SCIENTIFIC
May-Thurner Syndrome in a Ten-Year-Old
AUTHORS :
Janani Narumanchi , MD Department of Pediatrics , West Virginia University
TaraChandra Narumanchi , MD Department of Pediatrics , West Virginia University
Naveena Yanamala , PhD Division of Cardiovascular Disease and Hypertensions , Rutgers University
CASE PRESENTATION
A 10-year-old boy with a history of autism , asthma , constipation , gastroesophageal reflux disease , multiple food and medication allergies , recurrent otitis media status post myringotomy tube placements , pica , idiopathic syrinx , and global developmental delay presented with worsening left lower extremity swelling for the past year and new onset hematuria . While the swelling initially started below the knees , predominantly on the left ( Figure 1 ), as time progressed it frequently extended to his thighs and was often accompanied by a purplish discoloration . His mother noted that when his legs were swollen , he walked less and winced as if in pain if his legs were palpated . The swelling was more noticeable when the child was in a warmer environment and more active , otherwise the swelling occurred randomly . Elevating the legs did not result in improvement of symptoms . In time , the swelling progressed to involve the hands and face , which was not associated with any pain . His history was negative for associated trauma , fever , chills , upper respiratory symptoms , fatigue , vomiting , and / or diarrhea . He also complained of intermittent headaches without history of head injury . He began the habit of holding his hands on the sides or the front and back of his head almost daily for the same duration as the other symptoms . These headaches resolved with over-the-counter pain medications
While gross hematuria was never noted , a urinalysis was performed due to the
ABSTRACT
May-Thurner syndrome ( MTS ) is an unusual cause of intermittent lower extremity edema in pediatric patients . We present the case of a 10-year-old child who presented with gradual onset of intermittent headaches , hematuria , and intermittent worsening edema of his lower extremities . After extensive evaluation ,
his tory of leg swelling . Multiple urine samples collected over the period of six months revealed the presence of five to 15 red blood cells on high power field and no proteinuria . His physical examination , upon multiple occasions , revealed a nondysmorphic child in no distress and without leg edema . Although he was found to have mild non-pitting edema of the lower extremities on one occasion ( Figure 1 ), he was noted to have normal perfusion of his lower extremities throughout this period . Due to his varied symptoms , he was evaluated by specialists in various fields , including nephrology , neurology , genetics , and rheumatology . The differential diagnosis of unilateral lower leg swelling is broad as it can be due to trauma , orthostatic edema of adolescence , cellulitis , deep vein thromboses ( DVT ) myositis , compression due to a tumor , lymphadenopathy , nephrotic syndrome , liver diseases , and cardiac causes like congestive heart failure . Extensive laboratory studies were obtained to evaluate for the potential entities considered in the differential diagnosis . Serum electrolytes revealed a mild metabolic acidosis . Liver function tests , thyroid function tests , and a hemoglobin electrophoresis cascade were normal . Studies to rule out antineutrophil cytoplasmic antibody-associated vasculitis and Lyme disease were negative . Additional investigations , including genetic testing for a single gene disorder , chromosomal breakage syndrome , and methylation defects , along with whole exome sequencing , were unremarkable . A transthoracic echocardiogram and imaging studies of the brain , kidney , and bladder were also normal . the possible cause of the lower extremity edema was determined to be MTS . Since this diagnosis is usually considered in the differential diagnosis of teenagers and adults , our case raises aware ness of this possible diagnosis in younger pediatric patients suspected of having deep vein thromboses .
Results of the magnetic resonance angiogram ( MRA ) and magnetic resonance imaging ( MRI ) of the abdomen and pelvis revealed compression of the common iliac veins by the overlying right common iliac artery at the great vessel bifurcation , consistent with a variant of May-Thurner syndrome ( MTS ) ( Figure 2 ). The compression of the left common iliac vein was more pronounced than that of the right one ( Figure 2 ). Doppler ultrasound of the lower extremities ruled-out the presence of DVT bilaterally . Pediatric dermatology and hematology consultants concluded
FIGURE 1 : Bilateral leg edema , worse on the left
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