Bent Out of Shape: A Case of Abdominal Pain

We present the case of a 9-year-old boy with abdominal pain and retching. An abdominal computed tomography revealed free air under the diaphragm, and surgical exploration showed perforation of the sigmoid colon. Ultimately, he was diagnosed with vEDS. We review the literature and discuss the clinical presentation, diagnosis, and life-threatening crises associated with vEDS.
Source: Pediatric Emergency Care - Category: Emergency Medicine Tags: Illustrative Cases Source Type: research

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AbstractHypermobile Ehlers-Danlos syndrome (hEDS) and hypermobility spectrum disorders (HSD) are heritable connective tissue disorders associated with pain, activity limitations and participation restrictions. A key feature is reported to be reduced stiffness and increased extensibility and elasticity of connective tissues. Yet diagnosis relies on assessment of joint range of motion, which may be influenced by other factors, and semi-quantitative assessment of forearm skin extensibility. The  objective of this systematic review was to determine if quantitative measures of tissue mechanics can discriminate between hEDS...
Source: Clinical Rheumatology - Category: Rheumatology Source Type: research
Conclusions There are only four reports of V-EDS causing splenic rupture in the literature to date. These patients were all adults and only one had not previously been diagnosed with V-EDS. All underwent splenectomy. While V-EDS presenting with abdominal visceral rupture in children has been reported, this is the first report of a child with V-EDS presenting with splenic rupture. It is the only case of splenic rupture secondary to V-EDS that has been managed minimally invasively by embolization. [...] Georg Thieme Verlag KG Stuttgart · New YorkArticle in Thieme eJournals: Table of contents  | &nb...
Source: European Journal of Pediatric Surgery Reports - Category: Surgery Authors: Tags: Review Article Source Type: research
In this study we provide clinical and molecular presentation of two new patients with DSE related mcEDS. Analysing clinical exome data, a homozygous pathogenic DSE variant, c.1150_1157del p.(Pro384Trpfs*9), was identified in a 32 year old man with bilateral congenital talipes equinovarus, characteristic facial features, myopia, hyperextensible skin at the elbows, significant palmar wrinkling, bilateral inguinal hernias and chronic leg, back and joint pain. Electron microscopical examination of skin biopsy showed changes consistent with mild compensatory elastic fibre hypertrophy and mildly loose collagen bundles. The varia...
Source: European Journal of Medical Genetics - Category: Genetics & Stem Cells Authors: Tags: Eur J Med Genet Source Type: research
Australian singer/songwriter Sia (born Sia Kate Isobelle Furler), 43, is known for wearing elaborate wigs and headpieces that hide her face. However, the“Chandelier” singer is not hiding the fact that she was recently diagnosed with the connective tissue disorder Ehlers-Danlos syndrome. In a  tweet, Sia wrote:“Hey, I’m suffering with chronic pain, a neurological disease, […]Find jobs at  Careers by KevinMD.com.  Search thousands of physician, PA, NP, and CRNA jobs now.  Learn more.
Source: Kevin, M.D. - Medical Weblog - Category: General Medicine Authors: Tags: Conditions Rheumatology Source Type: blogs
Ehlers-Danlos syndrome affects connective tissues that support the skin, bones, blood vessels and other organs and tissues, according to the U.S. National Institutes of Health.
Source: WebMD Health - Category: Consumer Health News Source Type: news
Ehlers-Danlos syndrome affects connective tissues that support the skin, bones, blood vessels and other organs and tissues, according to the U.S. National Institutes of Health.
Source: WebMD Health - Category: Consumer Health News Source Type: news
Lori Pinkley, a 50-year-old from Kansas City, Mo., has struggled with puzzling chronic pain since she was 15. She has had countless disappointing visits with doctors. Some said they couldn ’t help her. Others diagnosed her with everything from fibromyalgia to lipedema to the rare Ehlers-Danlos syndrome. Pinkley has taken opioids a few times after surgeries, but they never helped her […]Find jobs at  Careers by KevinMD.com.  Search thousands of physician, PA, NP, and CRNA jobs now.  Learn more.
Source: Kevin, M.D. - Medical Weblog - Category: General Medicine Authors: Tags: Meds Medications Pain Management Source Type: blogs
A 30 year-old female was admitted with a five-day history of chest and abdominal pain with vomiting and constipation . She had undergone laparoscopic Roux-en-Y gastric bypass in Chile five months prior. Her medical history included Ehlers Danlos syndrome (vascular type) and bipolar disorder. A CT chest and abdomen revealed a large diaphragmatic hernia with small bowel, gastric pouch and gastric remnant within the left hemithorax. She was transferred to St Mary ’s Hospital under the care of the Bariatric Surgery team and underwent an emergency laparoscopy.
Source: Surgery for Obesity and Related Diseases - Category: Surgery Authors: Source Type: research
Lori Pinkley, a 50-year-old from Kansas City, Mo., has struggled with puzzling chronic pain since she was 15.She's had endless disappointing visits with doctors. Some said they couldn't help her. Others diagnosed her with everything from fibromyalgia to lipedema to the rare Ehlers-Danlos syndrome.Pinkley has taken opioids a few times after surgeries but says they never helped her underlying pain."I hate opioids with a passion," Pinkley says."An absolute passion."Recently, she joined a growing group of patients using an outside-the-box remedy: naltrexone. It is usually used to treat addiction, in...
Source: Psychology of Pain - Category: Anesthesiology Source Type: blogs
We present the first case of bariatric surgery in a patient with Ehlers-Danlos syndrome and outline management challenges in the context of the relevant literature. A 56-year-old man with type IV Ehlers-Danlos syndrome and a body mass index of 41.8 kg/m2 was referred to the bariatric centre of the Churchill Hospital, Oxford, for consideration of surgery for morbid obesity. His comorbidity included type 2 diabetes, hypertension, dyslipidaemia and obstructive sleep apnoea. He underwent a laparoscopic Roux-en-Y gastric bypass. His initial recovery was uneventful and he was discharged on the first postoperative day. Six weeks ...
Source: Annals of the Royal College of Surgeons of England - Category: Surgery Authors: Tags: Ann R Coll Surg Engl Source Type: research
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