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Percutaneous Decompression: Treatment for Respiratory Distress Secondary to Multicystic Dysplastic Kidney
0022-534 7/90/1441-0113$02,00/0 THE JOURNAL OF UROLOGY
VoL l•A, July
Copyright© 1990 by AMERlCAN UROLOGICAL ASSOCIATION,
Printed
INC,
U.S.A.
PERCUTANEOUS DECOMPRESSION: TREATMENT FOR RESPIRATORY DISTRESS SECONDARY TO MULTICYSTIC DYSPLASTIC KIDNEY WALTER R. HOLLOWAY
AND
STEPHEN H, WEINSTEIN*
From the Department of Surgery, Division of Urology, University of Missouri School of Medicine, Columbia, Missouri
ABSTRACT
Multicystic dysplastic kidney is a common renal anomaly in the newborn. Long-term problems, such as pain, infection, hypertension and neoplasm, although infrequent, have been reported. Acute, life-threatening complications resulting from the size of the affected kidney are rare and emergency nephrectomy has been the only reported effective therapy, We present a case of ultrasound-guided percutaneous cyst decompression used as definitive treatment of respiratory failure associated with multicystic dysplastic kidney. (J, Ural., 144: 113-115, 1990) Although conservative management of multicystic dysplastic kidney is the trend, exploration with nephrectomy is indicated when the diagnosis is unclear, the mass is growing or if the rare complication of physiological compromise secondary to mass size occurs. 1 An emergency major operation in a critically ill neonate can be hazardous but, in the 2 reported cases of respiratory failure resulting from size of the dysplastic kidney, it was life-saving. 2 • 3 We present a case of such a situation when ultrasound-guided percutaneous cyst decompression was used as definitive therapy.
ing right kidney and no uptake of 99 mtechnetium-diethylenetriaminepentaacetic acid on the left side, An excretory urogram (IVP) when the patient was 2 months old confirmed the presence of a normal right system with no opacification of the left renal unit. When he was 2.5 months old left nephrectomy was performed because of a persistently protuberant abdomen and continued displacement of the bowel on x-ray, The specimen measured
CASE REPORT
B. S., a 2,900 gm., white male neonate, was delivered via cesarean section at 36 weeks of gestation to a gravida III, para 2, abortion O woman. Maternal history was significant for insulin-dependent diabetes mellitus. Fetal ultrasound at 30 weeks demonstrated a normal right kidney, while the left kidney measured 6 x 8 cm. with a cystic pattern consistent with either hydronephrosis or multicystic dysplastic kidney (fig. 1), No further anomalies were identified and followup ultrasound scans demonstrated no change. At delivery, respiratory distress with nasal flaring, and intercostal and substernal retractions were noted. The abdomen was protuberant with a large, nodular mass on the left side and a normal palpable right kidney, Physical examination was otherwise normal. Intubation was required due to worsening respiratory distress. Arterial blood gases on 100% oxygen were obtained: pH 7.24, carbon dioxide pressure 50, oxygen pressure 54, and saturation 82%. Pressures of 30 cm. water were required for ventilation. The initial chest/abdomen x-ray showed mild hyaline membrane disease and marked bowel displacement to the right side with significant soft tissue mass effect (fig. 2, A), Electrolytes were within normal limits. Abdominal ultrasound was obtained and diagnosis was multicystic dysplastic kidney. With ultrasound guidance 3 cysts were aspirated yielding a total of 100 cc serous fluid. There appeared to be no communication between the cysts. After decompression of the cysts the respiratory status markedly improved as evidenced by normalized ventilatory pressures and arterial blood gases of pH 7.48, carbon dioxide pressure 24 and oxygen pressure 83, with 96% saturation on room air. A repeat chest/abdomen film showed bowel gas in the left half of the abdomen although the mass effect persisted (fig. 2, B). Weaning from the ventilator was achieved without incident. Renal scan at 9 days after birth showed a normally functionAccepted for publication February 1, 1990, * Requests for reprints: Division of Urology, University of Missouri School of Medicine, One Hospital Dr., Columbia, Missouri 65212,
FIG, L Large multicystic structure (arrows) occupying greater than half of abdominal cross-sectional area, Note normal right kidney between cursors (white cross-hairs),
114
HOLLOWAY AND WEINSTEIN
FIG. 2. A, chest/abdomen x-ray shows shift of bowel gas completely to right half of abdomen. B, post-aspiration chest/abdomen film demonstrates return of bowel gas to left half of abdomen.
FIG. 3. Gross specimen of multicystic dysplastic kidney
10 x 6.5 x 5 cm. Cysts were noncommunicating and varied in size from 0.9 to 4.5 cm. (fig. 3). Histological findings confirmed a typical multicystic dysplastic kidney with focal collections of mature and immature glomeruli along with cartilage and neural elements. DISCUSSION
One of the most common abdominal masses in the newborn, multicystic dysplastic kidney, usually is asymptomatic. 4 Up to 80% of the cases are detected within the first 2 years of life. 3 More recently, in utero diagnosis of multicystic dysplastic kidney has been reported. Avni and associates demonstrated that with the classical ultrasound criteria of multicystic dysplastic kidney in children and adults (presence of interfaces between cysts, nonmedial location of the largest cyst, absence of an identifiable renal sinus, multiplicity of oval or round cysts that do not communicate and absence of parenchymal tissue) the condition could readily be distinguished from genitourinary anomalies in utero. 5 There is no consensus on this matter, although Hadlock and associates believe that the in utero
distinction between ureteropelvic junction obstruction and multicystic dysplastic kidney is not always precise. 6 Complications resulting from multicystic dysplastic kidney are uncommon. Pain, infection, hypertension, malignancy and pressure problems associated with the size of the dysplastic kidney have been noted. 1 • 7 Only 2 cases of respiratory embarrassment resulting from volume displacement have been reported. 2 , 3 A male newborn had onset of respiratory distress immediately after delivery. From physical examination and an IVP multicystic dysplastic kidney was suspected. Emergency nephrectomy was not only diagnostic but also curative and completely relieved the respiratory distress. One patient presented with respiratory failure, compromise of gastric volume and contralateral ureteral compression. This patient also had multiple other congenital anomalies. While percutaneous aspiration of the cystic structure provided temporary improvement in the respiratory status, emergency nephrectomy was required and resulted in normalization of respiratory, gastric and contralateral urinary function. Percutaneous aspiration for diagnosis and therapy of renal masses in children has been described. 8 - 11 Most uses have related to decompression of hydronephrosis secondary to urinary tract obstruction. In several cases pressure phenomena related to the size of the hydronephrotic kidney were lifethreatening and required emergency percutaneous nephrostomy. 9· 10 Techniques are comparable to those commonly used in adults. Cyst aspiration and decompression for treatment of high renin hypertension, presumably induced by mechanical segmental ischemia, have also been described. 9 • 12 ' 13 No case of volume displacement-induced respiratory failure primarily treated with cyst aspiration and decompression was identified in our review. In our patient a large cystic left kidney was found on prenatal ultrasound. The differential diagnosis included hydronephrosis and multicystic dysplastic kidney. Upon delivery, pressure from the large kidney resulted in progressive respiratory distress with severe hypoxemia. Despite 100% oxygen concentration the hypoxemia persisted and high ventilatory pressures were required. Transabdominal percutaneous aspiration via ultra-
MULTICYSTIC KIDNEY: EMERGENCY PERCUTANEOUS DECOMPRESSION
sound guidance allowed for partial decompression of the cysts with marked improvement in arterial oxygenation and ventilatory pressures. Bowel displacement was lessened. There were no further pressure-associated problems and the patient was weaned from the respirator uneventfully. By using a relatively noninvasive, low morbidity procedure the critically ill neonate was definitively treated, thereby avoiding nephrectomy when it would be least tolerated. We present a case in which ultrasound-guided cyst aspiration and decompression were used as definitive therapy for respiratory distress associated with multicystic dysplastic kidney. REFERENCES
1. Mandell, J.: Management of multicystic dysplastic kidney. Dial.
Ped. Urol., 10: No. 6, 1987. 2. Wise, H. A., II and Schirmer, H. K. A.: An unusual cause of respiratory distress in a newborn. J. Urol., 108: 792, 1972. 3. Middleton, A. W., Jr. and Melzer, R. B.: Neonatal multicystic kidney with associated respiratory distress, obstruction of contralateral ureter, and gastric compromise. Urology, 34: 36, 1989. 4. Emanuel, B. and White, H.: Intravenous pyelography in the differential diagnosis of renal masses in the neonatal period. Clin. Ped., 7: 529, 1968.
115
5. Avni, E. F.i Thoua, Y., Lalmand, B., Didier, F., Droulle, P. and Schulman, C. C.: Multicystic dysplastic kidney: natural history from in utero diagnosis and postnatal followup. J. Urol., 138: 1420, 1987. 6. Hadlock, F. P., Deter, R. L., Carpenter, R., Gonzalez, E. T. and Park, S. K.: Sonography of fetal urinary tract anomalies. AJR, 137: 261, 1981. 7. Peters, C. A. and Mandell, J.: The multicystic dysplastic kidney. AUA Update Series, vol. 8, lesson 7, pp. 50-55, 1989. 8. Steinhardt, G. F., Slovis, T. L. and Perlmutter, A. D.: Simple renal cysts in infants. Radiology, 155: 349, 1985. 9. Hoard, T. D. and O'Brien, D. P., III: Simple renal cyst and high renin hypertension cured by cyst decompression. J. Urol., 115: 326, 1976. 10. Bartone, F. F., Mazer, M. J., Anderson, J. C., Bickers, G. H. and Baltaxe, H. A.: Diagnosis and treatment of fluid-filled renal structures in children with ultrasonography and percutaneous puncture. Urology, 16: 432, 1980. 11. Macpherson, R. I. and Reed, M. H.: Percutaneous puncture of abdominal cystic masses in children. J. Canad. Ass. Rad., 30: 140, 1979. 12. Rockson, S. G., Stone, R. A. and Gunnells, J. C., Jr.: Solitary renal cyst with segmental ischemia and hypertension. J. Urol., 112: 550, 1974. 13. Babka, J. C., Cohen, M. S. and Sode, J.: Solitary intrarenal cyst causing hypertension. New Engl. J. Med., 291: 343, 1974.
VoL l•A, July
Copyright© 1990 by AMERlCAN UROLOGICAL ASSOCIATION,
Printed
INC,
U.S.A.
PERCUTANEOUS DECOMPRESSION: TREATMENT FOR RESPIRATORY DISTRESS SECONDARY TO MULTICYSTIC DYSPLASTIC KIDNEY WALTER R. HOLLOWAY
AND
STEPHEN H, WEINSTEIN*
From the Department of Surgery, Division of Urology, University of Missouri School of Medicine, Columbia, Missouri
ABSTRACT
Multicystic dysplastic kidney is a common renal anomaly in the newborn. Long-term problems, such as pain, infection, hypertension and neoplasm, although infrequent, have been reported. Acute, life-threatening complications resulting from the size of the affected kidney are rare and emergency nephrectomy has been the only reported effective therapy, We present a case of ultrasound-guided percutaneous cyst decompression used as definitive treatment of respiratory failure associated with multicystic dysplastic kidney. (J, Ural., 144: 113-115, 1990) Although conservative management of multicystic dysplastic kidney is the trend, exploration with nephrectomy is indicated when the diagnosis is unclear, the mass is growing or if the rare complication of physiological compromise secondary to mass size occurs. 1 An emergency major operation in a critically ill neonate can be hazardous but, in the 2 reported cases of respiratory failure resulting from size of the dysplastic kidney, it was life-saving. 2 • 3 We present a case of such a situation when ultrasound-guided percutaneous cyst decompression was used as definitive therapy.
ing right kidney and no uptake of 99 mtechnetium-diethylenetriaminepentaacetic acid on the left side, An excretory urogram (IVP) when the patient was 2 months old confirmed the presence of a normal right system with no opacification of the left renal unit. When he was 2.5 months old left nephrectomy was performed because of a persistently protuberant abdomen and continued displacement of the bowel on x-ray, The specimen measured
CASE REPORT
B. S., a 2,900 gm., white male neonate, was delivered via cesarean section at 36 weeks of gestation to a gravida III, para 2, abortion O woman. Maternal history was significant for insulin-dependent diabetes mellitus. Fetal ultrasound at 30 weeks demonstrated a normal right kidney, while the left kidney measured 6 x 8 cm. with a cystic pattern consistent with either hydronephrosis or multicystic dysplastic kidney (fig. 1), No further anomalies were identified and followup ultrasound scans demonstrated no change. At delivery, respiratory distress with nasal flaring, and intercostal and substernal retractions were noted. The abdomen was protuberant with a large, nodular mass on the left side and a normal palpable right kidney, Physical examination was otherwise normal. Intubation was required due to worsening respiratory distress. Arterial blood gases on 100% oxygen were obtained: pH 7.24, carbon dioxide pressure 50, oxygen pressure 54, and saturation 82%. Pressures of 30 cm. water were required for ventilation. The initial chest/abdomen x-ray showed mild hyaline membrane disease and marked bowel displacement to the right side with significant soft tissue mass effect (fig. 2, A), Electrolytes were within normal limits. Abdominal ultrasound was obtained and diagnosis was multicystic dysplastic kidney. With ultrasound guidance 3 cysts were aspirated yielding a total of 100 cc serous fluid. There appeared to be no communication between the cysts. After decompression of the cysts the respiratory status markedly improved as evidenced by normalized ventilatory pressures and arterial blood gases of pH 7.48, carbon dioxide pressure 24 and oxygen pressure 83, with 96% saturation on room air. A repeat chest/abdomen film showed bowel gas in the left half of the abdomen although the mass effect persisted (fig. 2, B). Weaning from the ventilator was achieved without incident. Renal scan at 9 days after birth showed a normally functionAccepted for publication February 1, 1990, * Requests for reprints: Division of Urology, University of Missouri School of Medicine, One Hospital Dr., Columbia, Missouri 65212,
FIG, L Large multicystic structure (arrows) occupying greater than half of abdominal cross-sectional area, Note normal right kidney between cursors (white cross-hairs),
114
HOLLOWAY AND WEINSTEIN
FIG. 2. A, chest/abdomen x-ray shows shift of bowel gas completely to right half of abdomen. B, post-aspiration chest/abdomen film demonstrates return of bowel gas to left half of abdomen.
FIG. 3. Gross specimen of multicystic dysplastic kidney
10 x 6.5 x 5 cm. Cysts were noncommunicating and varied in size from 0.9 to 4.5 cm. (fig. 3). Histological findings confirmed a typical multicystic dysplastic kidney with focal collections of mature and immature glomeruli along with cartilage and neural elements. DISCUSSION
One of the most common abdominal masses in the newborn, multicystic dysplastic kidney, usually is asymptomatic. 4 Up to 80% of the cases are detected within the first 2 years of life. 3 More recently, in utero diagnosis of multicystic dysplastic kidney has been reported. Avni and associates demonstrated that with the classical ultrasound criteria of multicystic dysplastic kidney in children and adults (presence of interfaces between cysts, nonmedial location of the largest cyst, absence of an identifiable renal sinus, multiplicity of oval or round cysts that do not communicate and absence of parenchymal tissue) the condition could readily be distinguished from genitourinary anomalies in utero. 5 There is no consensus on this matter, although Hadlock and associates believe that the in utero
distinction between ureteropelvic junction obstruction and multicystic dysplastic kidney is not always precise. 6 Complications resulting from multicystic dysplastic kidney are uncommon. Pain, infection, hypertension, malignancy and pressure problems associated with the size of the dysplastic kidney have been noted. 1 • 7 Only 2 cases of respiratory embarrassment resulting from volume displacement have been reported. 2 , 3 A male newborn had onset of respiratory distress immediately after delivery. From physical examination and an IVP multicystic dysplastic kidney was suspected. Emergency nephrectomy was not only diagnostic but also curative and completely relieved the respiratory distress. One patient presented with respiratory failure, compromise of gastric volume and contralateral ureteral compression. This patient also had multiple other congenital anomalies. While percutaneous aspiration of the cystic structure provided temporary improvement in the respiratory status, emergency nephrectomy was required and resulted in normalization of respiratory, gastric and contralateral urinary function. Percutaneous aspiration for diagnosis and therapy of renal masses in children has been described. 8 - 11 Most uses have related to decompression of hydronephrosis secondary to urinary tract obstruction. In several cases pressure phenomena related to the size of the hydronephrotic kidney were lifethreatening and required emergency percutaneous nephrostomy. 9· 10 Techniques are comparable to those commonly used in adults. Cyst aspiration and decompression for treatment of high renin hypertension, presumably induced by mechanical segmental ischemia, have also been described. 9 • 12 ' 13 No case of volume displacement-induced respiratory failure primarily treated with cyst aspiration and decompression was identified in our review. In our patient a large cystic left kidney was found on prenatal ultrasound. The differential diagnosis included hydronephrosis and multicystic dysplastic kidney. Upon delivery, pressure from the large kidney resulted in progressive respiratory distress with severe hypoxemia. Despite 100% oxygen concentration the hypoxemia persisted and high ventilatory pressures were required. Transabdominal percutaneous aspiration via ultra-
MULTICYSTIC KIDNEY: EMERGENCY PERCUTANEOUS DECOMPRESSION
sound guidance allowed for partial decompression of the cysts with marked improvement in arterial oxygenation and ventilatory pressures. Bowel displacement was lessened. There were no further pressure-associated problems and the patient was weaned from the respirator uneventfully. By using a relatively noninvasive, low morbidity procedure the critically ill neonate was definitively treated, thereby avoiding nephrectomy when it would be least tolerated. We present a case in which ultrasound-guided cyst aspiration and decompression were used as definitive therapy for respiratory distress associated with multicystic dysplastic kidney. REFERENCES
1. Mandell, J.: Management of multicystic dysplastic kidney. Dial.
Ped. Urol., 10: No. 6, 1987. 2. Wise, H. A., II and Schirmer, H. K. A.: An unusual cause of respiratory distress in a newborn. J. Urol., 108: 792, 1972. 3. Middleton, A. W., Jr. and Melzer, R. B.: Neonatal multicystic kidney with associated respiratory distress, obstruction of contralateral ureter, and gastric compromise. Urology, 34: 36, 1989. 4. Emanuel, B. and White, H.: Intravenous pyelography in the differential diagnosis of renal masses in the neonatal period. Clin. Ped., 7: 529, 1968.
115
5. Avni, E. F.i Thoua, Y., Lalmand, B., Didier, F., Droulle, P. and Schulman, C. C.: Multicystic dysplastic kidney: natural history from in utero diagnosis and postnatal followup. J. Urol., 138: 1420, 1987. 6. Hadlock, F. P., Deter, R. L., Carpenter, R., Gonzalez, E. T. and Park, S. K.: Sonography of fetal urinary tract anomalies. AJR, 137: 261, 1981. 7. Peters, C. A. and Mandell, J.: The multicystic dysplastic kidney. AUA Update Series, vol. 8, lesson 7, pp. 50-55, 1989. 8. Steinhardt, G. F., Slovis, T. L. and Perlmutter, A. D.: Simple renal cysts in infants. Radiology, 155: 349, 1985. 9. Hoard, T. D. and O'Brien, D. P., III: Simple renal cyst and high renin hypertension cured by cyst decompression. J. Urol., 115: 326, 1976. 10. Bartone, F. F., Mazer, M. J., Anderson, J. C., Bickers, G. H. and Baltaxe, H. A.: Diagnosis and treatment of fluid-filled renal structures in children with ultrasonography and percutaneous puncture. Urology, 16: 432, 1980. 11. Macpherson, R. I. and Reed, M. H.: Percutaneous puncture of abdominal cystic masses in children. J. Canad. Ass. Rad., 30: 140, 1979. 12. Rockson, S. G., Stone, R. A. and Gunnells, J. C., Jr.: Solitary renal cyst with segmental ischemia and hypertension. J. Urol., 112: 550, 1974. 13. Babka, J. C., Cohen, M. S. and Sode, J.: Solitary intrarenal cyst causing hypertension. New Engl. J. Med., 291: 343, 1974.