Non-surgical approach works to stop major bleed in child with kEDS
11-year-old boy's conservative therapy offers guidance for future care
In the first case of its kind, doctors report that a child with kyphoscoliotic Ehlers-Danlos syndrome (kEDS) recovered from a major bleeding emergency without needing surgery. The case suggests a non-surgical approach may be feasible for patients with the rare condition.
Doctors managed the boy, 11, conservatively with blood transfusions and supportive treatments.
“This case report underscores the importance of a multidisciplinary approach, tailored management strategies, and ongoing research to optimize outcomes and minimize complications in pediatric patients with the kyphoscoliotic variant of Ehlers-Danlos syndrome presenting with vascular hemorrhage,” researchers wrote.
The study, “Pediatric Ehlers-Danlos syndrome VI with retroperitoneal Hemorrhage: case report and management strategy,” was published in Oxford Medical Case Reports.Â
Sudden-onset excruciating pain
EDS refers to a group of genetic disorders that cause abnormalities in the body’s connective tissue. Some types of EDS, particularly vascular EDS, are marked by blood vessel fragility, which can lead to blood vessels breaking and causing serious bleeding.
kEDS is a form of EDS typified by kyphoscoliosis, a type of abnormal curvature in the spine. Severe bleeding due to blood vessel breaks has been documented in adult patients, but there has not been a report of this type of complication affecting a child with the syndrome, according to the researchers.
“While vascular rupture is documented within the spectrum of the kyphoscoliotic variant in the literature, no such cases have been ever reported to date with presentation in pediatric age group, to the best of our knowledge,” the researchers wrote.
This report details the case of a boy who sought emergency medical treatment after experiencing sudden-onset, excruciating pain in his abdomen and lower back. His discomfort worsened in the emergency department, and lab work showed low hemoglobin (a protein in red blood cells responsible for oxygen delivery to tissues) and low levels of immune cells called neutrophils. This suggested a “possible underlying inflammatory process or acute insult,” the scientists wrote.
A CT scan revealed the boy was experiencing a hemorrhage in the retroperitoneal space, or the region in the abdominal cavity located behind the peritoneum, the membrane lining the abdominal wall.
Once the hemorrhage was identified, a multidisciplinary team of clinicians convened to discuss how best to proceed, debating whether it would be worth the risks of performing surgery. The boy’s condition was worsening, and clinicians found an aneurysm (a bulge in a blood vessel wall). Thus, doctors opted for a conservative approach, giving blood transfusions to maintain blood cell levels as well as medications to manage blood pressure and pain.
The patient was closely monitored. His pain eased gradually, his hemoglobin levels were stable, and he was discharged home on the fifth day. He “remained well on follow up,” the scientists wrote.
They noted that this boy’s experience was markedly different from what has been reported in children with vascular EDS, where major bleeding typically requires surgery to manage effectively.
“Our patient with kyphoscoliotic variant of EDS had a favorable outcome with conservative management, highlighting a distinct clinical course,” the scientists wrote.
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