Loin Pain Haematuria Syndrome Complicating Nehroptosis: Case Report

Abbreviations: IVU: Intravenous Urography; CAT: Computer Axial Tomography; MRI: Magnetic Resonance Imaging; DSA: Digital Subtraction Angiography; IR: Isotope Renography; RSD&N: Renal Sympathetic Denervation and Nephropexy; RGP: Retrograde Pyelography; DSA: Digital Subtraction Arteriography; LPHS: Loin Pain Haematuria Syndrome; SN: Symptomatic Nephroptosis; TIA: Transient Ischaemic Attacks ARTICLE INFO abstract


Conclusion:
The only way to diagnose SN is by doing IVU with erect film. All other ancillary imaging's were normal being feasible only on supine posture. The case demonstrates the progression of SN to the complication of LPHS which occurred after 3.5 years. The surgery of RSD&N has proved successful in many other cases, but this patient refused it.

Case Report
A 25 years old Saudi single female school teacher presented 9 years ago to the haematologist with iron deficiency anaemia (Haemoglobin was 8.3 gr/l. During her stay in hospital, she suffered from bilateral epistaxis. Mucoperiostal elevation cured her epistaxis after 7 bilateral cauterizations had failed to control her nasal bleeding. No bleeding or coagulation abnormalities were detected. She was also reported to have dysmenorrhea, dysurea and right loin pain. Attending physicians excluded genitourinary and other system abnormalities, Loin pain and microscopic haematuria reoccurred. Urological investigations including Urine analysis and culture, full blood count, renal function tests, grayscale ultrasound and intravenous urography (IVU) were repeatedly normal. No cause for her painful haematuria was found for 5 years but it was confirmed to originate from her right kidney on cystoscopy when bleeding was seen spyrting from the right ureteric orifice. Retrograde pyelography (RGP) was initially normal. She had repeated investigations done at various hospitals for her loin pain and haematuria.
These included computer axial tomography (CAT) scan, magnetic resonance imaging (MRI), digital subtraction arteriography (DSA) and isotope renography which were all normal in supine posture.
Psychological disorders, opiate dependency and/or imaginary pain were thought on repeated admissions to be the cause of her undiagnosed and treated suffering. Psychiatric assessment excluded personality disorders and she was denied opiate therapy for years.
Five years ago, right nephroptosis was suspected after palpating a mass at the right iliac foss on erect examination which disappeared on lying supine-unless the kidney mass was held down by gripping

Discussion
The natural history of this case was followed up from the initial onset of loin pain to occurrence of recurrent microscopic haematuria, to diagnosis of symptomatic nephroptosis (SN), to the development of LPHS [1]. To my knowledge this is the first case report of LPHS to complicate SN. It is certainly not the last [2]. It demonstrates many of the problems encountered in the diagnosis and therapy of both SN and LPHS. All textbooks do not index SN as it was disparaged long ago [3] but mention LPHS. Chance diagnosis of SN on supine imaging is unlikely. So, SN has become a universally forgotten diagnosis [3]. Vascular anomalies, ischaemic renal scarring and messengial proliferative glomerulonephritid are features of LPHS [1]. Most of these renovascular complications of LPHS are also documented in SN [4][5][6][7]. Despite known overinvestigations, no erect imaging was ever done in LPHS. Previous reports on erect artieriography [5][6][7] and isotope renography [7] demonstrated these complications in SN. Recurrent stretch of renal vessels in erect posture causes renal artery elongation and narrowing in the presence or absence of stenostic lesions [5].
Her painful episodes of haeematuria are also known "Dietl crisis" of SN, who also advised knee-chest position for temporary relief. This tragic case represents the tip of an iceberg. Experience at King Khaled Hospital in Najran Saudi Arabia with 190 cases suffering from SN, of whom LPHS complicated SN in 36 (18.9%) of patients [8,9]. This figure represents cases with gross haematuria only. It is higher when cases with microscopic haematuria are included. Making an early diagnosis of SN by erect IVU may upright issues and brings relief to the unfortunate sufferers. Based on recent reports, SN has proved to be a preventable cause of LPHS when the correct surgical procedure is done timely. The surgery of renal sympathetic denervation and nephropexy has proved curable for Both SN and LPHS. Both nephropexy for SN and renal sympathetic denervation of the LPHS were reported separately [10,11].
Modern favourable results of renal sympathetic denervation and nephropexy (RSD&N) have proved 100% success rate for both SN and LPHS [8,9]. Nephroptosis was disparaged and nephropexy was abandoned >70 years ago, both have been deleted from surgical and urological textbooks and have become universally forgotten. This is because of the many problems in diagnosis and therapy that have been recently addressed and resolved [10,11].