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Paediatric Nephrectomy: Patterns, indications and outcome in a developing country

Uchechukwu Obiora Ezomike1, Victor Ifeanyichukwu Modekwe 2, Sebastian Okwuchukwu Ekenze1

  1. Sub-Department of Paediatric Surgery, Faculty of Medical Sciences, College of Medicine, University of Nigeria, Ituku/Ozalla Campus, Enugu, Nigeria
  2. Paediatric Surgery Unit, Department of Surgery, Nnamdi Azikiwe University Teaching Hospital, Nnewi, Nigeria.

Corresponding Author: Uchechukwu Obiora Ezomike : uchechukwu.ezomike@unn.edu.ng, ezomikeuche@yahoo.com

Published: June 2018 • https://dx.doi.org/10.4314/mmj.v30i2.8

ISSN: 1995-7262


Abstract

Background

Patterns of and indications for nephrectomy vary in different age groups, geographical locations and time periods. In some series, nephrectomies were mainly for malignant conditions while in others, they were predominantly for non-malignant conditions. Such data on patterns, indications, and outcomes of nephrectomy in children is limited in our environment.

Objectives

To evaluate nephrectomy in childhood at the Sub-Department of Paediatric Surgery University of Nigeria Teaching Hospital, Ituku/Ozalla Enugu with a focus on pattern, indications, and outcome.

Patients and methods

Medical records of all patients aged ≤16years who had nephrectomy from January 2007 to December 2016 were studied with emphasis on age, sex, side of nephrectomy, duration of symptoms before presentation, indication for nephrectomy, in-hospital complications, length of hospital stay, and in-hospital mortality. SPSS version 15 was used for data entry and analysis.

Results

There were 52 nephrectomies; 32 males and 20 females. They were for 35 malignant and 17 non-malignant conditions. Most of the malignancies were Wilms tumor (34/35) while non-malignant conditions were late-presenting pelvi-ureteric junction (PUJ) obstruction (9), large multi-cystic dysplastic kidneys (4), renal trauma with pedicle avulsion (1), posterior urethral valve (PUV) with atrophic kidney (1) and duplex system with non-functioning upper pole moiety (2). Mean age at nephrectomy was 5.10±3.66 years (range 7 weeks to 16 years); 59% of the nephrectomies were on the left and 41% on the right. Mean duration of hospital stay was 31.78±16.59 days (range 7-66 days). In-hospital mortality rate was 5.8%.

Conclusions

In our unit, nephroblastoma is the main indication for paediatric nephrectomy and is the only indication in females. Neglected pelvi-ureteric junction obstruction is the major non-malignant indication and occurred only in males. Most nephrectomies are done in the age range of 1-5 years. Nephron-sparing nephrectomy, major morbidity, re-operations are uncommon and in-hospital mortality from nephrectomy is still high at 5.8%.

Key words:  Paediatric; Nephrectomy; Pattern; Indications; Outcome


© 2018 The College of Medicine and the Medical Association of Malawi. This work is licensed under the Creative Commons Attribution 4.0 International License. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/)


Introduction

Surgical removal of the kidney in children is a major undertaking. The procedure may be for non-malignant conditions of the kidney and upper urinary tract causing poorly functioning nephroureteral units1. These non-malignant conditions may include chronic destructive infections, chronic obstructions with obstructive uropathy or nephropathy and severe trauma. Nephrectomy in children may also be for malignant conditions of the kidney and adrenal glands. The major indications for nephrectomy vary in different parts of the world and in different age groups and sexes2 with some recording more benign conditions3 and others more of malignancies4, 5.

Traditionally, nephrectomy is undertaken by open surgery and this is still the practice in our unit as well as other centers in Low and Middle Income Countries (LMICs)6,7. More recently, however, minimally invasive laparoscopic surgical techniques have been increasingly applied for nephrectomy in High Income Countries (HICs) 8, 9, 10, 11.  There has also been  a growing interest in the use of nephron-sparing surgery for selected patients. However, late presentation in our environment, especially for malignant diseases of the kidney, is still a daunting challenge, making such renal conserving surgeries uncommon.

Materials and Methods

This was a retrospective review of all children aged ≤ 16years who had nephrectomy from January 2007 to December 2016. The medical records department was written to, requesting for permission to have access to patients’ data from the theatre records and folders. The medical records were reviewed and data retrieved with emphasis on age, sex, side of nephrectomy, duration of symptoms before presentation, indication for nephrectomy, post-operative complications during admission, length of hospital stay, and mortality during admission. The indications were broadly classified into malignant and non-malignant conditions. Data entry and analysis were done with Statistical Package for Social Sciences (SPSS version 15.0 Chicago Illinois, USA).The results are expressed as means ± standard deviation, ratios, percentages, charts and tables.

Results

There were 52 nephrectomies (35 for malignant conditions and 17 for non-malignant conditions), carried out in 32 males and 20 females with a male to female ratio of 1.6:1. Mean age at nephrectomy was 5.1 ± 3.66 years. Mean duration of symptoms before presentation was 7.6 months (4 hours – 42 months). There were no bilateral cases in malignancy. Of the malignancies, 34 were Wilms tumor while 1 was renal rhabdomyosarcoma. The non-malignant conditions were neglected pelvi-ureteric junction obstruction with loss of renal function on intravenous urography (9), large multicystic dysplastic kidneys with non-function on intravenous urography (4), renal trauma with pedicle avulsion (1), posterior urethral valve (PUV) with no function of one kidney on renal scintigraphy (1), and duplex system with non-functioning upper pole moiety (2). In the first five year period, 27 nephrectomies were done while 25 nephrectomies were done in the second five year period. Of the nephrectomies, 59% were on the left and 41% on the right. Mean duration of hospital stay was 31.78 ±16.32 days (range 7-66 days).

Male: Female ratio for malignancy was 0.75:1. Malignancy was indication for all nephrectomies in females (20/35). Of all nephrectomies for malignancies and nephrectomies, 60% (21/35) and 54% (28/52) were in children aged 1-5 years respectively. Mean follow-up duration was 25.82 ± 34.12 weeks (range 0-156 weeks). There was only one nephron-sparing surgery in a patient with duplex renal system and non-functioning upper moiety (1.9%). All others had total nephrectomy. Complications of treatment included stitch reaction (2), small bowel volvulus and bowel gangrene (1), and hypertrophic scar (1). Three (3) mortalities in 2 males and 1 female were noted while on initial hospital admission (mortality rate of 5.8%): two with Wilms tumor died intra-operatively on the operating table and one neglected PUJ obstruction died of an undetermined cause in the early postoperative period.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Discussion

In this series, 67% of the nephrectomies were for malignant renal conditions and 33% for benign conditions. This was similar to findings by Bouhafs et al7 in Morocco where 62.5% of the nephrectomies were for nephroblastoma. This finding, however, is contrary to many other studies on pediatric nephrectomy from different parts of the world, where there were more benign than malignant indications recorded. From Sammon et al8 in USA, 73.8% of nephrectomies were for benign indications; Daradka3  in Jordan, 59%; Hammad et al12  in New Zealand, 76%; Adamson et al13  in England, 70.5%; Chabchoub  et al6  in Tunisia, 78.7%; Featherstone et al14  in London, 67.5%; and Nggada et al15  in Nigeria, 60.3%. However, in some studies on indications for nephrectomy in the adult population in both developed16,17  and Low and Medium Income Countries (LMICs)4,5, more malignances were noted as indication for nephrectomy in adults. This may be due to the higher incidence of malignancy in older patients and the higher proportion of children who present with congenital malformations12. One may not readily explain why malignancy was the major indication for nephrectomy in our paediatric population just like in adults in the same environment. However, this may be related to absence, in this study, of key non-malignant indications like complications of vesicoureteric reflux (VUR) and renal stone disease which were major indications in some other studies3, 13.

In this study the malignant cases were mainly Wilms tumor (96.5%). Wilms tumor was also the commonest malignant indication for nephrectomy in some other studies3, 15, 18. Neuroblastoma featured prominently as an indication for nephrectomy in another study3, but in our study, there were no neuroblatomas as indications for nephrectomy. This may be due to late presentation of neuroblastoma cases in our environment when they are no longer resectable. There was one renal rhabdomyosarcoma in this study and this agrees with the rarity of such renal tumor which exists in literature as mainly case reports19, 20.

In this study, non-malignant cases were mainly late-presenting PUJ obstruction with loss of function on intravenous urography and is also the most common non-malignant indication in some other series2, 21. However, in some studies, complications of vesicoureteric reflux (VUR) were the most common benign indications1,3,13. Other non-malignant indications were enlarging multicystic dysplastic kidney (MCDK) disease, Posterior Urethral Valve (PUV) with unilateral non-functioning kidney, duplex renal system with non-functioning upper moiety and renal trauma with pedicle avulsion.

It is important to note that complicated VUR, which is generally more common in females 3, was not an indication for nephrectomy in this study. This may be related to the lower incidence of VUR, hence its associated complications in blacks when compared to Caucasians22. Since VUR is the most common benign condition in some series and also more common in females3, this finding may explain why benign indications were less likely in females in this study.  In the current study, however, non-malignant indications were seen only in males and these were mainly complicated PUJ obstruction. It must be noted that PUJ obstruction is generally seen more in males than females3, 23.

The second most common non-malignant indication in this study is enlarging multicystic dysplastic kidneys (MCDK) with non-function on IVU. MCDK was the most common indication for nephrectomy in some studies which also recorded reducing incidence of PUJ obstruction as an indication for nephrectomy12, 14. In another study15, chronic pyelonephritis was the most common non-malignant indication, but this was not an indication in the current study.

Although PUV is the most common cause of bladder outlet obstruction in children, only 1 child (1.9%) had nephrectomy due to PUV-associated complications. Similarly, another study recorded that 2.5% of nephrectomies were for PUV-associated complications3. This may be explained by the fact that most late-presenting cases of PUV may have developed chronic kidney disease and end stage renal disease where various renal substitution strategies, and not nephrectomy, are employed in their treatment.

In other studies, stone disease was the major non-malignant indications for nephrectomy6, 11, 24. It is noteworthy that no nephrectomy due to renal stone disease or renal tuberculosis was observed in this study.

Early investigation of children suspected to have urological malformations, or who present with urinary tract infection, will encourage early diagnosis and reduce incidence of nephrectomies for non-malignant conditions25. In this way, most cases of PUJ obstruction will present earlier for monitoring and possible pyeloplasty instead of presenting very late for nephrectomy. In a report by Ocheke et al in Cape Town, Republic of South Africa, no nephrectomy was done for PUJ obstruction despite the substantial number of prenatal diagnoses of PUJ obstruction23.

Most studies had non-malignant indications for nephrectomy more in females and malignancy more in males3,11,24. In this series, however, all nephrectomies in females were for malignancy and 59% of all malignancies were in females. All non-malignant conditions were in males.

In the current study, we had only one emergency nephrectomy following renal trauma with pedicle avulsion in a 15-year old male who had low-velocity penetrating abdominal trauma. This corroborates with findings of other studies where abdominal trauma was an uncommon indication for nephrectomy3,4,5,12 .

Though our unit is in a teaching hospital, mean age at nephrectomy of 5.1± 3.7 years is higher than 3 years Sammon et al recorded for Teaching Hospitals in the USA8. This may be attributed to late presentation generally seen in LMIC’s. However, this mean age is comparable to 6 years for Non-Teaching Hospitals in The USA8, 4.75 years in New Zealand as reported by Hammad et al12 and 5 years by Bouhafs et al in Morocco 7.

In the current study, peak age range for nephrectomy is 1-5 years and least in 11-16 years (table 1). In another study by Sammon et al in USA, peak age range at nephrectomy was 0-1 years and least in 6-9years8. In Ekenze et al, nephrectomy peaked at 2-5years age range26. Mean age at nephrectomy for malignancy was 3.4 years in this study while in Daradka et al it was 3.6years3. Of all nephrectomies and all nephrectomies for malignancy, 54% and 60% occurred in the age range of 1-5 years respectively. This is opposed to another study where most nephrectomies (36%)  were in the 0-1 year age range and the indications were mainly benign8.

Overall male: female ratio for all nephrectomies was 1.6:1 comparable to 1.5:1 recorded by Hammad et al12 and 1.6:1 by Nggada et al15. However our study showed more malignancy in females with M: F ratio for malignancy of 0.75:1. Comparable male to female ratios for malignancy were recorded by Featherstone et al14 (0.8 : 1) and Eke et al5 (1:1.09). This is contrary to some studies like Ekenze et al26 (1.1:1 ) and  Ghalayini et al27 (1.3 : 1) where there were more malignancy in males.

On the whole, 60% of the nephrectomies were for Wilms tumor. The contribution of Wilms tumor to nephrectomy in this study is very high when compared to 29% recorded by Daradka et al3, 26% by Sammon et al8, 29.16% by Nouira et al28 and 33% by Choubchab et al6 .

Renal scintigraphy was done only in one patient who had PUV to assess differential renal function. He showed 1% function on one side and hence ipsilateral nephrectomy was done. In other patients with non-malignant renal conditions, no function or marked reduction in renal function were assessed using intravenous urography. Facilities for renal scintigraphy were not readily available in our environment.

There were more nephrectomies on the left side with left nephrectomies done in 55.2% and right nephrectomies done in 44.8% of cases respectively.  This finding corroborates with findings of other studies2,10. Most nephrectomies in PUJ obstruction, which is the most common benign condition for nephrectomy, were done in the age range of 11-16 years (5/9) as shown in table 3.

There was a marginal reduction in total number of nephrectomies from 27 in the first 5 years of the study period to 25 in the second 5-year period (figure 1). In some other studies2, 3, 12, 28,  increase in number of nephrectomies were noted over various time periods while others29 noted no change. Woldrich et al studying an adult population revealed a significant increase in annual incidence attributable to rise in incidental discovery of renal masses on cross-sectional imaging30. The median annual hospital volume in childhood nephrectomy in this study is 8 while in Sammon et al8 it is 12. Mean number per year was 5.

The mean follow-up duration of 26±37 weeks (range 0—156 weeks) is short. It is encouraged that children with a solitary functioning kidney have long-term follow-up until they reach adulthood because they could experience subtle renal deterioration at that time or later in adult life31. Long term follow up is important in early detection of subtle renal deterioration31.

In 2010, Sammon et al in USA noted that 5.8% of their pediatric nephrectomies had minimally invasive nephrectomy, though open trans-abdominal approach was still the most common route for nephrectomy in their series. In the current study, however, there were no facilities and manpower in our hospital for minimally invasive nephrectomy during the study period; hence, as in some other studies4, 6, 7, 12 all nephrectomies were done via the transabdominal route.

In Hammad et al12, the most partial nephrectomies were for duplex renal systems and there was increase in number of partial nephrectomies done over their study period. In the current study, however, only one partial upper pole nephrectomy was done in a patient with duplex system and non-functional upper pole moiety. The lower pole was preserved in this patient. There was no partial nephrectomy for Wilms tumor in the current study. This may be due to the fact that many patients present late as already documented in a previous study from the same environment26. Furthermore, there were no cases of bilateral renal tumors in this study as well as other studies from the same environment26,33. Other authors reported cases of bilateral Wilms tumor in their series and the patients subsequently had nephron-sparing nephrectomy3.

Major post-operative complications were not common as we recorded stitch reaction in 2 patients and hypertrophic scar in 1. Only 1 patient, who had emergency left nephrectomy following low velocity penetrating abdominal injury, had small bowel volvulus with gangrene post-operatively and required re-exploration and bowel resection.

Mortality during the period of hospitalization for nephrectomy was 5.8%. This is high when compared to lower 30-day mortality rates of 0.9% in Jordan27, 0.8% in India2, 1.29% in Pakistan24 and 0.8% in Spain34 found in other series. The mortalities were in 2 Wilms tumor patients (one male and one female) who died on the operating table from intraoperative complications. One male who had PUJ obstruction died in the early post-operative period from an undetermined cause.

Study Limitations

This study is limited by the fact that number of nephrectomies per year is low, follow-up is short and the study is retrospective and done in single paediatric surgery unit. We hereby recommend larger multi-centre prospective studies with long term follow-up periods in assessing indications, pattern and outcome of nephrectomies in our environment.

Conclusions

In our unit, nephroblastoma is the main indication for paediatric nephrectomy and was the only indication in females. Neglected pelviureteric junction obstruction was the major non-malignant indication and occurred only in males. Most nephrectomies were done within the age range of 1-5 years. Nephron-sparing nephrectomy, major morbidity and re-operation are uncommon. In-hospital mortality from nephrectomy is still high at 5.8%.

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