Congenital megaloureter

Congenital Megaloureter

Congenital megaloureter is a condition characterized by an abnormal dilation of the ureter, which is the tube that carries urine from the kidney to the bladder. This condition is present at birth and can affect one or both ureters. It is important to understand the underlying causes, symptoms, diagnosis, and treatment options for congenital megaloureter to provide effective care and management.

Etiology

Congenital megaloureter can be classified into three main types based on the underlying cause:

• Obstructive Megaloureter: This occurs when there is a functional or anatomical obstruction at the distal ureter, often at the ureterovesical junction, leading to dilation of the ureter.
• Refluxing Megaloureter: This type is associated with vesicoureteral reflux, where urine flows backward from the bladder into the ureter, causing dilation.
• Non-obstructive, Non-refluxing Megaloureter: In this type, the ureter is dilated without any apparent obstruction or reflux, and the cause is often idiopathic.

Pathophysiology

The pathophysiology of congenital megaloureter involves the abnormal development of the ureteral wall or the presence of functional obstructions. In obstructive megaloureter, the obstruction leads to increased pressure in the ureter, causing it to dilate. In refluxing megaloureter, the backward flow of urine increases the volume and pressure within the ureter, leading to dilation.

Clinical Presentation

Patients with congenital megaloureter may present with a variety of symptoms, including:

• Urinary Tract Infections (UTIs): Frequent UTIs can be a common presentation, especially in children.
• Abdominal Pain: Some patients may experience pain due to the distension of the ureter.
• Hematuria: Blood in the urine can occur in some cases.
• Palpable Abdominal Mass: In severe cases, a mass may be felt in the abdomen due to the enlarged ureter.

Diagnosis

The diagnosis of congenital megaloureter is typically made using imaging studies, such as:

• Ultrasound: This is often the first imaging modality used to detect dilation of the ureter.
• Voiding Cystourethrogram (VCUG): This test can help identify vesicoureteral reflux.
• Magnetic Resonance Urography (MRU): Provides detailed images of the urinary tract and can help assess the degree of dilation and any associated abnormalities.

Treatment

The treatment of congenital megaloureter depends on the severity and underlying cause:

• Observation: Mild cases may be monitored with regular follow-up and imaging.
• Antibiotic Prophylaxis: Used to prevent UTIs in cases with vesicoureteral reflux.
• Surgical Intervention: Indicated in severe cases or when there is significant obstruction or recurrent infections. Surgical options include ureteral reimplantation or ureteral tapering.

Prognosis

The prognosis for congenital megaloureter varies depending on the severity and treatment. Many children with mild forms of the condition may improve with time, while others may require surgical intervention to prevent complications such as kidney damage.

Also see

• Vesicoureteral Reflux
• Hydronephrosis
• Ureteral Obstruction
• Pediatric Urology