Sudden death and the forensic evaluation of neurocutaneous syndromes

Jensen, Lisbeth; Heath, Karen; Scott, Grace; Byard, Roger W.

doi:10.1016/j.jflm.2009.04.004

Cited by 8 publications

(4 citation statements)

References 50 publications

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“…36 For anesthetic management, it is crucial to prevent a rise in intraocular and intracranial pressure in conjunction with minimal airway manipulation to prevent seizure attacks during anesthesia induction. Furthermore, the therapeutic level of antiepileptics and perioperative use of anesthetics with anticonvulsant property should be considered.…”

Section: Discussionmentioning

confidence: 99%

Correction of Facial Deformity in Sturge–Weber Syndrome

Yamaguchi

Lonic

Chen

et al. 2016

Plastic and Reconstructive Surgery - Global Open

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Background:Although previous studies have reported soft-tissue management in surgical treatment of Sturge–Weber syndrome (SWS), there are few reports describing facial bone surgery in this patient group. The purpose of this study is to examine the validity of our multidisciplinary algorithm for correcting facial deformities associated with SWS. To the best of our knowledge, this is the first study on orthognathic surgery for SWS patients.Methods:A retrospective chart review included 2 SWS patients who completed the surgical treatment algorithm. Radiographic and clinical data were recorded, and a treatment algorithm was derived.Results:According to the Roach classification, the first patient was classified as type I presenting with both facial and leptomeningeal vascular anomalies without glaucoma and the second patient as type II presenting only with a hemifacial capillary malformation. Considering positive findings in seizure history and intracranial vascular anomalies in the first case, the anesthetic management was modified to omit hypotensive anesthesia because of the potential risk of intracranial pressure elevation. Primarily, both patients underwent 2-jaw orthognathic surgery and facial bone contouring including genioplasty, zygomatic reduction, buccal fat pad removal, and masseter reduction without major complications. In the second step, the volume and distribution of facial soft tissues were altered by surgical resection and reposition. Both patients were satisfied with the surgical result.Conclusions:Our multidisciplinary algorithm can systematically detect potential risk factors. Correction of the asymmetric face by successive bone and soft-tissue surgery enables the patients to reduce their psychosocial burden and increase their quality of life.

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Section: Discussionmentioning

confidence: 99%

Correction of Facial Deformity in Sturge–Weber Syndrome

Yamaguchi

Lonic

Chen

et al. 2016

Plastic and Reconstructive Surgery - Global Open

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“…While haemangiomas are vascular lesions that are most often found in infants and children, where they may be solitary and transient or part of more generalised or syndromic conditions, 16,17 haemangiomas and vascular malformations may also be found in in adults. Although usually incidental, they may rarely be involved in the mechanism of death if, for example, they develop in critical locations such as the upper airway.…”

Section: Discussionmentioning

confidence: 99%

Vascular lesions simulating bruising

Byard

Bellis²,

Langlois

2016

Med Sci Law

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Careful examination of the skin and soft tissues is a routine part of the forensic autopsy to identify and characterise traumatic lesions. Three cases are reported where benign vascular lesions presented as bruises. In case 1, a 44-year-old man with a history of epilepsy was found dead on the floor. Dissection of the neck revealed a haemorrhagic area 15 mm × 1.5 mm, deep to the left thyrohyoid muscle. In case 2, an 87-year-old man was found dead with a plastic bag over his head attached to an opened cylinder of nitrogen gas. On the mucosal surface of his upper lip, there was an apparent 5 mm blue/purple bruise. In case 3, examination of the back of a 16-month-old girl revealed an apparent 15 mm bruise. Histological examination of all three lesions revealed benign vascular lesions. In the absence of microscopy, these lesions would have been considered traumatic in origin, raising the possibility of inflicted trauma.

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“…Epistaxis is common, in contrast to other neurocutaneous syndromes, with one study of the acute complications showing that epistaxis accounted for 36.5% of emergency presentations . Deaths usually, however, result from epilepsy, brain abscess, or intracranial or gastrointestinal hemorrhage . The average age for the first episode of nasal bleeding is 12 years, with a mean frequency of 18 episodes per month .…”

Section: Specific Entities Associated With Epistaxismentioning

confidence: 99%

Lethal Epistaxis

Byard

2016

Journal of Forensic Sciences

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Epistaxis or nosebleed refers to bleeding from the nostrils, nasal cavity, or nasopharynx. Occasional cases may present with torrential lethal hemorrhage. Three cases are reported to demonstrate particular features: Case 1: A 51-year-old woman with lethal epistaxis with no obvious bleeding source; Case 2: A 77-year-old man with treated nasopharyngeal carcinoma who died from epistaxis arising from a markedly neovascularized tumor bed; Case 3: A 2-year-old boy with hemophilia B who died from epistaxis with airway obstruction in addition to gastrointestinal bleeding. Epistaxis may be associated with trauma, tumors, vascular malformations, bleeding diatheses, infections, pregnancy, endometriosis, and a variety of different drugs. Careful dissection of the nasal cavity is required to locate the site of hemorrhage and to identify any predisposing conditions. This may be guided by postmortem computerized tomographic angiography (PCTA). Despite careful dissection, however, a source of bleeding may never be identified.

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Sudden death and the forensic evaluation of neurocutaneous syndromes

Cited by 8 publications

References 50 publications

Correction of Facial Deformity in Sturge–Weber Syndrome

Correction of Facial Deformity in Sturge–Weber Syndrome

Vascular lesions simulating bruising

Lethal Epistaxis

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