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Splenogonadal fusion: Report of a rare variety
Abstract
We report an unusual case of splenogonal fusion in a 10-year-old boy with an undescended left testis. He suffered from congenital limb defects, a known association with splenogonadal fusion, and had originally been admitted for orchidopexy.
Splenogonadal fusion is a rare entity with approximately 150 reported cases until the year 2005. The entity is a rare congenital anomaly in which there is fusion of splenic and gonadal anlagen or mesonephric derivatives. Splenogonadal fusion has two continuous and discontinuous types. About ten cases have been reported in association with bilateral cryptorchidism. Very few cases have been diagnosed preoperatively. Many cases present as a testicular swelling and undergo an unnecessary orchiectomy with the suspicion of a testicular neoplasm. Herein, we report a new case of discontinuous splenogonadal fusion in a 29 year old man with bilateral cryptorchidism who underwent surgery. Surprisingly two masses were noted adjacent to undescendent testis in left groin in operative room which were resected and proved to be a new case of splenogonadal fusion in histological exam.
Abstract Ectopic and heterotopic tissues within the testis and paratesticular structures are frequent. Here we describe abnormalities of testicular blastema, and different forms of ectopic location of testicular elements, as well as aberrant anatomic relations between the testis and other organs or tissues.
Nebenmilzen finden sich autoptisch in 10–15% der Bevlkerung, in 1–2% liegen sie im Pankreasschwanz. Folglich mssen sie in die Differenzialdiagnose intra- und peripankreaner tumorser Lsionen einbezogen werden. Nach splenorenaler Fusion knnen sie pararenal-retroperitoneal zu finden sein, nach splenogonadaler Fusion mit den Gonaden ins Becken oder Skrotum deszendieren. Da sie fast immer asymptomatisch sind, werden sie meist als Zufallsbefund entdeckt. Die Diagnose wird per Szintigramm mit Technetium-99-markierten, hitzedenaturierten autologen Erythrozyten gesichert. Sollte ein Resektionseingriff erforderlich sein, so ist das der enkapsulierten Nebenmilz adhrente Organ zu schonen. Anhand dreier Fallbeschreibungen (intrapankreane Nebenmilz, retroperitoneale Nebenmilz, torsionsinfarzierte Wandermilz) wird die chirurgische Relevanz der Ektopien des Milzgewebes errtert.According to autoptic studies, accessory spleens may be found in 10% to 15% of the population, in 1% to 2% they are located in the pancreatic tail. They thus have to be taken into account in the differential diagnosis of intra- and peripancreatic tumorous lesions. After splenorenal fusion, they can be found pararenally and retroperitoneally, and after splenogonadal fusion they can descend into the pelvis or scrotum. Since they usually are asymptomatic, they are mostly discovered accidentally. The diagnosis is ascertained by a scintigram with technetium-99-marked, heat-damaged red blood cells. If resection is necessary, the organ adherent to the encapsulated accessory spleen should be preserved. By means of three case reports (intrapancreatic accessory spleen, retroperitoneal accessory spleen and torsion-infarcted wandering spleen), the surgical relevance of ectopic splenic tissue is discussed.
Splenogonadal fusion is a rare abnormality. We report the B-mode and color Doppler sonographic appearances of a superior testicular pole splenogonadal fusion and describe the pattern of vascularity present in this rare benign abnormality.
Two types of splenogonadal fusion (SGF) have been proposed. In the continuous type the spleen is connected to the gonad, and often it is associated with limb defects and other anomalies such as micrognathia, microglossia, anal atresia, and hypoplastic lungs. Associated abnormalities are lower in the discontinuous type of SGF, where there is fusion between the gonad and accessory splenic tissue, without connection with the normal spleen. The cause of SGF and SGF with limb defects (SGFLD) is unknown. On the basis of associated orofacial limb abnormalities, some authors have suggested that SGFLD may be related to the Hanhart complex. Others have noted the overlap between SGFLD and femoral-facial syndrome (FFS). We consider SGFLD a developmental field defect that originates during blastogenesis. Although the cause is unknown, the earlier its action, the more severe the involvement resulting in SGFLD; later action may result only in SGF.
Splenogonadal fusion is a rare congenital malformation in which there is an abnormal fusion between the spleen and the gonad or derivatives of the mesonephros. It can occur in both sexes but has been reported far more frequently in males. There are two types of this malformation: the continuous and the discontinuous type, depending on the presence or absence of a structural connection between the regular spleen and the ectopic splenic tissue that is fused to the gonad. In one-third of all reported cases splenogonadal fusion is associated with other congenital defects. This association is predominantly found within the continuous type. Peromelia is present in a high percentage of cases, varying from total absence of all limbs to absence of parts of the lower limbs.
On the basis of a case report and a review of 84 published cases, the causal, pathogenetic and nosologic aspects of this malformation are discussed. We hypothesise that splenogonadal fusion with peromelia and splenogonadal fusion without peromelia represent two ends of one spectrum determined by the developmental stage during which the causal factor acts.
hrs reported in the literature by Bostroem in~ i883 but it was Pommer, who in 1889 described in detail a ease which was associated with limb defects and other congenital abnormalities, no- tably mierognathia and anal atresia. It has been suggested that this teratologie syndrome of splenogonadal fusion, limb defects, and other Congenital abnormalities, which constitutes a subgroup of patients with splenogonadal fu- sion, be called the Pommer syndrome. Putschar and Manion in 1956 published the first detailed literature review of splenogonadal fusion in which details of 30 clinical eases and a classifi- cation system were discussed. Since their review was published the urologic and pediatric surgi- cal literature have contributed almost another 100 cases which have been mainly in the form of single ease reports with exceptionally, 2 and 3 cases described. Nevertheless, many of the re- ports conclude with the performance of an or- chieetomy, in the mistaken belief that this entity represents a malignant process. Thus it is vital that urologists, pediatric and general surgeons are aware of this condition and appreciate its benign nature.
• A case of continuous variety of splenotesticular fusion in a child having undescended left testis associated with a rare variant of hydrocele and in whom an ultrasound imaging has shown a correct preoperative diagnosis consistent with this abnormality is reported.
A male infant was born with symmetrical tetramelic limb deficiency consisting of bilateral upper limb amelia with severe symmetrical proximal focal femoral deficiency and fibula deficiency associated with left splenogonodal fusion of the discontinuous type, micrognathia, and a prominent capillary haemangioma of the face. The parents are first cousin Lebanese muslims. This observation suggests the possibility of recessive inheritance in some cases of the Amelia, Femur-Fibula dysostosis syndrome with or without splenogonadal fusion.