pain.
View Article and Find Full Text PDFJ Neuroradiol
September 2025
Department of Nuclear Medicine, General Hospital of Ningxia Medical University, 804 Shengli Street, Xingqing District, Yinchuan 750004, China. Electronic address:
Neurosci Biobehav Rev
September 2025
Murdoch Children's Research Institute, Melbourne, Australia; Department of Pediatrics, University of Melbourne, Melbourne, Australia.
This systematic review was conducted to provide a comprehensive summary of biopsychosocial factors associated with attention-deficit/hyperactivity disorder (ADHD) in children and adolescents with Neurofibromatosis Type 1 (NF1), and identify key limitations and gaps in the current literature. Systematic literature searches were conducted in Scopus, PsycINFO, Web of Science, PubMed, and ProQuest Dissertations and Theses Global in March 2024. The searches identified 2,345 unique articles.
View Article and Find Full Text PDFJBJS Case Connect
July 2025
Department of Orthopaedics, All India Institute of Medical Sciences, Rishikesh, India.
Case: A 12-year-old girl with neurofibromatosis type 1 presented with progressive thoracic scoliosis and neurological deficit. Imaging revealed a dystrophic curve, dorsal syrinx, and tethering of the cord by a plexiform neurofibroma arising from the T7 dorsal ramus. She underwent staged surgery: detethering through T6-T8 laminectomy, followed by posterior spinal deformity correction with Schwab type 2 osteotomies and instrumentation.
View Article and Find Full Text PDFDev Med Child Neurol
September 2025
Murdoch Children's Research Institute, Parkville, VIC, Australia.
Aim: To examine visual engagement to social stimuli and response to joint attention in young children with neurofibromatosis type 1 (NF1) and typically developing peers (controls).
Method: Forty-five preschool children were studied cross-sectionally (mean age [SD] = 4 years 3 months [10 months]), 25 with NF1 and 20 typically developing controls. Participants passively viewed two eye-tracking paradigms.