Correspondence *Department of Urology, Guy's and St Thomas' Hospitals, St Thomas Street, London SE1 9RT, UK

Email prokarurol@gmail.com

The case

A 32-year-old woman was referred to our clinic for consideration of minimally invasive surgical management of her symptomatic left giant hydronephrosis. During the preceding year she had experienced repeated episodes of left loin pain and swelling associated with a palpable left kidney and a single documented Escherichia coli urinary tract infection (UTI). At the first consultation her urine was sterile and renal function normal (serum creatinine 60 mol/l). An ultrasound scan showed a markedly hydronephrotic left kidney (approximately 18 cm bipolar length) with a very thin cortex and a normal right kidney (10 cm bipolar length). Intravenous urography demonstrated a delayed left sided nephrogram with no contrast seen in the ureter at 4 hours (Figure 1), and a Technetium⁹⁹-labelled mercaptoacetyltriglycine (MAG-3) renogram suggested minimal left kidney function (Figure 2). Together, these findings were in keeping with pelvi-ureteric junction obstruction (PUJO).

Figure 1 Intravenous urograph

The 4-hour delayed IVU film demonstrates a normal right kidney and giant hydronephrosis of the left kidney with no contrast seen in the left ureter, suggesting PUJO as the cause of obstruction. Abbreviations: IVU, intravenous urography; PUJO, pelvi-ureteric junction obstruction.

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Figure 2 MAG-3 renogram

The gamma camera is positioned behind the patient. The radionucleotide image shows a poorly functioning hydronephrotic left kidney and a normal right kidney. Abbreviation: MAG-3, mercaptoacetyltriglycine.

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While awaiting elective laparoscopic left nephrectomy, the patient informed us that she was 4 weeks pregnant. This was an 'unplanned' first pregnancy, which she hoped to complete in her home country of Poland.

The patient presented at 8 weeks gestation with severe loin pain and a left-sided abdominal swelling measuring approximately 5–6 cm in diameter. She required hospital admission for management of the severe pain from her giant hydronephrosis. She was jointly managed by the urology and obstetric teams. Ultrasonography confirmed a viable first trimester pregnancy. Treatment was commenced with prophylactic, low-dose cephalosporin antibiotics. Her loin pain slightly improved with lying prone, but she continued to require opiate analgesia.

The patient was presented with three treatment options, which included conservative management, prolonged nephrostomy drainage throughout the remainder of the pregnancy or retroperitoneal nephrectomy during first trimester (either open or laparoscopic). The risks associated with prolonged nephrostomy drainage include infection, premature delivery and the need for change of nephrostomy during pregnancy to prevent encrustation; the patient was intending to travel overseas later in the pregnancy and may not have been able to do so with a nephrostomy in situ. The risks associated with nephrectomy include miscarriage and the standard risks of surgery. The patient, following counselling, opted for retroperitoneoscopic nephrectomy at 9 weeks pregnancy in view of her persistent symptoms; she was fully aware of the fetal risks, including miscarriage.

A left retroperitoneoscopic nephrectomy was performed under general anesthesia and amoxicillin cover, using a standard three-port technique. Balloon dilatation of the retroperitoneal space was performed using a visual balloon dilating trocar (balloon inflated to 500 ml). CO₂ insufflation was commenced and maintained at 10 mmHg. The Gerota's fascia was opened, and the giant hydronephrosis was identified and dissected intact. The renal artery and vein were clipped and cut. The dilated collecting system was punctured under vision and the urine was aspirated. The ureter was clipped and cut. The kidney was bagged and removed by extending the middle camera port. A 20 F Robinson's drain was placed in the perinephric space. The total operating time was 180 minutes and the estimated blood loss was 10 ml. The drain and urinary catheter were removed on day 2 post-surgery.

Surgery and the postoperative course were uncomplicated. Ultrasonography on the second postoperative day confirmed fetal viability. The patient was discharged on the seventh postoperative day. At week 3 post-surgery, she was well, had excellent wound healing and a viable pregnancy.

She travelled during her second trimester to complete her pregnancy in Poland. She delivered, per vaginum, a healthy 3.2 kg boy at term without complication.

Discussion of diagnosis

Giant hydronephrosis was first reported over 50 years ago, and describes massive renal pelvi-calyceal dilation, usually secondary to PUJO.¹ In adults the renal pelvis is often larger than 5 cm, and can present as a palpable abdominal mass. The hydronephrosis causes compression of the renal parenchyma, often with only a thin rim of renal cortex remaining, which leads to a non-functioning kidney.

E. coli UTI during pregnancy is very common, and, with a non-functioning hydronephrosis, the potentially serious consequence of pyonephrosis exists. Furthermore, there is an association between UTI in pregnant women and preterm birth and/or low birth weight, but not between UTI and congenital abnormalities.² Abdominal pain that is unrelated to the pregnancy and that requires surgery is uncommon, and occurs in 2 pregnancies per 1000; however, indications for surgery can be affected by the altered abdominal anatomy and the physiological changes of pregnancy.

The combined radiological features of left-sided giant hydronephrosis with thin renal cortex, no dilated ureter, delayed pyelogram and poor renal function were all in agreement with chronic left PUJO.

Treatment and management

The management of giant hydronephrosis depends on the presence or absence of sepsis, and on residual renal function in the obstructed kidney and the contra-lateral renal unit.

Sepsis warrants urgent decompression, usually by percutaneous nephrostomy. In a sterile giant hydronephrosis, the options include observation, nephrectomy and, occasionally, internal ureteric stent or nephrostomy for patients unfit for surgery. Nephrectomy is advised for pain and recurrent infection in a non-functioning kidney. Rarely, if renal function is preserved in giant hydronephrosis, pyeloplasty can be considered. Observation and stents are generally reserved for those in whom surgery may be hazardous, for example in patients with co-morbidity or a solitary kidney.

Maternal ill-health during pregnancy involves difficult management decisions in view of potential risks to both mother and fetus; therefore, the management of giant hydronephrosis during pregnancy is complex. Giant hydronephrosis in pregnancy was reported over 40 years ago, including descriptions of both intervention and drainage of the kidney, and of spontaneous delivery.^{3, 4, 5}

Currently, when PUJO complicates pregnancy, the risks to both patient and fetus need careful consideration. The risks to the fetus from conservative observational management include an increased chance of miscarriage or premature labour from recurrent UTI, and the consequences of prolonged pain throughout pregnancy. The interventional options for symptomatic non-functioning giant hydronephrosis, however, also carry risks to the fetus. Flexible cystoscopy and retrograde stent insertion can be performed under local anesthetic, and are recommended for obstructed ureteric calculi during pregnancy. This procedure is, however, associated with a small miscarriage rate. Nephrostomies can be safely inserted during pregnancy; however, with placement during early pregnancy there is an increased chance of the nephrostomy either falling out or becoming calcareous, which will necessitate repeat nephrostomy replacement throughout pregnancy.

It is generally recommended to delay surgery during pregnancy, if possible, until the second trimester in order to avoid the period of organogenesis (first trimester) and the risk of preterm labour (third trimester). The decision to operate is always complex; delayed intervention can result in premature labour or fetal demise, and unnecessary surgery is risky for both mother and fetus.

The physiological changes that occur during pregnancy have to be considered by both the surgeon and the anesthetist. These include hyper-coagulability and increased plasma volume, decreased colloid oncotic pressure, aortocaval compression in later pregnancy causing decreased placental blood flow, and gastro-oesophageal reflux secondary to the thoracic displacement of the intra-abdominal oesophagus.

The risks associated with open nephrectomy during pregnancy are high. In instances of bleeding angiomyolipoma, renal artery aneurysms or renal cell carcinoma, the risk of fetal loss must be balanced with maternal safety, which is paramount.

Laparoscopy in pregnant women has been used over the last 10 years with increasing confidence. Laparoscopy allows rapid postoperative mobilization, thus decreasing the risk of thrombo-embolic events. Decreased postoperative analgesic requirements, maternal hypoventilation and wound complications all have positive effects on fetal well-being.

Laparoscopic cholecystectomy, appendicectomy and laparoscopic management of ovarian disease in pregnancy are now established.^{6, 7} More recently, two reported cases of maternal renal cell carcinoma in pregnancy (one in the first and the other in the second trimester) were treated by laparoscopic nephrectomy.^{8, 9}

Laparoscopic procedures during pregnancy are not without risk, however, and a number of pregnancy-related complications have been reported. Friedman et al.¹⁰ reported a misplaced Veress needle entering a gravid uterus at 21 weeks, which led to subsequent pneumo-amnion and delivery of a stillborn fetus. Early studies of laparoscopic cholecystectomy and appendicectomy during pregnancy reported two intrauterine fetal deaths, as well as two incomplete abortions.¹¹ Three of the four adverse events occurred in the immediate postoperative period, and the other occurred 1 month later.

It might be that many of the complications associated with laparoscopy during pregnancy arise from the CO₂ pneumo-peritoneum used in trans-peritoneal laparoscopic procedures. The pneumo-peritoneum increases intra-abdominal pressure, which decreases venous return and cardiac output. There also seems to be a suppression of splanchnic blood flow, which, if prolonged, gives rise to anaerobic metabolism and lactic acidosis.¹² Metabolic effects include respiratory acidosis, which can lead to tachyarrhythmias if it is not compensated for.

A retroperitoneal approach can be used when performing nephrectomy; surgeons should be encouraged to use this route, especially in pregnant patients. Not only are the metabolic and mechanical effects of the CO₂ pneumo-peritoneum reduced, there is also the added advantage of decreased ileus, bowel injury and port site hernias.

Conclusions

Giant hydronephrosis associated with UTI during pregnancy can result in potentially serious consequences to both mother and fetus. A multidisciplinary approach to such cases is important. Counselling and informed consent from the patient in such cases is paramount as the published literature in this area is limited. We believe that retroperitoneoscopic nephrectomy offered the patient the best option for both her own health and the health of her fetus.

References

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Subject areas under which this article appears: Female urology