Surgical treatment of abdominal wall weakness and lumbar hernias in Ehlers-Danlos syndrome – Case report

Objective: This case report presents the orthopaedic impact of arthrochalasia-type Ehlers-Danlos syndrome (EDS) (former type VIIA and B) in a young girl.Methods: The characteristics of EDSs are skin abnormalities and tissue fragility with orthopaedic consequences including joint hypermobility and dislocations. In EDS arthrochalasia type (former type VIIA and B) severe general hypotonia and congenital bilateral hip dislocation are distinctive symptoms. This type of EDS is less common than other types and just a few cases have been reported.Results: This report describes a case of a 3-year old girl with EDS arthrochalasia type and bilateral high hip dislocation, spontaneous shoulder dislocation and scoliosis as orthopaedic consequences.Conclusions: Orthopaedic consequences in these cases can be severe so conservative or surgical treatment will be necessary to reduce disability in later life. Timing of either conservative or surgical intervention is essential. According to the literature, most conservative treatments fail. Also, surgical treatment is rarely effective in most of the cases. Both conservative and surgical treatment should be considered carefully in the treatment of EDS arthrochalasia type, each of which has pros and cons. The best treatment depends on the prognosis and current situation of the child.

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The role of the dental practitioner in diagnosing connective tissue and other systemic disorders: a case report of a patient diagnosed with type III Ehlers-Danlos syndrome

Oral Surgery ◽

10.1111/ors.12129 ◽

2014 ◽

Vol 8 (2) ◽

pp. 96-101 ◽

Cited By ~ 2

Author(s):

D. Awal ◽

T.W. Lloyd ◽

H.J. Petersen

Keyword(s):

Case Report ◽

Connective Tissue ◽

Ehlers Danlos Syndrome ◽

Type Iii ◽

Dental Practitioner ◽

Danlos Syndrome

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Appendicitis in puerpeium: Case report

Acta chirurgica iugoslavica ◽

10.2298/aci1104111b ◽

2011 ◽

Vol 58 (4) ◽

pp. 111-112 ◽

Cited By ~ 1

Author(s):

Milica Berisavac ◽

Biljana Kastratovic-Kotlica ◽

V. Tosic ◽

N. Markovic ◽

S. Ljustina ◽

...

Keyword(s):

Case Report ◽

Surgical Treatment ◽

Early Detection ◽

Acute Appendicitis ◽

Abdominal Wall ◽

Clinical Signs ◽

Signs And Symptoms ◽

Laboratory Infection ◽

Infection Parameters ◽

Clinical Signs And Symptoms

Acute appendicitis in puerperium is often diagnosed too late, because clinical signs can be unrelaible. Abdominal wall rigidity is rarely noticed in puerpeium because of weak abdominal wall muscles, laboratory parameters are not enough relaible and atipycal appendix presentation makes difficulties in diagnosis3,4. Knowing clinical signs and symptoms of appendicitis, possible complications and their early detection, make a chance for a good surgical outcome. Measuring of axillar and rectal temperature can take confusion in, and prolong time until surgical treatment. Leucocytosis in puerperium is not valid for diagnosis. We report a case of patient in puerperium with high laboratory infection parameters. Diagnosis of appendicitis is made based on clinical signs and symptoms, that is proved intraoperatively and histologicaly. Appendectomy without perforation carries less risks for mother and fetus.

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An Intradermal Spindle Cell Lipoma on the Plantar Hallux in a Patient with Scleroderma and Ehlers-Danlos Syndrome: A Case Report

The Journal of Foot & Ankle Surgery ◽

10.1053/j.jfas.2021.08.006 ◽

2021 ◽

Author(s):

Jason Birkel DMP ◽

Gene Mirkin DMP ◽

Xingpei Hao

Keyword(s):

Case Report ◽

Spindle Cell ◽

Spindle Cell Lipoma ◽

Ehlers Danlos Syndrome ◽

Danlos Syndrome

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Ehlers-Danlos syndrome with a spontaneous caroticocavernous fistula occluded by detachable balloon: case report and review of literature

Neuroradiology ◽

10.1007/s002340050316 ◽

1996 ◽

Vol 38 (6) ◽

pp. 595-597 ◽

Cited By ~ 1

Author(s):

P. Forlodou ◽

A. de Kersaint-Gilly ◽

J. Pizzanelli ◽

M. P. Viarouge ◽

E. Auffray-Calvier

Keyword(s):

Case Report ◽

Review Of Literature ◽

Detachable Balloon ◽

Ehlers Danlos Syndrome ◽

Caroticocavernous Fistula ◽

Danlos Syndrome

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Abdominal wall hernias

Current Surgical Guidelines ◽

10.1093/med/9780198794769.003.0054 ◽

2018 ◽

pp. 529-540

Author(s):

Abdullah Jibawi ◽

Mohamed Baguneid ◽

Arnab Bhowmick

Keyword(s):

Connective Tissue ◽

Abdominal Wall ◽

Chronic Cough ◽

Incidental Finding ◽

Clinical History ◽

The Body ◽

Abdominal Pressure ◽

Ehlers Danlos Syndrome ◽

Danlos Syndrome ◽

Groin Hernias

Hernias are abnormal protrusion of an organ through a weakness/defect in the body wall that contains it. Classifications include groin hernias, ventral abdominal wall hernias (umbilical, femoral), incisional, Spigelian, and lumbar hernias. Inguinal hernias are the commonest types of abdominal wall hernias (~75%). Male are affected 15-times more frequently. Hernias are more common in smokers, patients with underlying connective tissue disorders (Ehlers Danlos Syndrome, Marfan syndrome), and patients with increased intra-abdominal pressure (obesity, heavy lifting, chronic cough, and chronic straining during defecation and urination). Hernias present as incidental finding on imaging, asymptomatic lumps, painful lumps, or incarcerated or strangulated hernias. Clinical history and examination are the mainstay of diagnosis. Most hernias are treated with surgical repair (open or laparoscopic). Conservative wait and watch policy is indicated in some cases.

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