NEPHROLOGY 2005; 10, 189–191 doi:10.1111/j.1440-1797.2005.00377.x © 2005 Asian Pacific Society of Nephrology. Blackwell Science, LtdOxford, UKNEPNephrology1320-53582005 Asian Pacific Society of NephrologyApril 2005102189191Case Report Renal hypoplasiaK Kaynar et al. Correspondence: Dr Kubra Kaynar, Karadeniz Teknik Üniversitesi, Tıp Fakültesi, Nefroloji Bilim Dalı, 61080 Trabzon, Turkey. Email: kkaynar@yahoo.com Accepted for publication 26 November 2004. Case Report Renal hypoplasia and situs inversus totalis KUBRA KAYNAR, 1 SUKRU ULUSOY, 1 SEMIH GUL, 1 GULSUM OZKAN, 2 REFIK CAYLAN 3 and POLAT KOSUCU 4 Departments of 1 Nephrology, 2 Internal Medicine, 3 Otorhinolaryngology and 4 Radiology, School of Medicine, Karadeniz Technical University, Trabzon, Turkey SUMMARY: A spectrum of renal abnormalities of patients with situs inversus has been reported. Renal dysplasia is the most common. Herein is described for the first time, an association of situs inversus totalis, unilateral congenital renal hypoplasia and external ear cartilage deformity. KEY WORDS: ear deformity, renal hypoplasia, situs inversus. INTRODUCTION A spectrum of renal abnormalities of patients with situs inversus totalis has been reported. Renal cell carcinoma and renal dysplasia have been reported to be associated with situs inversus totalis. 1,2 Three cases of renal cell carcinoma have been described in the literature. 1,3,4 Five cases of bilateral renal dysplasia with situs inversus totalis and hepatic and pancreatic fibrosis have also been noted. 2,5–8 Pinar and Rogers called these spectrum of anomalies a new syndrome. 6 Association of situs inversus totalis and renal hypoplasia has not been reported. In the present case we report the association of renal hypoplasia and situs inversus totalis for the first time. CASE REPORT A 32-year-old female patient presented to the nephrol- ogy department of Karadeniz Technical University with hypertension. The patient had a history of abortion due to pre-eclampsia and giving birth of an ex male fetus with multiple abnormalities (cleft palate, cleft lip, low set ears, spinabifida) 5 years previously. At physical examination, blood pressure was 140/100 mmHg and there was bilat- eral congenital cartilage deformity in her external ears (Fig. 1). Ear nose throat examination revealed normal hearing levels. There was no family history of situs inver- sus totalis and consanguinity. Laboratory evaluation revealed creatinine, 114 mmol/L, blood urea nitrogen (BUN), 8.2 mmol/L, albumin, 39 g/L, haemoglobin, 12.9 g/dL. Creatinine clearance was 75 mL/min, and pro- tein excretion in urine was 420 mg/day. Chest X-ray revealed dextrocardia. Abdomen ultrasonography and computed tomography revealed left renal hypoplasia (Fig. 2a). The liver was found to be on the left side, and the spleen was found to be on the right side (Fig. 2b). Right kidney was 105 mm in length with paranchymal thickness of 20 mm, pelvis and parenchymal appearance were both normal. The left kidney was miniature in size, at 55 mm in length and 10 mm in paranchymal thick- ness. The patient had no history of urinary tract infec- tion and no sign of vesico-ureteral reflux. The patient had grade II hypertensive retinopathy. Losartan was administered at 50 mg/day and after lifestyle modifica- tion her blood pressure came down to normal (120/ 80 mmHg). DISCUSSION We describe an apparently new entity characterized by situs inversus totalis, renal hypoplasia and external ear cartilage deformity. This entity is quite different from the syndrome reported by Pinar and Rogers. They defined situs inversus totalis, bilateral renal dysplasia and multi- system fibrosis. Huang and Chen encountered another case with situs inversus totalis and bilateral renal dyspla- sia. 2 In that case, hepatic and pancreatic fibrosis was not as significant as the syndrome defined by Pinar and Rogers. 6 Yokoyama et al. suggested a gene mapped to mouse chromosome 4 in a region homologous either to human 6q or 9q resulting in situs inversus totalis. 9 Het- erotaxy results from failure to establish normal left–right asymmetry during embryonic development. Most famil-