|Year : 2012 | Volume
| Issue : 1 | Page : 44-46
Acute pyelonephritis mimicking acute abdomen: An atypical presentation of unilateral dysplasia of the kidney in an adult
Nitin Joshi1, Shilpi Sahu2, Vishal Yadav3, Raviraj Jadhav3
1 Department of Urosurgery, MGM Medical College and Hospital, Kamothe, Navi Mumbai, Maharashtra, India
2 Department of Pathology, MGM Medical College and Hospital, Kamothe, Navi Mumbai, Maharashtra, India
3 Department of Surgery, MGM Medical College and Hospital, Kamothe, Navi Mumbai, Maharashtra, India
|Date of Web Publication||28-Aug-2012|
302, Dhavalgiri, Plot no. 11, Sector 11, South Kartik Marg, New Panvel (E), Raigad - 410 206, Maharashtra
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Multicystic dysplastic kidney is a nongenetic, congenital, cystic renal disease in which the renal cortex is replaced by numerous cysts of multiple sizes. The most common clinical presentation of unilateral renal dysplasia is abdominal lump in a new born infant who is otherwise healthy. Unilateral renal dysplasia is compatible with life. In adults, it is either diagnosed incidentally, can be an autopsy finding, or very rarely is symptomatic. Presented here is an unusual clinical presentation of unilateral renal dysplasia in a 30-year-old woman who presented with acute severe right lumbar and iliac fossa pain. Ultrasound and computerized tomography scans of the abdomen revealed absent right kidney with compensatory hypertrophy of the left kidney. As symptoms did not settle with standard conservative line of treatment, cystoscopy with retrograde pyelography was performed for evaluation of microscopic hematuria. A diagnosis of dysplastic kidney was made; exploratory laparotomy with right nephroureterectomy was performed. Patient had a smooth postoperative course. Histopathology confirmed renal dysplasia with pyelonephritis.
Keywords: Adult, multicystic dysplastic kidney, nephroureterectomy, retrograde pyelography, unilateral renal dysplasia
|How to cite this article:|
Joshi N, Sahu S, Yadav V, Jadhav R. Acute pyelonephritis mimicking acute abdomen: An atypical presentation of unilateral dysplasia of the kidney in an adult. Ann Nigerian Med 2012;6:44-6
|How to cite this URL:|
Joshi N, Sahu S, Yadav V, Jadhav R. Acute pyelonephritis mimicking acute abdomen: An atypical presentation of unilateral dysplasia of the kidney in an adult. Ann Nigerian Med [serial online] 2012 [cited 2020 Aug 15];6:44-6. Available from: http://www.anmjournal.com/text.asp?2012/6/1/44/100227
| Introduction|| |
Multicystic dysplastic kidney (MCDK) is a nongenetic, congenital, cystic renal disease in which the cortex is replaced by numerous cysts of multiple sizes.  The condition is usually associated with ureteral or uretropelvic atresia and the affected kidney is nonfunctional.  The ureteric bud theory proposed by Mackie and Stephens suggests that MCDK results from an abnormal induction of metanephric mesenchyme by the ureteral bud.  Exposure to teratogens and some genetic mutations have also been reported as the causes of MCDK. 
The final structure of the dysplastic kidney depends on the timing of injury to the ureteric bud and the effect of injury on the ureteric bud branching. Whether involution of the cysts is associated with involution of the intervening renal parenchyma is unclear. Extraordinary feature of MCDK is that these massively enlarged structures involute and disappear due to programmed cell death thus becoming undetectable prenatally or postnatally. , The residual parenchyma may be too small to be identified with conventional diagnostic imaging studies.
With the advent of routine antenatal ultrasound (US) check up, MCKD is diagnosed in utero as early as 15 weeks of gestation. In a new born infant,  unilateral renal dysplasia most commonly presents as a lump in the abdomen and is the most common cystic disorder of the kidney in childhood. ,
In adults, MCDK is usually asymptomatic and can remain undetected into adulthood. It may be detected during investigation for urinary tract infection, voiding dysfunction, hypertension, or during diagnostic imaging study performed for some nonurinary problem.
| Case Report|| |
A 30-year-old woman presented with acute onset of right lumbar and iliac fossa pain associated with vomiting and low-grade fever. There were no associated lower urinary symptoms. She had undergone appendicectomy 3 years prior to this presentation. On examination, she was acutely ill, pale, and febrile with normal blood pressure. There was tenderness and guarding in the right lumbar and iliac fossae. Laboratory investigations showed low hemoglobin with polymorphonuclear leucocytosis. Urinalysis, urine microscopy, and other routine investigations were within normal limits. Initial US scan and then computerized tomography (CT) scan of abdomen showed absent right kidney with compensatory hypertrophy of the left kidney [Figure 1].
|Figure 1: CT abdomen showed only absence of right kidney with compensatory hypertrophy of left kidney|
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Patient was treated with intravenous antibiotics, analgesics, and intravenous fluid infusion based on suspicion of urinary tract infection (UTI). However, pain, fever, tachycardia, and leucocytosis did not improve despite conservative treatment for more than 48 hours. Repeat urine examination showed microscopic hematuria. To investigate this, diagnostic cystoscopy was performed. On cystoscopy, right ureteric orifice was visualized and hence a retrograde pyelography was performed. This showed a right blind ending ureter terminating at the level of the fifth lumbar vertebra. Because patient was still symptomatic, she was subjected to exploratory laparotomy through modified Gibson's incision. Rudimentary renal tissue with ureteric atresia at pelviureteric junction and upper and mid ureter was encountered. Right nephroureterectomy was performed. A 5 × 3 × 2 cm grayish brown soft tubular mass attached to a long cord-like structure was removed [Figure 2]. Patient had a smooth postoperative course and she remained asymptomatic for over 1 year of follow-up.
|Figure 2: Received grayish brown soft tubular tissue mass measuring 5 × 3 × 2 cm|
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Histopathological examination showed primitive duct lined by undifferentiated epithelium surrounded by a fibromuscular collar [Figure 3]. In view of the lobar disorganization with atrophied renal structure and cystically dilated ducts [Figure 4], a diagnosis of renal dysplasia was made. The specimen showed histologic evidence of pyelonephritis. The long cord-like structure (atretic ureter) was composed of fibrocollagenous tissue and did not show any lumen.
|Figure 3: Primitive duct lined by undifferentiated epithelium surrounded by fibromuscular collar|
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|Figure 4: Lobar disorganization with atrophied renal structure and cystically dilated duct|
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| Discussion|| |
With the advent of routine antenatal US, MCDK is diagnosed in utero as early as 15 weeks of gestation. The condition is usually unilateral with an incidence of 1 in 4000. ,
The commonest clinical presentation of unilateral dysplasia is a lump in the abdomen in new born infant. Review of literature revealed only a handful of symptomatic cases in adults who were symptomatic due to hypertension and UTI. ,,
The common causes of acute right lumbar or iliac fossa pain in a 30-year-old woman are inflammation of appendix, a tubo-ovarian mass, ectopic pregnancy, or infection. The common causes in our case were ruled out as appendicectomy was performed 3 years back and CT scan of the abdomen showed normal tube and ovary on the right side. Evaluation for microscopic haematuria led us to the diagnosis of right MCDK. We presume that lower ureteritis may be probable cause of haematuria in our case. Infection of MCKD was proved on histopathology by microscopic evidence of pyelonephritis. Patient has remained asymptomatic for over a year following surgery.
The extraordinary feature of MCDK is that these massively enlarged structures involute and disappear due to programmed cell death and become undetectable prenatally or postnatally. , It usually is asymptomatic and remains undiagnosed in adulthood. As the incidence of development of complications like hypertension, malignancy, or UTI in dysplastic kidney is low, surgical intervention in dysplastic kidney is rarely required.  Surgery in dysplastic kidney is required only when enlarged kidney fails to regress, causes obstructive uropathy, develops malignancy, or the dysplastic kidney become a cause of hypertension or UTI. The need for nephrectomy for acute abdominal pain is unique.
Though inflammation of MCDK causing acute abdominal pain has not been described in the literature, it should be kept in mind as a differential diagnosis when one kidney is not visualized on US or CT scan.
| References|| |
|1.||Aremu AA, Osiatuma VA, Olajide A, Asaleye CM, Famurewa OC, Adetiloye VA. The atypical presentation of Multi- cystic Dysplastic Kidney in Nigerian. Adults: Two Case Reports. J Clin Diagn Res 2011;5:12. |
|2.||S-Urban A, Craig L. Multicystic renal Dysplasia. Available from: http://www.emedicine.medscape.com/article/982560-overview. [Last accessed 2011 Nov 9]. |
|3.||Mackie GG, Stephens FD. Duplex kidneys: A correlation of renal dysplasia with position of the ureteral orifice. J Urol 1975;114:274-80. |
|4.||Hains DS, Bates CM, Ingraham S. Management and etiology of unilateral multicystic dysplastic kidney: A review. Pediatr Nephrol 2009;24:233-41. |
|5.||John U, Rudnik-Schoneborn S, Zerres K. Kidney growth and renal function in unilateral multicystic dysplastic kidney disease. Pediatr Nephrol 1998;12:567-71. |
|6.||Winyard PJD, Nauta J, Lirenman DS. Deregulation of cell survival in cystic and dysplastic renal development. Kidney Int 1996;49:135-46. |
|7.||Michael Mc B. Unilateral renal dysplasia in adult. BJU Int 1971 93:830-4. |
|8.||Longino LA, Martin LW. Abdominal masses in newborn infant. Pediatrics 1958;21:596-604. |
|9.||WU CY, Chiou YH, Sung PK. Left multicystic dysplasia kidney with contralateral ectopic kidney and ureterovesicle junction obstruction. Zhonghua Yi Xue Za Zhi (Taipei) 2001;64:129-32. |
|10.||Kim EK, Song TB. A study on fetal urinary tract anomaly: Antenatal ultrasonographic diagnosis and postnatal follow-up. J Obstet Gynaecol Res 1996;22:569-73. |
|11.||Liebeschuetz S, Thomas R. Unilateral multicystic dysplastic kidney. Arch Dis Child 1997;77:369. |
|12.||Wackman J, Phipps L. Report - The multicystic kidney register: Prelimary finding. J Urol 1993;150:1870-2. |
|13.||Narchi H. Risk of hypertension with multicystic kidney disease: A systemic review. Arch Dis Child 2005;90:921-4. |
|14.||Burgler W, Hauri D. Vital complication in multicystic kidney degeneration (multicystic dysplastia). Urol Int 1983;38:251-6. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4]