In the classic type, the skin is hyperelastic, fragile and smooth and velvety to touch. Skin is prone to rupturing even after minor trauma. The joints are hyperextendable. Joint dislocations are common. Wound healing is impaired. Other signs and symptoms include:
- Hypotonia and delayed motor development
- Easy bruising
- Spontaneous vessel ruptures
- Valvular prolapse
- Atrophic scars
In hypermobility type, grotesque contortions are seen, such as bending the thumb backwards to touch the forearm or bending the knee almost at a right angle. Chronic pain, joint dislocations and joint hypermobility are other manifestations. Bowel disorders might also be seen.
The vascular type manifests as
- Thin skin
- Arterial or uterine rupture
- Small joint hyperextensibility
In kyphoscoliosis type, the manifestations are:
- Joint laxity
- Congenital scoliosis
- Ocular fragility
In arthrochalasia variant, the following findings are present.
- Severe joint hypermobility
- Mild skin changes
The dermatosparaxis type shows up as:
- Severe skin fragility
- Cutis laxa
- Easy Bruising
AEBP1 Comprehensive EDS Panel Hypermobility Type (EDS type III) or Tenascin Deficient Type Marked large and small joint hypermobility, joint pain, easy bruising, easy bleeding, normal scars Dominant TNXB (<5%) (Not available through CDL) Vascular Type [uwcpdx.org]
Easy bruising. Pes planus. Recurrent joint dislocation.Chronic joint pains. Easy brushing. Varicose veins. Intestinal rupture. Pelvic adhesions. Positve DNA test III-5 Thin, delicate straight nose, thin lips. [laeknabladid.is]
Skin hyperextensibility, easy bruising, tissue fragility, atrophic scars, loss of muscle tone, Kyphoscoliosis, and osteopenia (bones which are less dense than normal) are also possible clinical manifestations. [rarediseases.about.com]
Ehlers-Danlos syndrome (EDS) is associated with easy bruising and bleeding complications in the majority. [ncbi.nlm.nih.gov]
Ehlers-Danlos Syndrome Symptoms Because EDS is a collection of disorders, symptoms can vary by the specific disorder; however, joint hypermobility (the ability to extend beyond the normal range of motion) and excessively stretchy skin or easy bruising [arthritis.org]
- Prolonged Bleeding
These may include: Nose bleeds lasting longer than 10 minutes despite holding pressure on the nostrils Prolonged bleeding with minor cuts, with bleeding lasting longer than 10 minutes, despite steady pressure against the wound Excessive bleeding with [nationwidechildrens.org]
For patients with a history of prolonged bleeding, referral to a hematologist to test for an unassociated bleeding diathesis and possible treatment may be necessary. [clinicaladvisor.com]
0005692 Joint stiffness Stiff joint Stiff joints [ more ] 0001387 Muscular hypotonia Low or weak muscle tone 0001252 Mutism Inability to speak Muteness [ more ] 0002300 Osteomalacia Softening of the bones 0002749 Osteopenia 0000938 Osteoporosis 0000939 Prolonged [rarediseases.info.nih.gov]
A prefabricated acrylic splint/plate is placed over the surgical site as sutures do not hold well. ,, The dentist may encounter prolonged, recurrent bleeding from the socket following a tooth extraction. , Hemostatic therapy in the form [jdrr.org]
If a history of severe or prolonged bleeding is present, consider hematologic evaluation for von Willebrand disease, thrombocytopenia, or other bleeding diathesis. [web.archive.org]
Entire Body System
- Chronic Fatigue Syndrome
EDS is considered an “invisible disability” and is often misdiagnosed as other conditions including hypochondriasis, depression, and chronic fatigue syndrome. [diseaseinfosearch.org]
Chronic fatigue is an important contributor to impaired health-related quality of life in Ehlers-Danlos syndrome. There is overlap in the symptoms and findings of EDS and chronic fatigue syndrome. [ncbi.nlm.nih.gov]
Fatigue Syndrome Fibromyaligia Postural Orthostatic Tachycardia Syndrome, Dysautonomia Prolong QT interval Chronic Bronchitis, Infection. [patch.com]
He specializes in the evaluation and treatment of adolescents and young adults with conditions characterized by chronic fatigue, including chronic fatigue syndrome, orthostatic intolerance, and joint hypermobility. [doi.org]
Chronic Fatigue Chronic Fatigue Syndrome, also known as CFS, affects people will all sorts of chronic illness. Those of us with chronic fatigue are also known as #Spoonies. See Christine Miserandino's Spoon Theory for more info on that! [buzzfeed.com]
- Marfanoid Habitus
Homocystinuria also results in a Marfanoid habitus but presents with inferior and medial displacement of the lens ( downward and inward )! [amboss.com]
habitus" which is characterized by long, slender fingers (arachnodactyly); unusually long limbs; and a sunken chest (pectus excavatum) or protruding chest (pectus carinatum) majority of patients with kEDS harbor biallelic mutations in PLOD1; recently [gpnotebook.com]
Marfanoid habitus This may be present in association with EDS (usually incomplete). It is a common finding in hypermobile and kyphoscoliotic EDS but is not necessary for diagnosis of EDS. [online.epocrates.com]
- Inguinal Hernia
A history of learning disability and bilateral inguinal hernia was present. Blood and imaging studies were unremarkable. [ncbi.nlm.nih.gov]
The musculoskeletal manifestations include joint laxity with recurrent dislocation and postural abnormalities such as kyphoscoliosis. 4 There is weakness of supporting structures with increased risk of inguinal hernias, eventration of the diaphragm, and [web.archive.org]
Children may present with pneumothorax, inguinal hernia and recurrent joint dislocation. [onlinelibrary.wiley.com]
She also reported palpitations and dyspnea caused by efforts greater than usual, and referred to sporadic dizziness but no syncopes. [scielo.br]
It may alternate with diarrhea. - Gastro-oesophagal reflux (76%) with multiple concomitant complications affecting the airways. - Dyspnea (83%) arising as a result of insignificant effort ( “the staircase sign” ). - Frequent sensations of respiratory [avensonline.org]
This explanation can be expanded towards the mechanism causing dyspnea at effort, as the mechanoreceptors of the joints do not transmit the proper signals to the respiratory centers. [symbiosisonlinepublishing.com]
However, the coexistence of persistent exertional dyspnea, unrefreshing sleep, postexertional malaise, and reduced stamina, combined with major limitations of the daily activities, likely indicates pathologic fatigue. [hindawi.com]
- Mitral Valve Prolapse
[…] problems (mitral valve prolapse and others) Other types of EDS The other types of EDS are extremely rare. [web.archive.org]
Ehlers-Danlos syndrome Cardiovascular Features of heart valve defects (particularly mitral valve prolapse) Features of aneurysms/dissections of the iliac, splenic, renal arterties, or the aorta Berry/saccular aneurysms of the cerebral arteries → features [amboss.com]
If mitral valve prolapse is diagnosed, monitoring and screening are indicated to address subacute bacterial endocarditis precautions. [emedicine.medscape.com]
Additional features include fatigue, development of hernias, increased risk of aortic root dilation, and mitral valve prolapse. Postural orthostatic tachycardia syndrome (POTS) is a type of dysautonomia that may affect patients with EDS. [medcomic.com]
" mitral valve prolapse in the ecocardiographic study. [ncbi.nlm.nih.gov]
- Spontaneous Rupture of Membranes
Problems during delivery Due to the fragility of connective tissue, those with EDS are more prone to prelabour spontaneous rupture of membranes (SROM), including preterm SROM. [patient.info]
A slightly extensible, thin and translucent skin, associated with dysmorphic facies, acrogeria, multiple ecchymoses, hypermobility of the small joints, dorsal kyphosis, genu valgum, flat feet, elongated upper limbs, and low muscle tone were all evident [ncbi.nlm.nih.gov]
Disease definition Ehlers-Danlos syndrome type IV, also known as the vascular type of Ehlers-Danlos syndrome (EDS), is an inherited connective tissue disorder defined by characteristic facial features (acrogeria) in most patients, translucent skin with [orpha.net]
Other manifestations that may be found in the Vascular Type include: acrogeria (premature aging of the skin of the hands and feet); early onset varicose veins; arteriovenousfistula (an opening between an artery and vein), carotid-cavernousfistula (abnormal [tadcoalition.org]
- Cutis Laxa
KEYWORDS: ATP6V0A2-related cutis laxa; Ehlers-Danlos-like skin lesions; autosomal recessive cutis laxa; type 2A (ARCL2A) [ncbi.nlm.nih.gov]
[…] recessive 1B EFEMP2 Del Dup NGS Cutis laxa, autosomal recessive IC LTBP4 Del Dup NGS Cutis laxa, autosomal recessive IIA ATP6V0A2 Del Dup NGS Cutis laxa, autosomal recessive IIIA ALDH18A1 Del Dup NGS Cutis laxa, autosomal recessive, type IIB & type IIIB [ctgt.net]
Van Meekeren: De dilatabilitate extraordinaria cutis. In: Observations Medico-Chirugicales. Chapter 32. Amsterdam, 1682. N. A. Chernogubow: Über einen Fall von Cutis laxa. [whonamedit.com]
Orphanet: 209 Medscape entry on Cutis Laxa [en.wikipedia.org]
Cutis laxa acquisita associated with multiple myeloma: a case report and review of the literature. Cutis. Apr 1996;57(4):267-70. [Medline]. 11.Gupta A, Helm TN. Acquired cutis laxa associated with multiple myeloma. Cutis. Feb 2002;69(2):114-8. [hoanmysaigon.com]
- Thin Skin
[…] hypermobile EDS (hEDS) loose joints easy bruising muscle pain muscle fatigue chronic degenerative joint disease premature osteoarthritis chronic pain heart valve problems Symptoms of vascular EDS fragile blood vessels thin skin transparent skin thin [healthline.com]
Thin, translucent skin (especially noticeable on the chest/abdomen) Easy bruising (spontaneous or with minimal trauma) Characteristic facial appearance (thin lips and philtrum, small chin, thin nose, large eyes) Acrogeria (an aged appearance to the extremities [uwcpdx.org]
Symptoms include: Skin that bruises very easily, thin skin with visible small blood vessels, particularly on the upper chest and legs fragile blood vessels that can bulge or tear, resulting in serious internal bleeding, a risk of organ problems, such [metro.co.uk]
People with vEDS may have: skin that bruises very easily thin skin with visible small blood vessels, particularly on the upper chest and legs fragile blood vessels that can bulge or tear, resulting in serious internal bleeding a risk of organ problems [nhs.uk]
- Delayed Wound Healing
Patients with vascular EDS are at higher risk for uterine rupture and postpartum hemorrhage. 8 Some further complications of delivery in EDS include abnormal fetal presentations, delayed wound healing, uterus atonia, hemorrhage, pelvic prolapse, deep [rheumatologyadvisor.com]
History revealed skin hyperextensibility since early childhood, presence of easy bruisability & delayed wound healing but there was no dislocation of joints or relevant family history. His development milestones were apparently normal. [ncbi.nlm.nih.gov]
wound healing with atrophic scarring.  Depending on the type, EDS can be diagnosed through laboratory studies or clinical examination. [emedicine.medscape.com]
- Subcutaneous Nodule
The disorder started at approximately age 22 with the development of subcutaneous nodules. Hyperextensibility of the skin in this region developed within the subsequent five years. [jamanetwork.com]
It is characterized by an unusual hyperelasticity and fragility of the skin and blood vessels, hypermobility of the joints, pseudotumors over the bony prominences, and disseminated, movable subcutaneous nodules. The subcutaneous nodules may calcify. [dxy.cn]
nodules due to herniation of subcutaneous fatty tissue and resemble lipomas histologically. [ncbi.nlm.nih.gov]
These are subcutaneous nodules due to herniation of subcutaneous fatty tissue and resemble lipomas histologically. The "reverse Namaskar sign"  [Figure 4], a valuable diagnostic sign has been described by Premalatha in patients with EDS. [idoj.in]
These five cardinal signs are skin fragility, blood vessel fragility, skin hyperelasticity, joint hypermobility, and characteristic subcutaneous nodules. [malacards.org]
- Blue Sclera
Other eye symptoms may include nearsightedness ( myopia ), a blueish tint in the white part of the eyes (blue sclera), and retinal detachment. [rarediseases.info.nih.gov]
However, there is no reason to think that environmental influences would not be important factors, especially since many of the individual features of connective tissue disorders, such as mitral valve prolapse, blue sclerae, pectus excavatum, dislocated [ctds.info]
sclerae, small jaw, hypertrichosis Dominant COL1A1, COL1A2 Arthrochalasia, type VII A/B (Exon 6 COL1A1/2 ) Dermatosparaxis Type (EDS type VIIC) Soft and very thin, fragile skin (tearing of the skin), stretchy skin, easy bruising, joint hypermobility [uwcpdx.org]
Blue sclera. Thin, translucient, fragile skin. Easy bruising. Pes planus. Recurrent joint dislocation.Chronic joint pains. Easy brushing. Varicose veins. Intestinal rupture. Pelvic adhesions. [laeknabladid.is]
Functional bowel disorders (functional gastritis, irritable bowel syndrome) Gastroparesis Nerve compression disorders (carpal tunnel syndrome, acroparesthesia, neuropathy) Vascular skin conditions: Raynaud's phenomenon, Livedo reticularis Blue sclera [medicalmarijuana.com]
- Joint Dislocation
The joints are hyperextendable. Joint dislocations are common. Wound healing is impaired. [symptoma.com]
Causes generalized joint hypermobility, frequent joint dislocations, skin hyperelasticity, easy bruising, tissue fragility, low muscle tone and low bone mineral density. [gillettechildrens.org]
Eye involvement Scoliosis common (curved spine) Arthrochalasia Type VII A and B 3% of all cases Noticeable joint hypermobility but less skin involvement Spontaneous joint dislocation common Dermatosparaxis Type VII C Other Type V 5% Extensive skin hyperelasticity [dermnetnz.org]
For example, overly flexible joints can result in joint dislocations and early-onset arthritis. Fragile skin may develop prominent scarring. [mayoclinic.org]
Symptoms of EDS include: Back pain Double-jointedness Easily damaged, bruised, and stretchy skin Easy scarring and poor wound healing Flat feet Increased joint mobility, joints popping, early arthritis Joint dislocation Joint pain Premature rupture of [medlineplus.gov]
The specific EDS type most associated with arthritis is hypermobility type (hEDS). This type is known for frequent joint dislocation, degenerative joint disease and chronic pain. [arthritis.org]
For more information on parent advocacy contact your local chapter of the Arthritis Foundation or the American Juvenile Arthritis Organization (AJAO). [orthop.washington.edu]
- Hip Dislocation
Clinical description The disease is marked by bilateral congenital hip dislocation, hyperlaxity of the joints, and recurrent partial dislocations. Other signs include hyperextensible skin, fragile tissues with atrophic scars, and muscular hypotonia. [orpha.net]
During follow-up at 18 months of age, strabismus, umbilical hernia, kyphoscoliosis, joint laxity, bilateral hip dislocation, muscular hypotonia, and motor developmental delay. [ncbi.nlm.nih.gov]
Arthrochalasia Type The distinguishing feature of the arthrochalasia type of Ehlers-Danlos is congenital hip dislocation. Severe joint hypermobility with recurrent subluxations is common. [rarediseases.about.com]
[…] syndrome A lot - including some interesting biochemical anomalies known to be heavily influenced by nutrition Here is a partial list of overlapping features found in most of the disorders listed above: Mitral valve prolapse Low Bone Densities Osteopenia Osteoporosis [ctds.info]
[…] prevalent in hypermobile EDS, shows a higher prevalence in a study of patients with hypermobility.2 Its correlation has been demonstrated with chronic fatigue syndrome, fibromyalgia7 anxiety, depression, panic attacks, agoraphobia2,6,8 dysautonomia and osteoporosis [reumatologiaclinica.org]
Osteoporosis and Dysautonomia in the Joint Hypermobility Syndrome. San Juan de Dios Hospital, Rheumatology Dept., Professor of Medicine. University of Chile Medical School, Santiago, Chile, Microsoft Power Point presentation, 97-2003. 3. [mthfrheds.com]
Examples include early onset arthritis, osteoporosis, and an inability to stand for more than a few minutes. In extreme cases, patients simply cannot stand unassisted and require a cane or wheelchair for mobility. [massroots.com]
- Neck Pain
Some patients present with symptoms, including local mechanical neck pain neurologic symptoms, in some patients with mechanical neck pain, Os odontoideum may be detected on radiographs. [pediatricneurosciences.com]
Neck pain. Having a gentle massage therapist who is knowledgeable about EDS can be crucial. Just another reason we need to spread awareness! 12. [buzzfeed.com]
There are also many neurological and spinal problems connected with EDS; early onset arthritis, scoliosis, neck pain, headaches, and fatigue are all common symptoms, Dr. Austin adds. [instyle.com]
Braces can be used to improve stability A soft neck collar may help decrease neck pain A wheelchair may help offload stress on lower extremity joints. [morphopedics.wikidot.com]
Care taken here might prevent post-operative complications, such as neck pain and compression-related neurological symptoms. Elective fibreoptic intubation should be considered when difficulties are anticipated. [ncbi.nlm.nih.gov]
The clinical evaluation and family history are important diagnostic tools. The qualitative and quantitative analysis, sequencing and biochemical testing can establish the type of Ehlers Danlos syndrome present in the patients. Deletion and duplication testing can also be done to pinpoint the mutations that have occurred. Prenatal diagnosis pre implantation genetic diagnosis (PGD) for at-risk pregnancies can also be done.
- Platelet Aggregation Abnormal
ROTEM™), platelet aggregation abnormalities can be expected in about 26% of patients [[ 21 ]]. New measures of platelet function such as PFA-100™ or Multiplate™ show normal values and should not be used routinely. [ncbi.nlm.nih.gov]
ROTEM™), platelet aggregation abnormalities can be expected in about 26% of patients []. New measures of platelet function such as PFA-100™ or Multiplate™ show normal values and should not be used routinely. [ojrd.biomedcentral.com]
Conservative treatment of Ehlers Danlos syndrome is possible with the following:
- Braces for joint support to prevent dislocation
- Appropriate therapy for gastritis/reflux /delayed gastric emptying/irritable bowel syndrome
- Beta-blockers for progressive aortic enlargement
- Analgesics for chronic pain
- Pain-oriented counseling
- Exercise to improve muscle tone 
- Calcium and vitamin D supplements to increase bone density
- Echocardiogram for pregnant women with known aortic root dilation.
- Genetic counseling
Symptomatic treatment of bone, gastrointestinal, dental, and hematologic manifestation should be done accordingly  .
The surgical treatment is delayed in favor of physiotherapy for as long as possible. Long-term improvement in shoulder stability with Achilles tendon allograft reconstruction of the joint capsule has been reported.
The prognosis is poor for the patients suffering from the vascular variety of Ehlers Danlos syndrome. Complications leading to morbidity and sudden death are often seen.
Prognosis is better for the other types of Ehlers Danlos syndrome, although there is reduced quality of life.
Based on the clinical and molecular patterns, Ehlers Danlos syndrome has been subdivided into six clinical forms.
Genes other than those coding for collagen have been hypothesized to be involved in the classical type of Ehlers Danlos syndrome. In 30 to 50% of these cases, mutations in the genes COL5A1 AND COL5A2 that encode type V collagen have been observed  . It is autosomal dominant.
It is also an autosomal dominant disorder. The gene involved in its causation has not been detected as yet.
An autosomal dominant disorder, the vascular type arises due to the mutations in COL31A gene encoding the type III collagen .
Inherited as an autosomal recessive trait, kyphoscoliosis type arises due to the mutations in the genes encoding lysyl hydroxylase, an enzyme responsible for hydroxylation of lysine residues during collagen synthesis.
Having an autosomal dominant pattern of inheritance, the arthrochalasia variant of Ehlers Danlos syndrome arises due to the mutations in COL1A1 or COL1A2 genes, resulting in formation of structurally abnormal pro α 1 or pro α 2 chains that resist cleavage of N-terminal peptides, a crucial step in type I collagen synthesis.
It is an autosomal recessive disorder, caused by defect in the conversion of procollagen to collagen.
Being a genetic disorder, the incidence of Ehlers Danlos syndromes, worldwide is 1:5,000 people worldwide. The hypermobility and classical variants are more common. Hypermobility Ehlers Danlos syndrome affects 1 in 10,000 to 15,000 while classical type occurs in as many as 1 in 20,000 to 40,000 people worldwide. Other forms of Ehlers Danlos syndrome are even rarer.
The disease is equally prevalent in all races. Ehlers Danlos syndrome is inherited in autosomal pattern . It occurs without the involvement of sex chromosomes, therefore both sexes are equally affected. Although present at birth, it becomes apparent much later.
Median life expectancy for patients with vascular type Ehlers Danlos syndrome is 50 years. Average life span of patients with Ehlers Danlos syndrome is 50 years. Only the spontaneous rupturing of vessels and intestines can pose life threatening risks. Uterine rupture during pregnancy can also endanger maternal and neonatal life.
Collagen is an integral part of the connective tissue. Faulty synthesis and functioning can give rise to hyperextensibility of the skin. As a result, grotesque deformities arise. Joint dislocations become common. Skin becomes lax and prone to injuries.
Collagen deficiency in arterial wall causes thinning of vascular walls which can rupture spontaneously. Being a part of scar tissue, fault in the laying down of scars after injury can lead to delayed wound healing and easy bruising even after minor injuries.
Type I collagen is present in muscles. Its deficiency leads to impairment of motor functions and difficulty in starting and carrying out the movements (hypotonia). Cramps, easy fatigability and malaise ensue. Being part of the connective tissue of the valves, deficient and faulty collagen can also lead to dysfunction of the valves.
The vitreous humor of eye also contains collagen, the deficiency of which can lead to visual disturbances. Being part of the bone and cartilage, deficiency of collagen also induces bony deformities like scoliosis.
Constant surveillance of at risk population with family history of Ehlers Danlos syndrome should be done as a primary preventive measure. Resistance exercise can increase muscle tone. High impact activities should be avoided. Crutches, canes, and walkers should be used but with caution, as they tend to exert pressure on the upper extremities.
Secondary interventions, like calcium and vitamin D supplementation should also be done.
Ehlers Danlos syndromes are a genetically inherited, heterogeneous group of disorders that arise due to abnormality of collagen fibers . There are several different types of Ehlers Danlos syndrome depending upon the type of the abnormality . The abnormality may arise in the structure or synthesis of collagen or in the proteins that closely interact with collagen. The defect may also arise in one of the proteins closely associated with collagen, like those taking part in its post transcriptional modification.
Collagen provides basic structural support to the connective tissue and is found in tendons, ligaments, vascular wall, cornea, cartilage, bone, gut, muscles and in the skin in abundance. Therefore, the changes in the structure and functions of collagen can lead to the manifestation of a wide range of clinical symptoms.
Ehlers Danlos syndrome is a syndrome in which anomalies arise in skin and other soft tissues of the body. The basic fault lies in the genetic makeup of the individual therefore, Ehlers Danlos syndrome tends to run in families.
As basic structural support of organs is lost in Ehlers Danlos syndrome, therefore, patient experiences signs such as thin and fragile skin, ability to bend the joints to degrees that are not normally possible, easy dislocation of joints, easy bruising even after minor injuries, bursting of blood vessels, difficulty in starting and maintaining movements, easy fatigability and delayed wound healing.
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- Parikh F, Sivaramakrishnan A, Pai-dhungat JV. Type VI Ehler Danlos Syndrome. The Journal of the Association of Physicians of India. Aug 2004;52:631.
- Ciarloni L, Perrigouard C, Lipsker D, Cribier B. [Ehler-Danlos syndrome type VIII]. Annales de dermatologie et de venereologie. Mar 2010;137(3):194-197.
- Wenstrup RJ, Florer JB, Willing MC, et al. COL5A1 haploinsufficiency is a common molecular mechanism underlying the classical form of EDS. American journal of human genetics. Jun 2000;66(6):1766-1776.
- Wenstrup RJ, Florer JB, Davidson JM, et al. Murine model of the Ehlers-Danlos syndrome. col5a1 haploinsufficiency disrupts collagen fibril assembly at multiple stages. The Journal of biological chemistry. May 5 2006;281(18):12888-12895.
- Eder J, Laccone F, Rohrbach M, et al. A new COL3A1 mutation in Ehlers-Danlos syndrome type IV. Experimental dermatology. Mar 2013;22(3):231-234.
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- Bathen T, Hangmann AB, Hoff M, Andersen LO, Rand-Hendriksen S. Multidisciplinary treatment of disability in ehlers-danlos syndrome hypermobility type/hypermobility syndrome: A pilot study using a combination of physical and cognitive-behavioral therapy on 12 women. American journal of medical genetics. Part A. Dec 2013;161A(12):3005-3011.
- Bergqvist D, Bjorck M, Wanhainen A. Treatment of vascular Ehlers-Danlos syndrome: a systematic review. Annals of surgery. Aug 2013;258(2):257-261.
- Jones ML. Orthodontic treatment in Ehlers-Danlos syndrome. British journal of orthodontics. Jul 1984;11(3):158-162.