Neonatal and Pediatric Medicine
Open Access

Abstract

Background: Splenogonadal fusion (SGF) is a rare congenital anomaly which occurs due to an abnormal fusion of splenic tissue with the derivatives of the perimesonephros. It usually presents as an inguinal hernia, testicular mass or undescended testis. To the best of our knowledge, this is the first time, SGF presented as a strangulated inguinal hernia.

Case: A six-year-old boy with an acute painful left inguinal swelling since that morning. No fever, abdominal distension or vomiting. General examination- tachycardia. Groin exam- A tense, tender, irreducible left inguinal swelling with normal bilateral descended testes. RS, CVS, CNS- Normal.

Decision making: - A soft tissue swelling as the content of the hernia with reduced vascularity. DDx- ? Torsion of supernumerary testis

Intra operative findings: Left indirect inguinal hernia, No omentum/ bowel, but a purplish mass connected distally to upper pole of the left testis and proximally entering the peritoneum through a fibrous cord, Fibrous cords???360� torsion ??? early gangrenous changes, Fibrous cords excised from upper pole of testis distally and as high as possible proximally. Herniotomy done.

Histopathology report: Histopathology revealed a fibroelastic capsule covering a tissue comprised of red pulp, white pulp, dilated sinusoids; consistent with an ectopic spleen. There was no testicular tissue noted in the excised specimen, clinching the diagnosis of SGF, the continuous type.

Discussion: SGF is a rare entity - less than 200 cases reported till date.The etiology-unknown, but few theories-(a). Post-inflammatory adhesions between gonadal ridge and spleen (b). An abnormal retroperitoneal pathway allowing the communication between the gonad and spleen 3 (c). The gonadal tunica enveloping the developing spleen. Treatment of choice-Complete excision of the splenic tissue with preservation of gonad. Unfortunately, 37% of the reported cases underwent unnecessary orchidectomy as many surgeons were unfamiliar with SGF.

Conclusion: Splenogonadal fusion, although rare, can mimic a strangulated inguinal hernia, uncomplicated inguinal hernia, undescended testis, torsion testis or supernumerary testis. Every surgeon should be aware of this condition to avoid the dreaded morbidity of orchidectomy in this beginning condition.

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