RESUMEN
Splenogonadal fusion (SGF) is a rare congenital anomaly of an aberrant accessory spleen-gonad connection. We present a rare case of continuous splenogonadal fusion in a full-term male with a left undescended testis, multiple congenital limb anomalies, and syndromic facies. Diagnostic laparoscopy revealed the "Echidna Splenule," a snake-like intraperitoneal splenule coursing from the spleen along the left paracolic region and engulfing an atrophic intra-abdominal testis preventing spontaneous descent and distally herniating into the left open internal inguinal ring. The atrophic testis and Echidna Splenule were resected. Splenogonadal fusion should be considered in children with left undescended testis and concomitant limb and facial anomalies.
Asunto(s)
Anomalías Múltiples , Criptorquidismo , Anomalías del Sistema Digestivo , Tachyglossidae , Niño , Animales , Humanos , Masculino , Criptorquidismo/diagnóstico , Criptorquidismo/cirugía , Criptorquidismo/complicaciones , Testículo/diagnóstico por imagen , Testículo/cirugía , Testículo/anomalías , Bazo/anomalías , Escroto , Anomalías Múltiples/diagnósticoRESUMEN
Congenital bladder underdevelopment has detrimental genitourinary effects. We present a male infant with declining renal function, recurrent urinary tract infections, and epididymo-orchitis, in the setting of bilateral high grade hydronephrosis and vesicoureteral reflux. Cystoscopic evaluation revealed the presumed "bladder" to be a massively dilated prostatic urethra. The small dysplastic "true bladder" was found at the anterior surface of the prostatic urethra with massively dilated ureteral orifices. Temporizing diversion was performed in the form of bilateral cutaneous ureterostomies, with an ultimate plan for reconstructive bladder surgery. This case presents diagnostic challenges utilizing current modalities due to a massively dilated prostatic urethra in the rare case of bladder dysplasia.