Leg Swelling and Abdominal Distension in an 11-day old

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ESPR288
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Abstract: :

History:

An 11-day old male presented to the pediatric emergency department (PED) due to concern for abdominal distension, vomiting and poor feeding. He was born at 38 weeks via c-section to a mother with gestational diabetes. He was discharged from the nursery at 48 hours of life. At home mom reported he was feeding well, urinating small amounts 5-6 times a day, and stooling appropriately. On the morning of arrival to the PED he was no longer feeding well and had one episode of non-bilious emesis.


Physical Examination:

On physical exam the patient was in significant distress. He was noted to have significant abdominal distension and tenderness, a sunken fontanelle, as well pitting edema to the lower extremities and poor tone. He was not in any respiratory distress. Initial vital signs showed mild tachycardia (157), but he was otherwise afebrile (36.6°C), respiratory rate of 46, and blood pressure of 85/55.


Laboratory testing and diagnostic imaging:

Point of care bedside ultrasound was able to identify severe bilateral hydronephrosis and a significantly distended bladder which was compressing flow in the inferior vena cava. Initial laboratory studies showed hypoglycemia (21), hyponatremia (113), hyperkalemia (8.7), hypochloremia (76), and renal failure (BUN 71 and Creatinine 6.8). Hypoglycemia was corrected with dextrose infusion.

A formal abdominal ultrasound was done after straight catheterization of the bladder resulting in 260cc of urine output. Formal abdominal ultrasound also noted marked bladder distension with severe bilateral hydrouteronephrosis, associated mild distal bilateral hydroureter and relatively static flow in the inferior vena cava, which was patent. 

Under the presumption of urinary tract obstruction a foley catheter was placed resulting in robust urine output. The patient's glucose was corrected, he was started on fluids and admitted to the neonatal intensive care unit where his electrolytes and urine output was monitored closely.


Final Diagnosis:

Initial presentation was concerning for abdominal infection and sepsis, as well as concern for congenital adrenal hyperplasia. The use of point of care ultrasound and formal imaging quickly placed urinary tract obstruction at the top of the differential diagnosis. Posterior urethral valves were confirmed by fluoroscopy urethrocystogram and cystoscopy on day 3 of admission and he had resection of the valves during the procedure. 


Discussion:

Posterior urethral valves (PUV) are a congenital malformation in males, occurring in 1 in 5,000 to 8,000 births, which results in urinary outflow obstruction from the proximal urethra. It is often found antenatally or while infants are still in the nursery due to poor urine output or urine stream. Outside of the nursery infants often present to the emergency department for urinary tract infections, failure to thrive or in urosepsis. In this case the patient presented with abdominal distension and lower extremity edema from enlarged bladder compressing the inferior vena cava. The electrolyte abnormalities (hyponatremia and hyperkalemia) in this case were attributed to severe hydronephrosis resulting in compression of the renal calices causing a secondary (transient) pseudohypoaldosteronism. Once the obstruction was relieved with the aid of a urinary catheter, abnormalities improved. This phenomenon has been previously reported in the literature associated with urinary tract malformations, but usually presents with urinary tract infection. Overall early detection of PUV is important as prolonged obstruction and hydronephrosis can lead to worse long-term prognosis and chronic kidney disease. Even after the posterior urethral valves are ablated, children can develop long term consequences including chronic kidney disease and bladder dysfunction.

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UCONN- Connecticut Children's
Connecticut Children's

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