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Polycystic Kidney Disease1
POLYCYSTIC KIDNEY DISEASE 1 MORTON M. MAYERS From the Department of Urology, Ross-Loos JJ1edical Group, Los Angeles, Calif.
Conservative surgical treatment for polycystic kidney disease is not new. In 1910 Rovsing described a procedure wherein the kidney is exposed and the cysts punctured and aspirated. Universal acceptance was not forthcoming and the procedure actually fell into disrepute because the end results did not justify the risk. Few differ regarding the need of surgery for the complications of polycystic kidney disease common to other kidney conditions such as stone, tumor and perinephric abscess. As to surgery for the disease per se there is a divergence of viewpoint with the weight of opinion favoring a "do-nothing" attitude. If one is willing to accept the conclusion that conservative surgery has no place in hydronephrosis, and that the surgical treatment of prostatic enlargement should be postponed until urination is greatly hindered or renal function is decidedly impaired, then an attitude of procrastination and expectancy in the treatment of polycystic kidney disease is compatible. All three have in common a gradual impairment of renal function consequent to destruction of renal parenchyma. The argument of doing nothing unless certain complications dictate otherwise is based on an apparently sound observation that often those afflicted ·with this disease live on for many years. This is shallow reasoning and as cogent as allowing hydronephrosis to progress to utter destruction of the kidney because the patient is symptomless. Prior lack of demonstrated success for surgery may have been an adequate excuse for avoiding surgery. If some new procedure gives promise of a brighter horizon, it should be used until its ultimate results either prove or disprove its value. With this in mind there are those who have been daring and are yet sufficiently conservative, to attempt something which might make for more comfort, or prolong life, or both. In 1935, A. E. Goldstein described his operation of marsupialization. In this, the kidney is exposed, decapsulated if possible, accessible cysts excised or otherwise destroyed, deeper cysts aspirated, the kidney partially bivalved, and the wound closed by suturing the wound edges to the edges of the split kidney. The end result was a wide scar through which enlarging cysts could be aspirated. Recently (April 1946) W. W. Young described a method of sclerosing these cysts with 5 per cent sodium morrhuate solution. He applied this method with satisfactory results in a 17 year old woman. The left kidney was exposed, cysts were aspirated and all large cysts were then injected with 5 per cent sodium morrhuate, using approximately 1 cc for each 10 cc of fluid withdrawn. The incision was then closed in the usual manner. Under observation for 4 years she not only was symptom free but had passed through a successful pregnancy. 1 Read at annual meeting, Western Section, American Urological Association, Yosemite Valley, Calif., May 21-23, 1947. 471
472
MORTON M. MAYERS
Young admits that this one case does not constitute real proof of the efficacy of sclerosing treatment, but he believes it is encouragement for further effort in this direction. The advantage of Goldstein's operation is that recurring enlargement of cysts can be aspirated with maximurn safety. The advantage of sclerosing cysts for permanent eradication as in Young's procedure is apparent. The disadvantage of his operation is that recurring cystic enlargements cannot be treated except by re-exposing the kidney. It occurred to us that a modified combination of both the Goldstein and Young procedures would be more ideal. We eliminate the incision into the kidney, treat larger cysts with sclerosing solution at the time of renal exposure, and marsupialize the kidney so that subsequent aspirations and injections could be done with safety. Our experience in treating over 200 hydroceles and spermatoceles with sclerosing agents had proved to us that quinine dihydrochloride 13.33 per cent (or quinine hydrochloride 13.33 per cent and urethane 6.66 per cent) causes less reaction than sodium morrhuate. As further support for the procedure of sclerosing renal cysts, Young cites Fish who reported 2 cases of solitary cyst cured by aspiration and instillation of 50 per cent dextrose solution. In Case 4 following, a combination of marsupialization and sclerosis was employed for the first time. CASE REPORTS
· Case 1.
Mrs. H. G. A., aged 54 years, was first seen on September 8, 1934. Entering the hospital after falling unconscious, she complained of severe neck pain. Her blood pressure was 188/98, the blood nonprotein nitrogen content was 117 mg. per cent, and the creatinin nitrogen 3.2 mg. per cent. The hemoglobin was 44 per cent, and the urine showed 5 pus cells and 2 red blood cells per high power field. On September 11, the nonprotein nitrogen content of the blood was 145 mg. per cent. The right kidney was markedly enlarged. As fatality appeared imminent the Rovsing operation was performed September 14. The patient expired on September 15. Case 2. Mrs. E. N., aged 38 years, entered the hospital March 21, 1936. She gave a history of severe pain over the left lumbar region with fever of 2 weeks duration. A similar episode occurred 9 years previously followed by poor health ever since. The pain was so severe that she was hospitalized as a probable "surgical belly". The nonprotein nitrogen content of the blood was 32 mg. per cent and the hemoglobin 72 per cent. The urine contained numerous pus cells. A cystoscopic study revealed polycystic kidney disease with B. coli infection. The patient improved under medical management and was discharged on April 5, 1936. She was readmitted on February 23, 1937 with a history of invalidism since her previous discharge. Her hemoglobin was 62 per cent and the nonprotein nitrogen 54 mg. per cent. On March 24, 1937, the Goldstein operation was performed on the left side. The wound healed uneventfully except for a mild pyocyaneus infection.
In 1929 a left nephrectomy had been performed.
POLYCYSTIC KIDNEY DISEASE
473
The patient was readmitted to the hospital on July 17, 1937, because of dull pain in the right upper quadrant with intermittent sharp pain, nausea with vomiting for 1 month, chills and fever for 1 week, and dysuria for several months. Examination revealed a tender, enlarged, right kidney. The left kidney was slightly enlarged, but free from symptoms. The hemoglobin was 62 per cent, the white blood cells numbered 22,800, and the nonprotein nitrogen was 53 mg. per cent. On July 21, 1937, a surgical procedure was carried out on the right side, similar to that done on the left side in March. Approximately 1 year later, the patient reported at the office because of severe pain in the right kidney region following a fall. Through the scar, no anesthesia being required, about 25 cc of dark, sanguinous fluid was removed with complete relief of pain. The patient remained asymptomatic and able to carry on normal activities, until 4 years later. On February 22, 1941, she had intense pain in the lower left side with nausea and vomiting. The temperature was 101.8°F., the nonprotein nitrogen 120 mg. per cent, and the phenosulphonphthalein excretion, 3.5 per cent in 2 hours. By aspiration through the scars, 15 cc of fluid were removed from the left kidney and a few cubic centimeters from the right. Despite supportive treatment the blood nonprotein nitrogen content rose to 148 mg. per cent and the patient expired on March 23, 1941. Comment: The result of the application of the Goldstein procedure in this case must be considered excellent. For several years before surgery, the patient had been an invalid unable to perform ordinary housework. Following operation on the left side, no symptoms developed on that side until 4 years later, just preceding death from renal failure. Aspiration was found to be necessary on one occasion with dramatic relief of pain and with that one exception, symptoms on the right cleared up following operation on the right kidney. Case 3. Mrs. M. C., aged 44 years, was first seen at the hospital on February 16, 1941. There had been gradually increasing weakness for several years. One week preceding admission, leg edema developed. Pain on the left side and headache were also present. The blood pressure was 190/110. Two large abdominal masses were felt, and pitting edema of the extremities was present. The hemoglobin was 44 per cent and the urine contained 3 to 6 pus cells per high power field. No phenosulphonphthalein appeared in the urine in 20 minutes. After a transfusion of 2000 cc of whole blood, the hemoglobin rose to 72 per cent. On February 27, the nonprotein nitrogen was 87 mg. per cent. On March 8 the urea clearance was 5 per cent, and the phenolsulphonphthalein test showed a trace of dye in 2 hours. Op. March 14, 1941 the Goldstein procedure, modified by eliminating the incision into the kidney, was carried out on .the right side resulting in reduction in size of the kidney to less than one-half. On the fourth postoperative day the blood nonprotein nitrogen was 95 mg. per cent, on the fifth day, 104, on the eleventh day, 120, and on the twenty-fourth day, 115 mg. per cent. Despite these rising values there was clinical improvement, the wound granulated nicely and the patient became ambulatory. The nonprotein nitrogen was 102 mg. per cent on the day of discharge from the hospital, April
474
MORTON M. MAYERS
16, 1941. Two weeks later the patient reported complaining of pain in the right side. Examination revealed a bulging mass in the right renal area which was aspirated, 50 cc of fluid being removed. Unfortunately a large vessel was punctured due to overzealous needling close to the pedicle, and resulted in severe hemorrhage into the bladder necessitating surgery. The patient expired May 10 in spite of transfusions and supportive treatment. Comment: Surgery was elected for this evident terminal case in the hope that death could be forestalled. It was gratifying to see definite symptomatic improvement following the Goldstein procedure. Case 4. Miss L. P., aged 49 years, was admitted to the hospital on April 15, 1946 with a temperature of 102°. One week previously she had chills and fever, dysuria, frequency, and abdominal pains, chiefly the right side. In 1936 a diagnosis of polycystic kidney disease had been made, but no treatment was given. Tue patient's mother had polycystic kidney disease and the maternal grandmother had died as a result of kidney disease. The blood pressure was 120/80. Both kidneys were enlarged but not tender. Treatment with penicillin, sulfathiazole and sulfadiazine was instituted. Excretory pyelograms revealed bilateral polycystic kidneys. The temperature remained high for 3 weeks and then dropped to 99°. On April 15, the specific gravity of the urine was 1.016, there was a trace of albumin, and numerous red blood cells were present. On April 16, the hemoglobin was 77 per cent, the red blood cells 4,480,000 and the white blood cells 12,800 of which 82 per cent were polymorphonuclear cells. The nonprotein nitrogen was 37 mg. per cent. The patient was discharged May 12, 1946. On readmission to the hospital on June 3, 1946, an examination revealed that the left kidney now filled the left half of the abdomen. The phenolsulphonphthalein test showed an appearance time of 6½ minutes on the right and in 11 minutes on the left, the total in 2-hour excretion being 61 per cent. Pyelography revealed essentially the same findings as before but the left renal shadow was much larger. On June 12, the total blood protein value was 7.1 per cent and the hemoglobin 84 per cent. On June 18, 1946 at operation the left kidney was found tremendously enlarged, filling the left half of the abdomen and containing innumerable cysts, pin-head to an orange in size, filled with clear fluid or pus. The cysts were aspirated, 500 cc of fluid being obtained, including 300 cc of pus. After aspirating the larger cysts, 13.3 per cent of quinine dihydrochloride solution was injected, using approximately 1 cc for each 10 cc removed. The actual cautery was applied to the surface cysts. Through and through mattress sutures of nylon penetrating kidney, Gerota's capsule and the body wall were inserted and moist dressings applied. On July 16, the hemoglobin was 62 per cent, the nonprotein nitrogen 39 per cent, the CO2 combining power of the blood was 54, the blood chloride content 460 mg. per cent and the total protein content, 5 gm. On that day, 200 cc of fluid (including 50 cc of purulent material) were aspirated through the wound. Improvement followed although the temperature remained at 101 ° to 102°F. Penicillin and blood transfusion were given. Ure-
POLYCYSTIC KIDNEY DISEASE
475
teral obstruction was suspected but cystoscopic examination on July 18 revealed none. Aspiration of the cysts was carried out several times. On July 24, 450 cc of purulent fluid was removed from a single cyst in the lower pole, and 8 cc of quinine dihydrochloride solution, 13.33 per cent, was injected. On July 25, the hemoglobin was 69 per cent and on August 7, 66 per cent. The urine contained albumin and numerous pus cells but no red blood cells. On September 28 the patient was discharged from the hospital after a stormy course. The kidney was markedly reduced in size. Improvement has since been progressive. The left kidney is now not much larger than a normal kidney. On January 2, 1947, the right pyelogram revealed little change, the left suggested only vaguely a polycystic kidney. Phenolsulphonphthalein appeared in 3 minutes from the left kidney and ~n 4 minutes from the right and the excretion in the following 20 minutes was 16 per cent for the right, and 7 per cent for the left. Urine from the right kidney was sterile; that from the left contained numerous pus cells and B. coli on culture. Two months later all urine specimens became sterile and have remained sterile to date. The patient is now in a satisfactory physical condition still retaining a kidney which may serve her well as the function decreases on the opposite side. (As of January 1948, she is engaged in her profession of school teaching.) SUMMARY
Conservative surgery for polycystic kidney disease was given its greatest impetus by Rovsing in 1910, by his cyst-puncturing operation. Surgery for the usual complications receives acquiescence but for treatment of the disease per se, the ·weight of opinion has favored a "watchful-waiting" attitude. Although the incidence of this disease in over 1,300,000 clinical admissions was approximately 1 in 4000, it was 1 in 400 in 87,000 autopsies. Such a disparity can only be explained by an acknowledgment that the disease is responsible for far more morbidity and mortality than is generally recognized. In the adult form, the average age at onset of symptoms and the average age at death is 38 and 50 respectively, further emphasizing its severity and possibly reflecting the attitude of no surgical interference until irreversible damage is done. The daring and yet sufficiently conservative procedure of Albert Goldstein in 1936 in "marsupializing" the kidney so that cysts could be aspirated postoperatively, folluwed by the recent work of W. W. Young in using 5 per cent sodium morrhuate for sclerosing the cysts, suggested a procedure combining the advantage of both these operations. The author used both marsupialization and sclerosing therapy so that aspiration of cysts and the injection of a sclerosing substance could be continued postoperatively. Quinine hydrochloride and dihydrochloride are preferred to sodium morrhuate as being less reactive. Two cases are presented in which a modified Goldstein procedure had been performed with results classified as good in one and poor in the other. A case in which the author employed a modified Goldstein-Young technique with apparently excellent results is described in detail.
476
MORTON M. MAYERS CONCLUSION
It is not to be construed that the author advocates routine surgical intervention for polycystic kidney disease. Being cognizant of the fact that innumerable small cysts comprise the bulk of the pathology, and as such cannot be eradicated by any method heretofore suggested, careful selectivity of cases is advised. When one suspects large cysts to be present, as enlarged kidney masses, and when other considerations exist, such as persistent pain, infection, hematuria and impending renal failure, the criteria suggested above for surgery appear to be present. It is only with the accumulation of further clinical data that one might become justified in urging that surgery be used in a prophylactic sense on earlier, apparently asymptomatic cases.
947 W. Eighth St., Los Angeles, Calif.
Conservative surgical treatment for polycystic kidney disease is not new. In 1910 Rovsing described a procedure wherein the kidney is exposed and the cysts punctured and aspirated. Universal acceptance was not forthcoming and the procedure actually fell into disrepute because the end results did not justify the risk. Few differ regarding the need of surgery for the complications of polycystic kidney disease common to other kidney conditions such as stone, tumor and perinephric abscess. As to surgery for the disease per se there is a divergence of viewpoint with the weight of opinion favoring a "do-nothing" attitude. If one is willing to accept the conclusion that conservative surgery has no place in hydronephrosis, and that the surgical treatment of prostatic enlargement should be postponed until urination is greatly hindered or renal function is decidedly impaired, then an attitude of procrastination and expectancy in the treatment of polycystic kidney disease is compatible. All three have in common a gradual impairment of renal function consequent to destruction of renal parenchyma. The argument of doing nothing unless certain complications dictate otherwise is based on an apparently sound observation that often those afflicted ·with this disease live on for many years. This is shallow reasoning and as cogent as allowing hydronephrosis to progress to utter destruction of the kidney because the patient is symptomless. Prior lack of demonstrated success for surgery may have been an adequate excuse for avoiding surgery. If some new procedure gives promise of a brighter horizon, it should be used until its ultimate results either prove or disprove its value. With this in mind there are those who have been daring and are yet sufficiently conservative, to attempt something which might make for more comfort, or prolong life, or both. In 1935, A. E. Goldstein described his operation of marsupialization. In this, the kidney is exposed, decapsulated if possible, accessible cysts excised or otherwise destroyed, deeper cysts aspirated, the kidney partially bivalved, and the wound closed by suturing the wound edges to the edges of the split kidney. The end result was a wide scar through which enlarging cysts could be aspirated. Recently (April 1946) W. W. Young described a method of sclerosing these cysts with 5 per cent sodium morrhuate solution. He applied this method with satisfactory results in a 17 year old woman. The left kidney was exposed, cysts were aspirated and all large cysts were then injected with 5 per cent sodium morrhuate, using approximately 1 cc for each 10 cc of fluid withdrawn. The incision was then closed in the usual manner. Under observation for 4 years she not only was symptom free but had passed through a successful pregnancy. 1 Read at annual meeting, Western Section, American Urological Association, Yosemite Valley, Calif., May 21-23, 1947. 471
472
MORTON M. MAYERS
Young admits that this one case does not constitute real proof of the efficacy of sclerosing treatment, but he believes it is encouragement for further effort in this direction. The advantage of Goldstein's operation is that recurring enlargement of cysts can be aspirated with maximurn safety. The advantage of sclerosing cysts for permanent eradication as in Young's procedure is apparent. The disadvantage of his operation is that recurring cystic enlargements cannot be treated except by re-exposing the kidney. It occurred to us that a modified combination of both the Goldstein and Young procedures would be more ideal. We eliminate the incision into the kidney, treat larger cysts with sclerosing solution at the time of renal exposure, and marsupialize the kidney so that subsequent aspirations and injections could be done with safety. Our experience in treating over 200 hydroceles and spermatoceles with sclerosing agents had proved to us that quinine dihydrochloride 13.33 per cent (or quinine hydrochloride 13.33 per cent and urethane 6.66 per cent) causes less reaction than sodium morrhuate. As further support for the procedure of sclerosing renal cysts, Young cites Fish who reported 2 cases of solitary cyst cured by aspiration and instillation of 50 per cent dextrose solution. In Case 4 following, a combination of marsupialization and sclerosis was employed for the first time. CASE REPORTS
· Case 1.
Mrs. H. G. A., aged 54 years, was first seen on September 8, 1934. Entering the hospital after falling unconscious, she complained of severe neck pain. Her blood pressure was 188/98, the blood nonprotein nitrogen content was 117 mg. per cent, and the creatinin nitrogen 3.2 mg. per cent. The hemoglobin was 44 per cent, and the urine showed 5 pus cells and 2 red blood cells per high power field. On September 11, the nonprotein nitrogen content of the blood was 145 mg. per cent. The right kidney was markedly enlarged. As fatality appeared imminent the Rovsing operation was performed September 14. The patient expired on September 15. Case 2. Mrs. E. N., aged 38 years, entered the hospital March 21, 1936. She gave a history of severe pain over the left lumbar region with fever of 2 weeks duration. A similar episode occurred 9 years previously followed by poor health ever since. The pain was so severe that she was hospitalized as a probable "surgical belly". The nonprotein nitrogen content of the blood was 32 mg. per cent and the hemoglobin 72 per cent. The urine contained numerous pus cells. A cystoscopic study revealed polycystic kidney disease with B. coli infection. The patient improved under medical management and was discharged on April 5, 1936. She was readmitted on February 23, 1937 with a history of invalidism since her previous discharge. Her hemoglobin was 62 per cent and the nonprotein nitrogen 54 mg. per cent. On March 24, 1937, the Goldstein operation was performed on the left side. The wound healed uneventfully except for a mild pyocyaneus infection.
In 1929 a left nephrectomy had been performed.
POLYCYSTIC KIDNEY DISEASE
473
The patient was readmitted to the hospital on July 17, 1937, because of dull pain in the right upper quadrant with intermittent sharp pain, nausea with vomiting for 1 month, chills and fever for 1 week, and dysuria for several months. Examination revealed a tender, enlarged, right kidney. The left kidney was slightly enlarged, but free from symptoms. The hemoglobin was 62 per cent, the white blood cells numbered 22,800, and the nonprotein nitrogen was 53 mg. per cent. On July 21, 1937, a surgical procedure was carried out on the right side, similar to that done on the left side in March. Approximately 1 year later, the patient reported at the office because of severe pain in the right kidney region following a fall. Through the scar, no anesthesia being required, about 25 cc of dark, sanguinous fluid was removed with complete relief of pain. The patient remained asymptomatic and able to carry on normal activities, until 4 years later. On February 22, 1941, she had intense pain in the lower left side with nausea and vomiting. The temperature was 101.8°F., the nonprotein nitrogen 120 mg. per cent, and the phenosulphonphthalein excretion, 3.5 per cent in 2 hours. By aspiration through the scars, 15 cc of fluid were removed from the left kidney and a few cubic centimeters from the right. Despite supportive treatment the blood nonprotein nitrogen content rose to 148 mg. per cent and the patient expired on March 23, 1941. Comment: The result of the application of the Goldstein procedure in this case must be considered excellent. For several years before surgery, the patient had been an invalid unable to perform ordinary housework. Following operation on the left side, no symptoms developed on that side until 4 years later, just preceding death from renal failure. Aspiration was found to be necessary on one occasion with dramatic relief of pain and with that one exception, symptoms on the right cleared up following operation on the right kidney. Case 3. Mrs. M. C., aged 44 years, was first seen at the hospital on February 16, 1941. There had been gradually increasing weakness for several years. One week preceding admission, leg edema developed. Pain on the left side and headache were also present. The blood pressure was 190/110. Two large abdominal masses were felt, and pitting edema of the extremities was present. The hemoglobin was 44 per cent and the urine contained 3 to 6 pus cells per high power field. No phenosulphonphthalein appeared in the urine in 20 minutes. After a transfusion of 2000 cc of whole blood, the hemoglobin rose to 72 per cent. On February 27, the nonprotein nitrogen was 87 mg. per cent. On March 8 the urea clearance was 5 per cent, and the phenolsulphonphthalein test showed a trace of dye in 2 hours. Op. March 14, 1941 the Goldstein procedure, modified by eliminating the incision into the kidney, was carried out on .the right side resulting in reduction in size of the kidney to less than one-half. On the fourth postoperative day the blood nonprotein nitrogen was 95 mg. per cent, on the fifth day, 104, on the eleventh day, 120, and on the twenty-fourth day, 115 mg. per cent. Despite these rising values there was clinical improvement, the wound granulated nicely and the patient became ambulatory. The nonprotein nitrogen was 102 mg. per cent on the day of discharge from the hospital, April
474
MORTON M. MAYERS
16, 1941. Two weeks later the patient reported complaining of pain in the right side. Examination revealed a bulging mass in the right renal area which was aspirated, 50 cc of fluid being removed. Unfortunately a large vessel was punctured due to overzealous needling close to the pedicle, and resulted in severe hemorrhage into the bladder necessitating surgery. The patient expired May 10 in spite of transfusions and supportive treatment. Comment: Surgery was elected for this evident terminal case in the hope that death could be forestalled. It was gratifying to see definite symptomatic improvement following the Goldstein procedure. Case 4. Miss L. P., aged 49 years, was admitted to the hospital on April 15, 1946 with a temperature of 102°. One week previously she had chills and fever, dysuria, frequency, and abdominal pains, chiefly the right side. In 1936 a diagnosis of polycystic kidney disease had been made, but no treatment was given. Tue patient's mother had polycystic kidney disease and the maternal grandmother had died as a result of kidney disease. The blood pressure was 120/80. Both kidneys were enlarged but not tender. Treatment with penicillin, sulfathiazole and sulfadiazine was instituted. Excretory pyelograms revealed bilateral polycystic kidneys. The temperature remained high for 3 weeks and then dropped to 99°. On April 15, the specific gravity of the urine was 1.016, there was a trace of albumin, and numerous red blood cells were present. On April 16, the hemoglobin was 77 per cent, the red blood cells 4,480,000 and the white blood cells 12,800 of which 82 per cent were polymorphonuclear cells. The nonprotein nitrogen was 37 mg. per cent. The patient was discharged May 12, 1946. On readmission to the hospital on June 3, 1946, an examination revealed that the left kidney now filled the left half of the abdomen. The phenolsulphonphthalein test showed an appearance time of 6½ minutes on the right and in 11 minutes on the left, the total in 2-hour excretion being 61 per cent. Pyelography revealed essentially the same findings as before but the left renal shadow was much larger. On June 12, the total blood protein value was 7.1 per cent and the hemoglobin 84 per cent. On June 18, 1946 at operation the left kidney was found tremendously enlarged, filling the left half of the abdomen and containing innumerable cysts, pin-head to an orange in size, filled with clear fluid or pus. The cysts were aspirated, 500 cc of fluid being obtained, including 300 cc of pus. After aspirating the larger cysts, 13.3 per cent of quinine dihydrochloride solution was injected, using approximately 1 cc for each 10 cc removed. The actual cautery was applied to the surface cysts. Through and through mattress sutures of nylon penetrating kidney, Gerota's capsule and the body wall were inserted and moist dressings applied. On July 16, the hemoglobin was 62 per cent, the nonprotein nitrogen 39 per cent, the CO2 combining power of the blood was 54, the blood chloride content 460 mg. per cent and the total protein content, 5 gm. On that day, 200 cc of fluid (including 50 cc of purulent material) were aspirated through the wound. Improvement followed although the temperature remained at 101 ° to 102°F. Penicillin and blood transfusion were given. Ure-
POLYCYSTIC KIDNEY DISEASE
475
teral obstruction was suspected but cystoscopic examination on July 18 revealed none. Aspiration of the cysts was carried out several times. On July 24, 450 cc of purulent fluid was removed from a single cyst in the lower pole, and 8 cc of quinine dihydrochloride solution, 13.33 per cent, was injected. On July 25, the hemoglobin was 69 per cent and on August 7, 66 per cent. The urine contained albumin and numerous pus cells but no red blood cells. On September 28 the patient was discharged from the hospital after a stormy course. The kidney was markedly reduced in size. Improvement has since been progressive. The left kidney is now not much larger than a normal kidney. On January 2, 1947, the right pyelogram revealed little change, the left suggested only vaguely a polycystic kidney. Phenolsulphonphthalein appeared in 3 minutes from the left kidney and ~n 4 minutes from the right and the excretion in the following 20 minutes was 16 per cent for the right, and 7 per cent for the left. Urine from the right kidney was sterile; that from the left contained numerous pus cells and B. coli on culture. Two months later all urine specimens became sterile and have remained sterile to date. The patient is now in a satisfactory physical condition still retaining a kidney which may serve her well as the function decreases on the opposite side. (As of January 1948, she is engaged in her profession of school teaching.) SUMMARY
Conservative surgery for polycystic kidney disease was given its greatest impetus by Rovsing in 1910, by his cyst-puncturing operation. Surgery for the usual complications receives acquiescence but for treatment of the disease per se, the ·weight of opinion has favored a "watchful-waiting" attitude. Although the incidence of this disease in over 1,300,000 clinical admissions was approximately 1 in 4000, it was 1 in 400 in 87,000 autopsies. Such a disparity can only be explained by an acknowledgment that the disease is responsible for far more morbidity and mortality than is generally recognized. In the adult form, the average age at onset of symptoms and the average age at death is 38 and 50 respectively, further emphasizing its severity and possibly reflecting the attitude of no surgical interference until irreversible damage is done. The daring and yet sufficiently conservative procedure of Albert Goldstein in 1936 in "marsupializing" the kidney so that cysts could be aspirated postoperatively, folluwed by the recent work of W. W. Young in using 5 per cent sodium morrhuate for sclerosing the cysts, suggested a procedure combining the advantage of both these operations. The author used both marsupialization and sclerosing therapy so that aspiration of cysts and the injection of a sclerosing substance could be continued postoperatively. Quinine hydrochloride and dihydrochloride are preferred to sodium morrhuate as being less reactive. Two cases are presented in which a modified Goldstein procedure had been performed with results classified as good in one and poor in the other. A case in which the author employed a modified Goldstein-Young technique with apparently excellent results is described in detail.
476
MORTON M. MAYERS CONCLUSION
It is not to be construed that the author advocates routine surgical intervention for polycystic kidney disease. Being cognizant of the fact that innumerable small cysts comprise the bulk of the pathology, and as such cannot be eradicated by any method heretofore suggested, careful selectivity of cases is advised. When one suspects large cysts to be present, as enlarged kidney masses, and when other considerations exist, such as persistent pain, infection, hematuria and impending renal failure, the criteria suggested above for surgery appear to be present. It is only with the accumulation of further clinical data that one might become justified in urging that surgery be used in a prophylactic sense on earlier, apparently asymptomatic cases.
947 W. Eighth St., Los Angeles, Calif.