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Journal of Indian Association of Pediatric Surgeons
     Journal of Indian Association of Pediatric Surgeons
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Year : 2020  |  Volume : 25  |  Issue : 4  |  Page : 259-260

Trivial trauma causing large intramuscular hematoma in a child with undiagnosed vascular Ehlers–Danlos syndrome

Department of Pediatric Surgery, SDM college of Medical Sciences, Dharwad, Karnataka, India

Date of Submission30-Aug-2019
Date of Decision07-Nov-2019
Date of Acceptance21-Dec-2019
Date of Web Publication24-Jun-2020

Correspondence Address:
Dr. Akshay B Kalavant
Department of Pediatric Surgery, Special Clinic, SDM College of Medical Sciences and Hospital, Satur, Dharwad, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiaps.JIAPS_147_19

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How to cite this article:
Hongal P, Kalavant AB. Trivial trauma causing large intramuscular hematoma in a child with undiagnosed vascular Ehlers–Danlos syndrome. J Indian Assoc Pediatr Surg 2020;25:259-60

How to cite this URL:
Hongal P, Kalavant AB. Trivial trauma causing large intramuscular hematoma in a child with undiagnosed vascular Ehlers–Danlos syndrome. J Indian Assoc Pediatr Surg [serial online] 2020 [cited 2022 Aug 8];25:259-60. Available from: https://www.jiaps.com/text.asp?2020/25/4/259/287654


Heritable disorders of connective tissue are often masked at presentation to clinicians; diagnosis may be overlooked if the subtle features of the disease are not detected. The following case discusses one such rare condition in an adolescent boy, who was diagnosed with Ehlers–Danlos syndrome. A 14-year-old boy presented to the emergency room with the complaints of pain and swelling over his left calf for 1 day following a trivial trauma at his school. There was no fever or medical history of bleeding disorders in his family. A physical examination showed stable vitals, but a tender swelling over the left calf region suggestive of intramuscular hematoma [Figure 1]. No other external injuries were present. The rest of the systemic examination was normal. The severity of the injury did not correlate with the history provided. The possibility of a high-velocity injury was scrutinized. A thorough general examination unveiled hallmark features of Ehlers–Danlos syndrome such as hyperextensibility of the skin, hypermobility of the joints, pes planus, and multiple scar marks of previous trauma. The patient did not have Marfanoid features. Computed tomography angiography of the left lower limb showed hematoma in the adductor compartment of the left thigh extending into the muscular compartment of the leg. A clinical diagnosis of Ehlers–Danlos syndrome with intramuscular hematoma secondary to trauma was made. Diagnosis of such an isolated presentation of a relatively rare disease is a challenge. Like most connective tissue disorders, Ehlers–Danlos syndrome has a wide cluster of clinical features. Hence, the condition has been classified into various subtypes, each possessing its own distinct feature with some overlaps. The most recent classification describes 13 types of Ehlers–Danlos syndrome.[1] Primary pathology involves the mutation in different types of collagen arising as a result of a genetic defect. Inheritance pattern varies with the type of Ehlers–Danlos syndrome, incidence being about 1 in 5000 births. Ehlers–Danlos syndrome can manifest in early life as severe muscular hypotonia at birth, congenital bilateral hip dislocation, fragile thin translucent skin with extensive bruising presenting as “cigarette-paper” scars, and absorptive periodontosis with premature loss of permanent teeth. Patients diagnosed with Ehlers–Danlos syndrome should be meticulously evaluated for conditions such as mitral valve prolapse, hernias, and kyphoscoliosis causing respiratory impairment. Other more catastrophic occurrences include spontaneous rupture of arteries, intestines, and eye globe even with minimal trauma. A comprehensive assessment of signs such as multiple scars and previous injuries can aid in early diagnosis and prevention of complications of connective tissue disorders.
Figure 1: Computed tomography scan image of the intermuscular hematoma along with classical clinical sign of the patient with Ehlers Danlos syndrome

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Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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There are no conflicts of interest.

   References Top

Malfait F, Francomano C, Byers P, Belmont J, Berglund B, Black J, et al. The 2017 international classification of the Ehlers-Danlos syndromes. Am J Med Genet C Semin Med Genet 2017;175:8-26.  Back to cited text no. 1


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