Clinical management of the horseshoe kidney: Part III (Conclusion)

CLINICAL MANAGEMENT OF THE HORSESHOE KIDNEY

PART III (CONCLUSION) *

ROBERT GUTIERREZ, M.D., F.A.C.S.

NEW YORK

x. REPORT OF CASES

Summary of rg Cases of Horseshoe Kidney Disease with

Preoperative Diagnosis

CASE I. D. S., aged eighteen, maIe, hospita1 No. 282,425 admitted Oct. 15, rgz8. Rbsumi: of Symptoms: Patient is a thin, we11 deveIoped young man but very anemic,

lying in bed and acuteIy iII, compIaining chiefIy of pain in epigastrium and umbiIica1 region, marked frequency of urination and nocturia, dysuria, pyuria, nausea, vomiting, headache and high temperature. Duration three weeks. He had been treated for persistent chiIIs and fever of undetermined origin, until he came under my observation. On physica examination the abdomen is much contracted and rigid, both kidney regions tender and painfu1 on paIpation. Patient stated that attacks of intermittent pain have occurred for several years and chronic constipation has been present from birth. He cIaims that he has been using enemas daily, from childhood, and his bowe1 troubIes have been accompanied by persistent pain in the umbiIica1 region and across his back which has made him unfit to work or to attend to his duties at schoo1.

Cystoscopic Findings and Functional Renal Tests: BIadder mucosa chronicaIIy inflamed, muItipIe ceIIuIes and trabecuIations. Both ureteric orifices markedIy congested and interureteric ridge of trigone markedIy hypertrophied. Both ureters catheterized. PhenoIsuIphonephthaIein appeared on right side in two and one-haIf minutes, on Ieft in ten minutes. Urine from bIadder foul and puruIent. Urine culture negative.

Roentgenograpby: No shadow indicative of stone in urinary tract. The shadow of the right kidney is irregular and Iow in position, about the IeveI of the fourth Iumbar vertebra, and there is an indefinite shadow, suggesting the isthmus of a horseshoe kidney.

Urograpbic Findings: Right pyeIogram, moderate degree of hydronephrosis; peIvis is turned and the caIices point inward toward the spina coIumn. Left pyeIogram, slight hydronephrosis with rotation of peIvis and caIices, which point inward.

Diagnosis and Type of Lesion: Horseshoe kidney with biIatera1 hydronephrosis and pyeIonephrosis infection pIus pyelitis and pyeIonephritis.

*Part I appeared in the December, rg3r issue, vol. 14, p. 657, and Part II in the January, 1932, issue, voI. 15, p. 132.

345

FIG. *,r . Drawing made from Case I to iIIustrate the value of the medica and urologica treatment in acute cases of horseshoe kidney disease with evidence of urinary stasis, pyelitis and pyeIone catheter to secure B

hritis, particuIarIy when using the method of the indwelIing uretera rainage, to reIieve pain and infection and to prevent fatal uremia.

Note aIso the presence of the minimum basal angle of tbe pyelograpbic triangle of the borsesboe kidney.

(I34611

FIG. 42. Represents the first step in the operation of renaI symphysiotomy for division of the parenchyma1 isthmus in horseshoe kidney disease.

FIG. 43. FIG. 44.

FIG. 43. IIIustrates the second step of the operation of renal symphysiotomy in normaI horseshoe kidney as the onIy possibIe means of obtaining permanent reIief from symptoms and uItimate cure.

FIG. 44. Re operation, whrc

resents the tina step in the techni .K consists of separation of the two 1.

ue of the renaI symphysiotomy Idneys from their incarcerated

position upon the aorta and vena cava, followed by suture of the raw surfaces of the cut renaI isthmus. This operation appears to be the idea1 method of treating the entity of horseshoe kidney disease. (Redrawn from Rovsing’s case.)

u347n

348 CLINICAL MANAGEMENT O’F HORSESHOE KIDNEY

Pathological Observations: At times uremic with temperature up to 104’F. P&e very irreguIar and rapid. DifficuIty in breathing and in precordia1 region. Persistent pain in epigastrium, rigidity of abdomina1 muscIes and vomiting. Genera1 weakness, poor circulation of extremities with edema and cyanosis of both Iegs.

Treatment: Medical: Urinary antiseptics, urotropin and sodium acid phosphate, t.i.d. Intravenous infusion of saIine soIution, cathartics and high coIonic irrigations, forced Auid, rest in bed, heat to kidneys and extremities, etc. Urological: IndweIIing ureteral catheters to secure drainage and reIieve pressure; kidney peIvis irrigations with acri- fl avine I : 10,000 to correct infection and prevent inhibition of kidney function. Surgical: None.

Results and Observations: Left hospita1 in two weeks, apparentIy cured of acute symptoms. Two months Iater still compIained of pain in epigastrium and in both kidney regions. Urine hazy and never free from pus. Patient is thin, abdomen much contracted; stiI1 compIains of chronic constipation for which he uses enemas daiIy and cathartics. Cystoscopic treatments, with dilatation of ureters and kidney pelvis lavage, have been carried out on various occasions and reIieved the symptoms somewhat, but patient states that while he is feeling much better he is not entireIy weII, and that the cause of his troubIe has never been expIained to him before. Symphysiotomy operation proposed but in view of his improvement the patient went South and no further data have been obtained.

CASE II. B. C., aged twenty-nine, maIe, hospital No. 284,810, date of admission Feb. 23, 1929.

R&urn6 of Symptoms: Burning on urination, pain on Ieft side, duration five days. Hematuria and pyuria. Occasional rise of temperature and chiIIs.

Cystoscopic Findings and Functional Renal Tests: Interior of bIadder negative. Both ureters catheterized without obstruction. PhenoIsuIphonephthaIein on left side in nine minutes, on right no appearance. Urine cuIture negative.

Roentgenograpby: No shadow indicative of stone in urinary tract. Shadow of Ieft kidney much Iarger and Iower than that of right.

Urogrupbic Findings: Right pyeIogram shows a typica arrangement of the calices of a horseshoe kidney. Left pyeIogram shows compIete excavation of the Ieft kidney, with infectious hydronephrosis.

Diagnosis and Type of Lesion: Horseshoe kidney with biIatera1 pyelonephritic infection and left hydronephrosis.

Pathological Observations: High temperature, severe pain in epigastrium, at times uremic symptoms.

Treatment: Medical: Urinary antiseptics. Urological: IndweIling uretera catheters with kidney peIvis irrigations. Surgical: None.

Results and Observations: Improved, and discharged March 30, 1929.

CASE III. M. D., aged twenty-one, maIe, hospita1 No. 175,268, date of admission

May 5. 1930. R&sum6 of Symptoms: Pain in lower right quadrant, aIso sIight pain in umbiIica1

region. SIight frequency and dysuria. Patient entered hospita1 with a provisional diagnosis of appendicitis.

Cystoscopic Findings and Functional Renal Tests: BIadder chronicaIIy inffamed throughout. Both ureters catheterized but catheter met obstruction on right side about z cm. from ureteral orifice. PhenoIsuIphonephthaIein appeared on right side in seven minutes, on Ieft side in three minutes. CuIture of urine showed negative growth.

REPORT OF CASES

Roentgenograpby: Left kidney shadow is Iower and huger than that on the right and the actua1 lower poIes of both kidneys are not seen. There is a smaII shadow in contact with the catheter just opposite the third sacral foramen on the right side, revealing the presence of a smaI1 stone impacted in the tower ureter.

Urogrupbic Findings: BiIateraI pyelograms taken on April 28, 1930, reveaI horseshoe kidney. Also later, on May 13, 1930, uroselectan pictures, twenty minutes after intravenous injection, showed a beautifu1 shadow of the entire urinary tract and the cahces of both pelves pointing towards the medran Iine. A fiIling defect of a round caIcuIus was seen in the Iower end of the right ureter.

Diagnosis and Type oJ Lesion: Horseshoe kidney, biIatera1 pyeIitis, caIcuIus in right lower ureter.

Pathological Observations: At times severe attacks of pain in right Iower quadrant and umbilica1 region with rise of temperature.

Treatment: Medical: Urinary antiseptics, rest in bed, cathartics and forced fluid. Urological: Cystoscopy, diIatation of ureters, Iavage of kidney peIvis with revonoI

dextrose I : 5000. IndwelIing uretera catheter at times. Surgical: None. Results and Observations: Patient Ieft hospita1 after four weeks with the diagnosis

just given and feeIing better, but in stiI1 receiving cystoscopic treatment. His genera1 condition is improved.

CASE IV. J. T., aged fifty, maIe, examined Dec. 20, rgz6; presented here by courtesy of Dr. Hernandez of Havana. Chief complaint, pain in epigastrium for over twenty years, gastric trouble for severa years. Pain in both renaI regions with dysuria and intermittent attacks of hematuria.

PhysicaI examination reveaIed that both kidneys were painfui on palpation of abdomen, and a definite tumor mass was easiIy made out on the Ieft side which apparently ran across to the opposite side where another definite hard tumor mass was aIso paIpabIe. Urine anaIysis showed microscopic pyuria and hematuria and a trace of aIbumin and casts. January 2, 1927, a pIain x-ray reveaIed the presence of a gigantic bilatera1 renaI caIcuIus occupying the entire site of both kidney peIves. On the right side there were two unusuaIIy pIaced shadows about the size of a Iime, quite cIose to the spina coIumn at the Ieve of the third and fourth lumbar vertebrae and near the midIine. On the Ieft side the shadow of the coraliform stone which was about the size of an orange, occupied the entire area of the Ieft kidney peIvis and appeared to be rotated inward with the cahces or branches of the stone pointing to the spinal column. A dehnite diagnosis of horseshoe kidney with biIatera1 nephrobthiasis couId be made from the pIain x-ray pictures.

Patient was cystoscoped January 13, 1927, and a right pyeIogram taken, confirming the diagnosis of horseshoe kidney with biIatera1 renaI stone and marked diminution of renal function. BIood chemistry Figures at that time were pretty high, and in view of patient’s general unsatisfactory condition palliative treatment aIone was recommended.

CASE v. W. L., aged hfty, male, hospital No. 269,959; a we11 deveIoped individua1 with bIood pressure 12o/gz, admitted to the UroIogical Department of New York HospitaI Nov. IO, 1926. Patient came to the clinic compIaining of pain in both kidney regions and in bIadder of over thirty years’ duration, but more severe for the last two months during which it has been accompanied by frequency of urination, dysuria and changing ffow of urine. He also had an attack of acute retention for which he entered the hospital

For the past ten years he has been having a great deal of troubIe physicaIIy and has had medica treatment for neurasthenia and general bodiIy weakness, aIso prostatic

350 CLINICAL MANAGEMENT OF HORSESHOE KIDNEY

massage and bIadder irrigations. Fifteen years ago he had a perinea1 operation for drainage of seminat vesicIes and three months ago an abdomina1 operation in another hospita1 at which horseshoe kidney was discovered but no reIief of abdomina1 pain obtained.

Physical examination reveaIed a Iarge scar at the Ieft side of the abdomen with sIight tenderness on deep pressure. There was suprapubic duIness extending to the umbiIicus. Rectal examination disctosed an enIarged prostate, Ieading to a diagnosis of hypertrophy of prostate with compIicating phIebitis and chronic cystitis, for which a suprapubic cystotomy was done Nov. 16, 1926.

Patient was discharged from hospita1 January 19, 1927, with a suprapubic drainage tube and with his genera1 condition improved; readmitted to hospita1 March 4, 1927,

compIaining of marked pain in the abdomen and bIadder region, with sIight fever; he was carrying a suprapubic Pezzer catheter which was not working satisfactoriIy. This was replaced by a doubIe suction tube, and a Young punch operation under regional anesthesia was planned to be carried out as soon as the genera1 condition shouId warrant it. The patient was discharged and advised to return to the hospita1 after a period of convaIescence when perineal prostatectomy was aIso to be considered.

Nov. IO, 1926, cystoscopy reveaIed a normal bIadder mucosa except for marked congestion of the trigone, considerable intrusion of the subcervica1 group and moderate infringement of the IateraI part of the prostate. Both ureters were catheterized and the renal function was found about equa1 on both sides. The roentgenogram reveaIed the shadow of the Ieft kidney larger than usua1, giving the impression of polycystic kidney disease. In the Ieft pyeIogram, the kidney was unusua1 in shape and turned inward, giving a definite impression of an infected horseshoe kidney. The second admission was on March 4, 1927, and the discharge on March II, rgq, with the result unimproved. No further surgica1 treatment was carried out and two weeks Iater patient died of pneumonia. No autopsy was secured.

CASE VI. B. L., housewife, aged forty-three, hospital No. 269,385, Russian, admitted to New York HospitaI Oct. 6, 1926, with diagnosis of chronic constipation and pain in Iower abdomen for over two years. An operation in some other hospital for chronic appendicitis had brought no reIief. Patient is married and has four children, Iiving and weI1. She has been suffering with occasiona attacks of duI1, recurring pain in right Iower quadrant of abdomen, for a period of nearly tweIve years, which at times radiated across abdomen and around the back on both sides. For the Iast two years since the appendectomy operation, pain has been constantIy present in varying degrees, at times fairly severe. It has been sIightIy more marked in the morning foIIowing a night’s rest, and has sometimes been reIieved by enemas and morphine. During the past few months the pain has graduaIIy become worse.

For past year and a half patient has been compeIIed to take daiIy enemas. She stated she had been constipated a11 her Iife but never to such a marked degree as now.

For the last six months there has been sIight gastric troubIe with marked gaseous eructations and meteorism, but no vomiting or pain after meaIs.

The menstrua1 history has always been reguIar except that in the past year patient has had “severe pain” throughout duration of menstrual periods.

Urine anaIysis has always been negative. But during past six months patient has had a very noticeabIe polyuria, with frequency during day of ten to fifteen times, and during night of two to three times. No pyuria or hematuria has been present at any time. PhysicaI examination was essentiaIIy uninteresting. AI1 sorts of examination in genera1 hospitat had been undertaken in order to find a possible cause of her symptoms but she was discharged Oct. 16, 1926, with the diagnosis of chronic constipation and

REPORT OF CASES 351

indefinite coIon pathoIogy, and with the resuIts of improvement. Patient was readmitted to the hospitaI Nov. 14, 1927, with the same compIaint. But the undetermined abdominal pain, particuIarIy in the umbiIica1 region, has been more severe, and she has aIso deveI- oped rectal tenesmus, for which proctoscopic examination was carried out but proved negative. X-ray with barium enema and barium meaIs did not discIose a true pathoIogy in the coIon or gastrointestina1 tract. As the patient had pollakiuria and dysuria and pus ceIIs in the urine, she was referred to the uroIogica1 department for cystoscopic examination. Cystoscopy Oct. 14, 1926, reveaIed a norma bIadder except for an unusual mucosa that was foIded over itseIf with marked trabeculations and ceIIuIes. Both ureters were catheterized and renal function found to be normal. The Ieft pyeIogram, however, reveaIed a rotated kidney peIvis with shaggy and diJated cafices, giving the impression of horseshoe kidney as the definite cause of her troubIe. A diagnosis of horseshoe kidney with pyeIonephritis was accordingIy made and patient was discharged improved Nov. 18, 1927, after a few cystoscopic treatments and kidney peIvis Iavage.

CUE VII. M. S., aged thirty-two, maIe, examined by Dr. Hernandez of Havana May 25, 1926. Chief compIaint renaI coIic and gastric trouble on different occasions; also poIIakiuria, dysuria and pain in both kidney regions and in epigastrium, on deep palpation.

PhysicaI examination and urinaIysis negative. Roentgenography disclosed the presence of shadows of a urinary caIcuIus in the area of the peIvis and caIices of the right kidney. Cytioscopy May 30, 1926, revealed a diffused cystitis and a good renal function. Right pyelogram showed that the opaque medium obscured tKe origina shadows of stone and that the peIvis was rotated with calices reversed and pointing toward the spinal coIumn, thus reveaIing the diagnosis of horseshoe kidney with stone in the right side. The right kidney was exposed and heminephrectomy done. Patient had an uneventful ConvaIescence.

CASE VIII. A. M., aged twenty-six, female, has been compIaining for severa years of chronic appendicitis with indefinite pain on the right Iower quadrant. AIso has pain across her back.

Cystoscopy on June 7, 1929, reveaIed two uretera orifices on the Ieft side and one opening normaIIy on the right side of the trigone. The three ureters were catheterized and specimens coIIected and sent to the Iaboratory for culture, and for urea and micro- scopica1 examination. PhenoIsuIphonephthaIein I C.C. injected intravenousIy appeared from the three ureters in three to four minutes with norma concentration. The culture of urine was negative. Roentgenography reveaIed no shadow indicative of stone in the urinary tract. The right pyelogram showed infection of two parts of the double pe!vis with a very peculiar arrangement of both the peIves and the caIices, giving the impression that the two pelves reached the common ureter and that the whoIe organ was rotated inward as in horseshoe kidney. A week Iater a pyeIogram of the two ureters and two corresponding kidney peIves on the Ieft side reveaIed aIso the fact that these were rotated toward the spina coIumn and thus confirmed the presence of a congenita1 ano- maIy of four pelves and four incompIete ureters in a horseshoe kidney. Since this diagnosis has been estabIished, the treatment carried out has been the routine cystoscopy, diIata- tion of the ureters and kidney peIvis Iavage, which brings reIief and very satisfactory resuIts in cIearing up the pyeIonephritic infection. The microscopica examination of sediment has shown that the urine coIIected from each kidney peIvis contains occasional pus ceIIs, epitheIia1 ceIIs and many red bIood ceIIs. The cuIture of the urine from the bIadder and from each kidney peIvis has at a11 times been negative. ApparentIy patient is improving under the routine uroIogica1 treatments.


CASE IX. M. K., aged fifty-eight, female, had aIways been in good heaIth, but complained of sIight backache for the last ten years. In the last few months she has noticed frequency and painfuI urination, for which cystoscopy was performed, reveaIing a normaI bIadder with ureteral orifice sIightIy congested, as were aIso the trigone and vesical orifice. The ureters were catheterized with a No. 6 French catheter. The renaI function was good from both sides. BiIateraI pyeIogram on March 14, 1929, reveaIed a pecuIiar shape of pelvis with marked rotation and the caIices opening toward the midIine. The ureters were somewhat distorted and diIated but apparentIy feII within norma Iimits. They reached the peIvis in a pecuIiar and abnorma1 angIe, their direction aIongside of the spina coIumn making a wide-mouthed “flower vase” figure as if approaching each other above and carrying the contents of a horseshoe kidney. After the diagnosis was estabIished, patient was submitted to cystoscopic treatment with diIata- tion of the ureters and kidney peIvis injection with a soIution of revono1 dextrose I : 2000.

In addition, because the position of her kidney shadow was Iower than normaI, she was given a kidney beIt to support the organ. WhiIe her genera1 condition has improved, her symptoms have not entireIy disappeared. It seems, therefore, that no definite cure or reIief of symptoms can be obtained unti1 the isthmus of the horseshoe kidney is divided by the symphysiotomy operation, separating the fused organ into two separate organs, and thus reIieving the continuous pressure that the isthmus of the horseshoe kidney exerts upon the aorta and vena cava and upon the nerve suppIy of the same.

CASE x. F. L., maIe, ItaIian, aged thirty-two, hospita1 No. 274,340, was admitted into the UroIogical Department of the New York HospitaI JuIy 21, 1927, compIaining of sweIIing of Ieft testicIe of three and one-half years’ duration, aIso dysuria and frequency. Patient states that in the past he has had attacks of renaI coIic, frequency, hematuria and intermittent pain in right Iumbar region. He further states that an operation was done in ItaIy fourteen years ago for the remova of stone from the right kidney, since which time he has been suffering with abdomina1 pain. The diagnosis made was Ieft chronic epididymitis and Ieft seminal vesicuIitis, and on JuIy 22, 1927, under IocaI anesthesia Ieft epididymectomy was done for a tubercuIous epididymis. The vas deferens was transpIanted to the surface of the skin about I in. beIow Poupart’s ligament and above the external inguina1 ring. Patient made an uneventfu1 recovery and was discharged from the hospita1 on JuIy 30, 1927. The laboratory report of the specimen proved that the epididymitis was of a tubercuIous nature. Patient was readmitted to the uroIog- ica1 service on Sept. g, 1929, with sweIIing of right side of scrotum of over four months’ duration. A right hydroceIe with a chronic right tubercuIous epididymitis was diagnosed, for which he was operated upon on Sept. IO under IocaI anesthesia. Right hydroceIectomy and epididymectomy were carried out in the usua1 manner. Again patient had an uneventfu1 recovery and was discharged Sept. 19, 1929. Two weeks Iater he came back compIaining of pain on the upper right quadrant where he had the scar of a previous kidney operation. Cystoscopy was done, and a plain roentgenogram and a right pyeIo- gram taken, revealing that the right and Ieft kidney shadows were indefinite but appeared to be Iow in position, giving the impression of a union between the two kidneys in the midIine. The pyeIogram thus indicated the presence of a horseshoe kidney with an excavation of the right peIvis and the presence of a pyohydronephrosis presumably of tubercuIous origin and representing the primary focus of tubercuIous infection of Iong standing. The calices were shaggy, distorted and dilated, the Iowermost ones pointing inward toward the midIine, thus confirming a definite diagnosis of horseshoe kidney. No further operation was considered in this case and the patient has been receiving the

REPORT OF CASES 353

usuaI anti-tubercuIous treatment in the postoperative tubercuIosis cIinic of the hospital with the diagnosis of renaI tuberculosis on the right side of a horseshoe kidney.

CASE XI. G. P., history No. 273,151, merchant, thirty-five years of age, admitted May 16, 1927, compIaining of pain in Ieft Iumbar region of over four years’ duration; had been operated on eIsewhere for appendicitis, but indefinite pain in the umbiIica1 region persisted after appendectomy. Had experienced a severe attack of pain on Ieft side with marked urinary disturbances six weeks previous to admission to hospita1. Cystoscopy and Ieft pyelography were done and pIain x-ray pictures of the genitourinary tract aIso taken. The Iatter showed a round shadow opposite the transverse process of the fourth Iumbar vertebra on the Ieft side, discIosing further the presence of a stone at the IeveI of the Ieft ureteropeIvic junction. The kidney shadows on both sides were particuIarIy interesting in that they were apparently connected by an isthmus at the Iower pole, forming a typica horseshoe kidney. The Ieft pyeIogram showed a bifid pelvis, with the calices of the upper portion of the kidney diIated and shaggy, pointing backward and Iaterally, whiIe those of the Iower portion pointed markedly inward toward the spinal coIumn, thereby reveaIing the presence of a horseshoe kidney with infection and stone in the ureteropeIvic junction on the Ieft side. The patient was advised to have diIatation of ureters and kidney pelvis Iavage, in an attempt to aIIow the stone to pass and to overcome the infection. The renal function on each side was sufficient in regard to urea excretion and phenoIsuIphonephthaIein elimination. Microscopical examination of sediment showed pus cells 20 per field on the Ieft, and blood and epitheIia1 ceIIs 3 per fIeId on the right. After severa cystoscopic treatments, the stone moved down into the intraumura1 vesica1 portion of the ureter and was removed from the Ieft uretera orifice with the Lowsley irrigating rongeur. Patient was discharged from the hospital two days later as improved but with the diagnosis of horseshoe kidney.

CASE XII. J. de R., male, Italian, aged twenty-two, History No. 262,783. Student by occupation, we11 nourished and we11 deveIoped but appearing acuteIy III on August 27, 1925, when he was admitted to hospita1, his chief compIaint being pain in Ieft kidney region and severe pain in rectum.

PhysicaI examination reveaIed a very rigid and contracted abdomen. BIood pressure systoIic 210, diastoIic I IO. Patient was brought to hospita1 on the theory that the severe pain in left kidney region was due to a uretera obstruction from an impacted stone in the peIvic ureter. BIood chemistry showed urea retention up to 167.90, thus reveaIing the critica condition of the patient, who was uremic and unconscious. TweIve years ago a stone had been removed from the bIadder by cystoscopic manipuIations. Eighteen months previous to admission to the uroIogica1 department patient had undergone an operation for appendectomy elsewhere. A reIative stated that at the time of the appendectomy the surgeon had toId him that the patient had an enormous kidney (?). On August 28, 1925, the patient was cystoscoped. A considerabIe amount of cIoudy and fou1 urine was removed from his bIadder, and after a profuse washing the bIadder mucosa

. . appeared very much InJected throughout, containing many particIes of pus on its surface. There was a buIging at the vertex of the bIadder, apparently due to some external pressure. No stones, tumor masses, diverticuIa or uIcerations were seen in the urinary bIadder. Both ureters were catheterized and a left pyeIogram was taken, about 13 C.C.

of sodium iodide being injected into the Ieft kidney pelvis. After the picture was taken the catheter was reinserted, the sodium iodide aIIowed to drain off, the kidney peIvis irrigated with steriIe water, and the instruments and catheters removed. Patient was sent to ward in fair condition. The pyeIogram reveaIed an unusua1 shape of peIvis which at first was thought to indicate a polycystic kidney, but in view of the fact that the


peIvis was rotated and that the calices were inverted, pointing toward the vertebra1 coIumn, a diagnosis of horseshoe kidney was made. The patient’s general condition appeared to be better for a day or so, but on Sept. 3, 1925, it became aggravated, with a high temperature and the blood urea rising as high as 261.28. Despite every care and attention patient died on Sept. 6, 1925, from acute uremia due to an infected horseshoe kidney condition.

CASE XIII. A. B., a Russian woman, aged twenty-four, admitted to medica division of the New York HospitaI and Iater on January 17, 1927, transferred to UroIogicaI Department, history No. 271,004. She had been compIaining of severe pain in both kidney regions with intermittent attacks of hematuria and pyuria over a period of severa years, accompanied with burning and difFicuIty of urination, which symptoms have become aggravated in the Iast two weeks. She has aIso been suffering with marked chronic constipation a11 her life. On physical examination the abdomen was very much contracted and there was definite pain on paIpation in the umbiIica1 region and aIso on the right Iower quadrant. Patient was cystoscoped and a right and Ieft pyeIogram taken revealing the presence of a rotated peIvis irreguIar in shape, with the Iower calices pointing inward toward the midIine thus demonstrating the presence of an infected horseshoe kidney. Three days after admission she became suddenIy hysterica and delirious, and deveIoped a menta1 compIex for which she was transferred to BeIIevue HospitaI for further observation and treatment. No further information has been recorded in this case.

CASE XIV. J. R., maIe, aged forty-four, admitted to the cIinic of the Urologica Department of the New York HospitaI on Jan. 14, 1930, history No. 172,381. AIthough appearing in good heaIth, he stated that he had been receiving medica attention for the Iast fourteen years for various conditions for which he had been treated in severa hospi- tals. These conditions according to his statements have been arthritis, rheumatic pain, stomach trouble and aIso uIcer of the stomach. He aIso stated that so far no treatment has reIieved his compIaint and that he came to the cIinic of the New York Hospital mainIy because of di&uIty and frequency of urination and indefinite pain in upper abdomen. A carefuI history and check up in connection with this series of horseshoe kidney cases have reveaIed very striking cIinica1 evidence of this Iongstanding disease.

Chief Complaints: (I) Pain in epigastrium and umbilical region of two years’ duration. (2) Pain on Ieft Ieg with edema of right foot and sensation of coId, due probably to

Iack of circuIation. (3) Marked chronic constipation. Patient has had to have a daiIy enema and Iaxa-

tives every other day for the Iast two years. (4) Gastric troubIe, sensation of fuIness, gastrointestina1 disorders. Patient cIaims

that other doctors have toId him he has uIcer of the stomach, but he has good appetite and has Iost neither bIood nor weight.

(5) DiffIcuIty and frequency of urination, marked dribbIing. Nocturia two to three times.

(6) Attack of right renaI coIic one year ago but never has had hematuria. (7) Persistent pus in urine and sometimes dysuria. Family History: Patient born in Russia, has one sister and 12 brothers, 2 of whom are

twins. Father is eighty-six years oId, living and in good heaIth Mother died twenty-one years ago of pain in the middIe of the abdomen and probabIy same sort of kidney trouble as that of which patient is compIaining.

Personal History: Patient has had the ordinary chiIdhood diseases without incidents. The first clinica manifestations of the present troubIe started twenty-three years ago after he had been in the military service abroad. During a Iong stretch of walking, he was

REPORT OF CASES 355

seized with a sudden attack of pain in the middIe of the abdomen. There was also pain in the right leg with a sensation of coId in the same, which made it impossibIe for him to continue in the army. The second attack of clinical manifestations appeared again fourteen years ago with the same symptoms as before but now more severe. A third simiIar attack of ilIness occurred two years ago with al1 the classic symptoms described here, from which he is stiI1 suffering at the present time.

Physical Examination: Patient is we11 deveIoped and we11 nourished for a man of his age, and weighs about 155 Ib. The abdomen was quite distended and on deep palpation on the right Aank a definite mass couId be made out which was tender to the touch and was presumabIy the kidney, which, apparentIy running across the midIine, presented a resistant body beIow the area of the umbilicus. AIso on the Ieft side on deep paIpation, Iower than normal and in the Ieft IateraI portion of the middIe of the abdomen, there could be detected a resistant mass of unusual shape and tender on paIpation, giving the impression of a possibIe ectopic fused kidney.

Exxternal Genitals: Normal. Rectal examination negative. The urine, first and second gIasses, was cIear and the third was sIightIy hazy.

Cystoscopy and Renal Functional Test: On January I 8 patient was cystoscoped after meatotomy. The interior of bIadder was negative. Both ureters were catheterized and specimens sent to the Iaboratory for cuIture and for urea and microscopica examination. The report of the uretera specimen was as foIIows: Character bloody, urea and phenoIsuI- phonephthaIein eIimination from both sides equaIIy good. The cuIture was negative.

Roentgenograpby and Pyelograpby: The x-ray report of the genitourinary tract was negative to shadows of urinary caIcuIi, but reveaIed a most interesting shadow of a fused organ, as in horseshoe kidney. The right pyeIogram showed a biiid type of kidney pelvis with some shagginess of caIices and slight distortion of the peIvis, but apparentIy the peIvis was in norma position. Two weeks Iater patient was cystoscoped again and a Ieft pyeIogram taken, which reveaIed aIso a slight degree of hydronephrosis, but with peIvis and caIices apparentIy normaIIy pIaced. However, the diagnosis of horseshoe kidney was estabIished and based on the Aat x-ray picture which corresponds with the cIinica1 findings. Patient has been receiving onIy uroIogica1 treatments, routine cystoscopy with dihltation of the ureters and kidney peIvis Iavage. He has improved somewhat but is still compIaining of the abdomina1 pressure and other symptoms for which he came to the hospita1, and gives the impression that not until the isthmus of the horseshoe kidney is divided by a symphysiotomy operation wiI1 he definitely be reIieved of his abdominal pain and urinary symptoms.

CASE xv. L. H. male, aged sixty-seven, was admitted to the Urologica Department of the New York HospitaI May 4, 1929, compIaining of frequency of urination day and night with intermittent hematuria for the Iast four years. AIso occasiona attacks of pain of moderate severity in the Ieft Iumbar region. He stated he had suffered with sIight pain across his back for more than twenty-four years, which he attributed to rheumatism. AI1 these symptoms have been getting worse during the Iast month and he entered the hospita1 for reIief. On physica examination a sIightIy tender mass couId be feIt on the left upper quadrant. UrinaIysis reveaIed pus 3 plus, blood 2 PIUS, specific gravity 1.015, acid reaction and appearance hazy with a slight trace of albumin. Wassermann test negative. On May 3 cystoscopy reveaIed no bIood from either ureteral orifice. A No. 6 catheter passed to each kidney peIvis without difftculty. Specimens from each side and aIso from the bIadder were sent to the Iaboratory for cuIture, and urea and microscopical examination. PhenoIsuIphonephthaIein appeared on the left side in five minutes, on the right in nine minutes. The urea concentration was 25 gm. per liter on the right, 18 gm.

FIG. 45. , PIain roentgenogram of Case xv revealing a shadow of a renaI caIcuIus in area of the Ieft kidney, and in contact with the ureteral catheter.

the

FIG. lower c: kidney.

46, Left pyeIoureterogram of same case, reveaIing rotation of peIvis with dices turning inward toward the midline? and assuring the presence of a horses Also hydronephrosis covering the prevxous shadow of the renal caIcuIus.

the ;hoe

REPORT OF CASES 35:

on the Ieft. The microscopical examination of sediment showed pus ceIIs on the left side. Culture was negative. PIain x-ray pictures and left pyelogram were taken. The roentgeno- graphical report reveaIed the presence of a shadow in the area ofthe Ieft kidnev, apparentIy

FIG. 47. Drawing of the operative technique in Case XV where heminephrectomy was performed with success. I. ObIique Iumbar incision. 2. Exposure of the Ieft haIf of the horseshoe organ and the division of the isthmus. 3. Showing the horseshoe kidney and the incision carried out in the isthmus where the Ieft haIf of the viscuc was removed (De Vries and Lowsley’s case).

a renaI caIcuIus. The Ieft pyelogram showed a very much dilated pelvis with shagginess and evidence of chronic infection, covering the previous shadow of the renaI calcu- Ius. The peIvis was unusua1 in shape, sIightIy rotated with the lower caIices pointed toward the midIine, thus reveaIing a congenital malformation in the shape of a horseshoe kidney. The patient was operated upon under paravertebral novocaine anesthesia on

FIG. 4% PIain roentgenogram (a) and pyeIogram (b) of the s ecimen in Case xv. Left half of horseshoe kidney removed at operation, showing t R e pathological Iesions.

49. Drawing of specimen removed at operation in same case, revealing the anterior and posterior surfaces of the Ieft heminephrectomized horseshoe kidney.

U358B

REPORT OF CASES

May 7, 1929 and the kidney exposed in the usuaI manner by Iumbar incision. The lower poIe was found firmly adherent and connected with the kidney of the opposite side by a thick isthmus of parenchymat renaI substance. It was easily made out and there was a

FIG. so. SagittaI view of the left half of the heminephrectomized horseshoe kidney of Case xv, showing the striking coincidence of caIcuIus, hydronephrosis, papiIIomata in the pelvis and hypernephroma in the Iower poIe of the same organ. Patient had an uneventful recovery.

definite indurated mass at the Iower poIe of the kidney. In view of these findings, heminephrectomy was carried out. The isthmus was cIamped and cut across, and after the usual Iigature of the ureter and pedicIe, haIf of the Ieft horseshoe kidney was removed. The stump of the raw isthmus was sutured and covered with fat and the edges of the wound brought together without difficulty. Two cigarette drains were pIaced, one in the renaI fossa and the other cIose to the sutured portion of the isthmus of the horseshoe kidney. The wound was cIosed in Iayers in the usuaI manner and patient had an uneventful recovery, Ieaving the hospita1 on May 28, 1929, definiteIy improved and apparentIy cured. The pathoIogica1 report of the specimen removed at operation reveaIed the haIf


of a horseshoe kidney with stone in the left kidney pelvis and a tumor at the lower poIe of the same half. The histological section of this kidney tumor has been cIassified as carcinoma. In this unusua1 case therefore, we fmd a coincidence of three distinct patho- Iogical lesions.

CASE XVI. T. S. D., maIe, aged forty, history No. 288,863, admitted to the Urologica Department of the New York Hospital on Sept. 25, 1929, compIaining of severe pain on left Iumbar region of a few hours’ duration.

On physical examination the abdomen was sIightly contracted with tenderness extending up into both lumbar regions which makes paIpation of the kidney impossibIe. The patient gave a history of having had on frequent occasions a dull lumbar aching at times in the umbiIica1 region. He had also suffered for several years with mild nocturia and frequency. Sept. 26, rgzg, cystoscopy, bIadder negative, both ureters catheterized, compIete uroIogica1 examination with functiona test done. X-ray fiIms revealed no shadow indicative of stone in urinary tract. The shadows of the kidneys were indefinitely made out. Both left and right lower poles appeared to be Iow in position. Left pyeIogram showed a moderate degree of hydronephrosis with a peculiar inverted peIvis and excavation of the Ieft kidney with the calices pointing inward, Right pyeIogram showed distorted calices with a somewhat rotated pelvis pointing toward the midIine. The impression was that of a horseshoe kidney with biIatera1 hydronephrosis and pyelonephritic infection. Patient was discharged improved October 3, Igzg, but was advised to return to the clinic for further cystoscopical treatment with diIatation of the ureters and kidney peIvis Iavage.

CASE XVII. A. Z., female, aged forty, housewife, referred to Arthritis CIinic of Hospital for Ruptured and Crippled of New York on April I, 1927, under the care of Dr. G. C. Snyder, complaining of “chronic arthritis” of more than four years’ duration. Patient is married, has z boys and 2 girIs all living and weI1. She states that she has been treated by numerous doctors and has been receiving treatment in various hospitaIs for her arthritis condition, aIso that the baking and massage have made her worse and brought no relief to the intermittent pain across her back. She is incIined to be chronically constipated, for which condition she has been receiving frequent enemas and high coIonic irrigations. Otherwise her genera1 condition is good. As she complained of pain across her back and sometimes in the Ieft Iower quadrant and umbiIica1 region, she was referred to the Urologica Department of the same HospitaI. Her chief comptaint at that time was of slight frequency of urination and nocturia with pain in the middle of the abdomen and radiating to both Iumbar regions. She aIso has pain across her back and in the spinal coIumn, which sometimes interferes with her breathing, and she is easiIy tired on working or standing. She states she has been suffering with arthritis and chronic constipation for many years for which she has been taking cathartics, enemas and rectal douches. She has also been compIaining of occasiona attacks of cystitis. The urine is hazy and contains microscopic pus. On May g, 1927, cystoscopy revealed a norma bIadder. The ureters were catheterized without diffIcuIty, and there was good renaI function on both sides. RoentgenographicaI examination revealed no shadow of stone anywhere in the urinary tract, but showed a Ieft kidney shadow of large size and very Iow in position, aIso a few shadows in the bony peIvis, apparently phIeboIiths. The left pyeIogram reveaIed rotation of the kidney peIvis with a slight degree of hydronephrosis, the Iower poIe extending beyond the midIine and giving the impression of horseshoe kidney with infection on the left peIvis. Later, on June 24, 1927, a pyeIogram of the right side revealed rotated kidney, with an unusua1 pelvis and some excavation of the middle caIices which pointed inward. The ureterograms were negative, except that the course of the ureters

REPORT OF CASES

aIongside of the spinal column gave the impression of a “ffower vase” which appears to be pecuIiar to the ureters in cases of horseshoe kidney. The renaI function was not par- ticuIarIy diminished on either side, but in view of the presence of pus ceIIs and red ceIIs in the catheter specimen, the patient was advised to have routine cystoscopic treatment with diIatation of the ureters and kidney peIvis Iavage in order to relieve infection and secure better drainage, which was accordingIy carried out on severa occasions. The result was gratifying, aIthough the patient still compIains from time to time of her horseshoe kidney symptoms but has not been able to return to the clinic for further treatments, for extraneous reasons.

CASE XVIII. W. S. maIe, ItaIian, aged thirty-eight, history No. 296,876, came to the admitting room of the UroIogrcaI Department of the New York HospltaI on Jan. 6, I 93 I, compIaining of pain in the bIadder region and in the epigastrium, radiating across the back and particularly to the right Iumbar region. Family history irrelevant. Patient has been suffering for severa years with dysuria, frequency and some bIadder symptoms; aIso with gastric trouble and chronic constipation for severa years. He denies venereal disease. On physica examination the abdomen was rigid and contracted but on deep paIpation the kidneys were easily paIpabIe and enlarged. There was aIso tenderness in the suprapubic region. The urine had been hazy for some time and apparently contained pus. BIood pressure was norma and Wassermann test negative. Cystoscopy January 7, 1931, reveaIed a much congested mucosa and buIbous edema. Around the uretera orifices and Aoor of the trigone, Iying at the fundus of the bIadder, was a stone about the size of the termina1 phalanx of the fifth finger. Both ureters were catherized without dificulty and revealed diminished function in regard to urea excretion and phenol- suIphonephthnIein elimination. Microscopical examination of the sediment showed pus and bIood ceIIs coming from both sides. The roentgen examination discIosed the presence of stone in the bIadder, and there was aIso a shadow about the size of a Iarge olive over the transverse process of the third Iumbar vertebra on the Ieft side, which moved con- siderabIy in the various pictures and was apparentIy a caIcified lymph-gland. The shadow of the kidney was larger than usuaI and the bitaterat pyelogram reveaIed rotation of the pelves with the Iower calices pointing inward toward the midIine, thus indicating the presence of a horseshoe kidney. In this case the stone was crushed and removed from the bIadder with the LowsIey rongeur. Patient has been receiving uroIogica1 treatment with dilatation of ureters and kidney pelvis irrigations. Hence no surgica1 intervention has been considered.

CASE XIX. N. S. maIe, aged thirty-six, flistory No. 177,402, came to the Urologica CIinic of the New York Hospital on July 17, 1930. compIaining chiefly of burning on urination, frequency, pain in the upper abdomen, particuIarIy in the umbilical region. The urine at times has been cIoudy and he has been receiving medical treatment by a private doctor on various occasions, but without definite retief. He has aIso been suffering with gastrointestina1 disorders and chronic constipation for many years, About tweIve years ago he had an attack of Neisser infection, since which time he has not had any urethral discharge. However, the urine has been cloudy and the symptoms have been aggravated in the Iast two years. Patient had various kinds of medica examinations and treatments by various doctors and had been cystoscoped elsewhere. He stated that guinea-pig examination of the urine had been negative, but a culture of the urine had disclosed the presence of BaciIlus coli communis. However, according to his statement, no one knew what was the cause of his troubIe. On physica examination patient was weII nourished and on deep paIpation of the abdomen the kidneys appeared to be enlarged and tender. He was cystoscoped July 20, 1930, and the bladder mucosa was found to bc


red and congested throughout but no evidence of pathoIogy found in the urinary bIadder. The ureters were catheterized without di&uIty and the specimen from both sides was ctoudy, with microscopic pyuria and hematuria. The function was sIightIy diminished, but urea excretion was good and phenoIsuIphonephthaIein appeared in five minutes on each side. The roentgen examination reveaIed no shadow indicative of stone in the urinary tract, and the kidney shadows were indefinitely made out, but were Iow in position. The biIatera1 pyeIogram reveaIed diIatation of both kidney pelves with sIight degree of hydronephrosis. Both peIves were rotated and the Iower caIices turned inward toward the vertebra1 coIumn, indicating the presence of a horseshoe kidney. Patient has been coming to the clinic for cystoscopic treatments with diIatations of ureters and kidney peIvis Iavage. WhiIe he has improved his symptoms have not cIeared up and he is stiI1 compIaining of the horseshoe kidney syndrome.

Cases of Horseshoe Kidney Diagnosed at Operation

CASE XX. M. F., male, aged fifty-six, history No. 280,898, admitted into the medical ward of the New York HospitaI JuIy 21, 1928, compIaining of indefinite pain in the umbiIica1 region and Iower abdomen with Ioss of weight, progressive weakness and a history of chronic constipation.

0 h n p ysica1 examination the abdomen was tender and an indefinite tumor mass about the right lower quadrant was made out, for which an expIoratory Iaparotomy was recommended. At operation a retroperitoneal fused kidney of the horseshoe type was found, together with chronic appendicitis. Appendectomy was done and the diagnosis after operation was that of a horseshoe kidney pIus chronic appendix. The patient made an uneventfu1 recovery and left hospita1 “improved.”

CASE XXI. C. M., femaIe, aged thirty-two, history No. 39,713, admitted to the medica service of the New York Hospital May 8, rgro with symptoms of pain in epigastrium and a history of stpmach troubIe after eating. The urine was apparentIy cIear. On physical examination there was sIight tenderness in the abdomen, particuIarIy at McBurney’s point. In the course of an operation for chronic appendicitis a fused kidney of the horseshoe type was discovered. The patient had an eventfu1 recovery from the appendectomy and Ieft the hospita1 apparentIy improved.

Cases of Horseshoe Kidney Found at Post Mortem

CASE XXII. P. W., male, aged sixty-six, admitted to the UroIogicaI Department of the New York HospitaI April 3, 1923, compIaining of inabiIity to urinate and pain over bIadder and umbiIica1 region. He had been tapped twice eIsewhere with temporary reIief but came to the hospita1 suffering with compIete retention after having used a uretera catheter for more than five weeks. On examination, a scar for perineal operation was noticed. There were moderate hemorrhoids and the prostate was enlarged to about twice the usua1 size and of leathery consistency. On account of the irritation and inabiIity to toIerate an indwelling catheter a suprapubic cystotomy for drainage was done ApriI 6 under IocaI anesthesia. Patient became very sick and liis renaI function alarming. On May 7 the bIood urea nitrogen was 34.27 and on May 8 he died in a uremic coma. The cIinica1 diagnosis was uremia, renal faiIure and chronic endocarditis, compIicated by enlargement of the prostate.

Autopsy No. 5732. Anatomical Diagnosis: Acute cystitis, chronic endocarditis, hypertrophy of prostate, chronic myocarditis, right hydroceIe and infected horseshoe kidney. The direct cause of death, whiIe undetermined, was apparentIy chronic endo-

REPORT OF CASES 363

carditis due probabty to the horseshoe kidney disease. PathoIogicaI findings and type of Iesion: The kidney is of the horseshoe type united by an isthmus which runs across the lower poles and in front of the great vessels. The organ weighs 280 gm. The ureters and

FIG. 51. FIG. 52. FIG. 5 I. Left pyeloureterogram reveaIing marked rotation and inversion of the

peIvis with the upper and Iower caIices pointing inward, at first giving the erroneous impression of horseshoe kidney. However, the outIine of the lower poIe is plainly visible and in addition the delineation of the psoas muscle can also be seen.

FIG. 52. BiIateraI pyeIogram of the same case, to iIIustrate the necessity of making biIatera1 p eIograms in order to ruIe out anomaIies of rotation which are not horseshoe kidney. T ii e pyeIogram of the opposite side shows no anomaly in shape or position, and the cIear outIine of the normaIIy pIaced peIvis and Iower poIe of the organ excludes the diagnosis of horseshoe kidney.

peIves lie anterior to the kidney parenchyma and are divided in such a manner that there is practicaIIy no renaI peIvis. This division of the ureters corresponds to the somewhat indefinite division of the fused kidney into four organs, which are suggestiveIy outIined by Iobulations. The vesseIs enter the horseshoe kidney at a number of definite points along the superior border of the fused organ: the position of the abnorma1 organ is rather Iow in the IumboiIiac peIvis, so that the upper poles are not in close contact with the adrenaIs. Sections revea1 preservation of norma Iine markings. However, micro- scopica1 examination of the kidney tissue shows that the tubuIar epitheIium and aIso the gIomeruIi are undergoing fairIy we11 marked degeneration. There appears to be slight increase in the interstitia1 connective tissue and further degeneration and changes of chronic nephritis.

CASE XXIII. A. M. W., female, aged fifty-two, history No. 289,909, admitted into the medica ward of the New York HospitaI Nov. 23, Igzg. The cIinica1 diagnosis was per-


forated gastric ulcer, geIatinous carcinoma of stomach, secondary anemia. Patient died Jan. 29, 1930.

Autopsy No. 6gog. Anatomical Diagnosis: Carcinoma of the stomach, gelatinous uIcer- ation of carcinoma; hourgIass constriction of stomach. BIood in intestina1 cana1. Gener- aIized edema. Moderate diIatation of heart. Hemorrhage through gastrointestinal tract. Infected horseshoe kidney. Direct cause of death: Carcinoma of stomach. (Horseshoe kidney?)

Pathological Findings and Type of Lesions: Kidneys are fused together, making a horseshoe kidney by the union of an isthmus across the vertebral column which unites both Iower poIes. The organ weighs 225 gm. The structure is crescent shaped. CapsuIes strip rather readiIy and the organ is rather light in coIor but has a smooth surface. On incision the markings are fairly reguIar; each haIf of the organ is drained by a single ureter which is attached to the anterior peIvis. The isthmus of the fused organ is made of kidney parenchyma. Microscopica findings show the tubuIes to be rather diIated and the gIomeruIi undergoing degenerative changes as in the characteristic Iesions of nephritis.

CASE XXIV. P. M., male, aged thirty-five, history No. 288,262, admit&d into the medical division of the New York Hospital Aug. 2 I, 1929.

Clinical Diagnosis: RetroperitoneaI Iymphosarcoma. Date of deatb: Sept. 21, 1929.

Autopsy No. 6834. Anatomical Diagnosis: RetroperitoneaI Iymphosarcoma with extension to stomach, spIeen and mediastinum, and with ulceration into the stomach. Generalized arterioscIerosis. Dilatation of heart. Horseshoe kidney with doubIe peIves and doubIe ureters on left side. Direct cause of death: RetroperitoneaI Iymphosarcoma with many metastases and marked emaciation. (Horseshoe kidney?)

Pathological Findings and Type of Lesions: Kidneys are connected across the midIine by the lower poles and in front of the great vesseIs, giving the horseshoe arrangement. The Ieft kidney has a doubIe peIvis and doubIe ureter, which opens into the Ieft side of the apparentIy norma bIadder. The ureters and peIves are situated anterior to the kidney substance; otherwise the kidney tissue and ureters are not unusua1, except for a moderate diIatation of the ureters and caIices. The kidney parenchyma appears normaI. MicroscopicaIIy one section of kidney shows granular degeneration of the parenchyma and edema of the gtomeruli. In another section there is a circumscribed mass of Iym- phocytes and poIymorphonucIears. The glomeruIi in this section show advanced hyaIine degeneration and other changes that suggest the presence of chronic interstitia1 nephritis.

CASE xxv. A. M., femaIe, aged fifty, hospita1 No. 288,691, admitted to the New York HospitaI Sept. 16, 1929.

Clinical Diagnosis: Aneurysm of arch of aorta. Lues. Carcinoma of rectum (?). Diabetes mellitus, mdd.

Date of Deatb: Sept. 30, 1929. Autopsy No. 6838. Anatomical Diagnosis: Aneurysm of arch of aorta. Luetic aortitis.

Carcinoma of rectum. Adherent pericarditis. Horseshoe kidney fused across the midline by the upper poIes.

Direct Cause of Death: Undetermined (syphilis, toxemia, cancer, horseshoe kidney disease).

Pathological Findings and Type of Lesion: Kidneys are fused by the upper poles in a most rare and unusual type of horseshoe kidney, the right being Iower than the leftlbut otherwise fairly normal in position. The entire specimen weighs about 350 gm. The isthmus that unites these upper pates is of definite renaI parenchyma1 tissue and the concavity opens downward. The pelves are smaII, the Ieft one containing two almost

TREATMENT 365

separate portions. The pelves and ureters appear to Iie anteriorIy to the renaI vessels and to the kidney parenchyma. The kidney substance seems rather paIe. The capsules strip with some difficuIty and leave scars behind.

Microscopical Findings: Rena1 degenerative changes of tubuIes and gIomeruIi. GIomeruIi are edematous in appearance whiIe others are hyaIinized as in lesions of chronic nephritis.

In Conclusion: This unusua1 type of horseshoe kidney is worthy of recording because, according to Papin and other writers, there have been onIy 13 cases of this type described in the Iiterature.

XI. TREATMENT

In the series of horseshoe kidneys which form the basis of this presentation the treatment has necessariIy been most varied, partIy because of the different types of cases and partIy because of the diffI- culty in foIIowing up out-patients. Furthermore, in some instances in which no gross concomitant pathoIogy was present the entity of

horseshoe kidney disease per se has not been recognized and therefore nothing more than the usua1 urinary antiseptics and kidney peIvis Iavage has been recommended. However, in studying the management of this condition together with the etioIogica1 factors and the rbIe of the anomaIy as predisposing to disease and to further pathoIogy, one must concIude that on genera1 principIes, in a11 cases of renaI fusion accompanied by the horseshoe kidney syndrome, with or without associated pathology, the fused organ must be considered in reaIity as a potentia1 surgica1 kidney. This study has aIso reveaIed that in the great majority of cases no reIief of symptoms can be definiteIy obtained unti1 the isthmus of the horseshoe renaI organ has been divided by the conservative surgica1 procedure of a

symphysiotomy operation. It is obvious, moreover, as I have aIready pointed out in the

chapters on etioIogy and anatomic structure, that the high ventra1 implantation of the ureters does not permit the normal drainage of the pelvis, and also that the reIative fixation and tension of the

organ across the midIine, pIus its weight and pressure upon the great abdomina1 vesseIs of the bIood, nerve and Iymphatic circuIation, interfere with the dynamic physioIogic contractions of the excretory apparatus, thus producing chronic stagnation of both the urinary and gastrointestina1 tracts. Hence the great majority of patients suffering from horseshoe kidney disease do not appear to obtain

permanent cure under the ordinary medica and urologica treat-


ment. It appears, therefore, that in the soIution of this probIem the onIy possibIe avenue to permanent reIief or cure must Iie in a surgica1 approach such as is offered by division of the isthmus of the fused organ and the Iiberation of the ureter to provide free drainage and restoration of norma function.

As an iIIustration of this fact we see that in this series of 25 cases onIy 2 appear to have been cured, and that these are preciseIy the two in which heminephrectomy was carried out for a stone com- pIicating hydropyonephrosis in one haIf of or on one side of the fused organ. The rest of these cases are cIassified as foIIows: Improved but stiI1 with symptoms, IO patients; unimproved, 7 patients; died,

2 patients; (one from pneumonia compIicating horseshoe kidney disease and the other from acute uremia due to the horseshoe kidney). Four other patients died from different causes, but horseshoe kidney was found in a11 at post mortem.

It seems, therefore, that in order to soIve this cIinica1 probIem before any concomitant Iesion arises and to bring permanent reIief of symptoms that have already appeared, three modes of management of this condition must be considered: the medica1, the uroIogica1 and the surgica1. These three modes may be summarized and discussed in two essential groups:

I. Acute cases of horseshoe kidney with or without associated

pathoIogy. 2. Chronic cases of horseshoe kidney disease with cIinica1

symptoms. I. In the group of cases of horseshoe kidney disease with acute

cIinica1 manifestations, after correct preoperative diagnosis the paramount consideration is the maintenance of urinary drainage in order to reIieve infection, autointoxication and back pressure. In the majority of instances the urinary stasis produced by Iack of proper drainage is the true cause of the infection and of the deveIop- ment of hydronephrosis, pyohydronephrosis, acute pyeIitis and

pyeIonephritis, nephritis, perinephritis and perinephro-ureteritis which, when cIinicaIIy acute, are manifested by high temperature, chiIIs, fever, sepsis, gastrointestina1 disorders, nausea, vomiting, severe pain in the epigastrium, uremic symptoms and pyuria, dysuria or hematuria, etc. When a cIinica1 picture of this type reveaIs the gravity of the condition, expectant medica and uroIogica1 treatment

TREATMENT 367

is indicated, consisting of the administration of urinary antiseptics internaIIy, and of forced fluid either by mouth or by rectum with enemas and high coIonic irrigations, cathartics and intravenous infusion of normaI saIine soIution, accompanied by rest in bed, heat and other common proper measures. At the same time the use

of indweIIing uretera catheters is essentia1 to secure drainage and to reIieve infection. SeveraI of the cases here reported have been treated in this way with satisfactory resuIt.s, as I have described eIsewhere with the report of my first case.’

Later on, when the patient is out of bed, he shouId be put on a

suitabIe non-protein diet with appropiate medication, and shouId receive cystoscopic treatments at ten-day or two-week intervaIs in order to diIate the ureters and irrigate the kidney peIvis, using a miId antiseptic soIution, such as acriffavine I : 1000, revono1 dextrose I :2000 or siIver nitrate I :IOOO, thus assuring the main-

tenance of perfect drainage and preventing the deveIopment of infection or formation of stone or further pathoIogy. The treatment, in short, is that of an ordinary case of pyeIitis or pyeIonephritis.

2. WhiIe patients suffering with acute horseshoe kidney may improve under this treatment, it has been observed that in the great majority of cases that have been traced over a Iong period of time, as in this series, they are bound to reIapse and to have frequent attacks of their troubIe. The horseshoe syndrome persists with its abdomina1 pain, its urinary symptoms, its gastrointestina1 disorders, and its habitua1 or usua1 chronic constipation; the urine is hazy or cIoudy from time to time and contains pus and bIood cells and albumin, a certain degree of nephritis is present and the individ- ual aImost invariabIy becomes incapacitated from horseshoe kidney disease. In this group of chronic cases, therefore, after paIIiative treatment has been exhausted, the symphysiotomy operation, to divide and separate the isthmus of the fused renaI mass, shouId constitute the most correct and fina indication for assuring a curative prognosis.

Martinow, on March g, Igog, was the first to divide the isthmus in order to separate the two fused kidneys. But it appears that Rovsing on June 17, 1910, was the first to describe in fuI1 the

1 Gutierrez, R. The value of indweIIing uretera catheters in urinary surgery. Surg.

Gynec. Obst., so: 441-454, 1930.


technique of the new operation that bears his name, reporting a successfu1 case and pointing out the advantages of the method even in the normaI horseshoe kidney. He recommended the transperitonea1 route of approach by the midhne incision of Iaparotomy, but whiIe the method was practiced by Kroisz, Brougersma, Van Houtum, Kidd and others it has never gained popuIarity for the reason that practicaIIy a11 these kidneys are infected; hence, the great damage infficted in the contamination of peritoneum is obvious.

It was Papin who, on October 22, 1922, introduced a modified technique using the Pian transverse Iumbo-anterior incision for an extraperitonea1 approach to separate the isthmus of the fused organ with satisfactory resuIts. According to this author and the tabulated study of cases cohected from the Iiterature by Eisendrath, Phifer and CuIver as we11 as other authors mentioned previousIy, it appears that a11 those patients who submitted to the division of the isthmus, or renaI symphysiotomy operation, have had compIete reIief of pain and symptoms. In dividing the isthmus, as Papin has pointed out, it is advisabIe to do a nephropexy or suspension of haIf of the organ on the side at which the operation has been performed, since this aIIows the kidney to swing back into the Iumbar fossa where it normaIIy beIongs and at the same time permits the other haIf on

the opposite side to move away from its unique position upon the soIar pIexus and the great abdomina1 vesseIs, thus uItimateIy serving to bring about a permanent cure. In addition care shouId

be taken of the aberrant renaI and pyelic vesseIs, as we11 as of the adhesive bands of tissue which interfere with free ureteral drainage. The kidney shouId be pIaced in such a position that the peIvis and inferior caIices wiI1 secure a perfect drainage. The ureter should be Iiberated from the isthmus and from its renaI surface and peritonea1 attachments in order to restore the physioIogica1 mechanism of its function. With a good preoperative diagnosis and a precise technique, in order to contro1 any oozing or bIeeding from the divided renaI isthmus, these operations can be carried out with safety and without great diffrcuIty. They resuIt in permanent cure, as in the two cases of heminephrectomy here reported in which the technique was more or Iess that of an ordinary nephrectomy or partia1 heminephrectomy.

Other operations may be required in the surgica1 management of horseshoe kidney, particuIarIy when the presence of associated

SUMMARY 369

pathology demands them and obviousIy when the other half of the organ has enough function to maintain Iife. In this series of cases, two patients were heminephrectomized successfuIIy for hydropyonephrosis compIicating stone in one-half of the organ, with curative resuIts. Two other patients were operated upon for the remova

of renaI stone by an anterior pyeIotomy. One cystostomy was done and one IithoIapaxy, and in 3 cases stone was removed from the ureters and bIadder by cystoscopic and uretera manipuIations.

AIthough many of these patients have improved considerabIy under medica and uroIogica1 treatment, some of them who stiI1 have cIinica1 symptoms and in whom a surgica1 procedure was

pIanned have not been traced or have not come back to the cIinic, whiIe others have refused the operation. The writer, however, is fIrmIy convinced of the beneficia1 resuIts of the symphysiotomy procedure and beIieves that it is properIy indicated in this type of cases of symmetric horseshoe renaI fusion. He expects in the near

future to be able to report an improved technique and the resuIts obtained from the same.

In summing up the cIinica1 management of horseshoe kidney it is obvious that accuracy in estabIishing a correct diagnosis and

the proper indications for medica1, uroIogica1 and surgica1 treatment are the paramount considerations for the assurance of a sound prognosis.

Horseshoe kidney disease as a new cIinica1 entity can be easiIy recognized both cIinicaIIy and radio-urographicaIIy, and whenever the horseshoe syndrome of nephraIgia and other cIinica1 manifestations is suggestively present, the urographic examination wiI1 con- firm and verify the fina diagnosis.

BiIateraI instrumental pyeIograms whenever possibIe (taken at different sittings if necessary) or intravenous uroseIectan pyeIo- grams are absoIuteIy necessary, not onIy for the verification of the diagnosis but aIso in order to excIude the possibiIities of other abnormalities, since the Iack of, or the incompIete, rotation and inward inversion of one peIvis seen in a uniIatera1 pyelogram, does not always mean the presence of horseshoe kidney. We must also

excIude the embryonic type of renaI peIvis and the unusuaIIy pIaced


peIvis due to an ectopic kidney or dystopic asymmetric renaI fusion, or to a certain degree of nephroptosis, or to torsion or renaI misplacements resuhing from trauma or intraperitonea1 or extra- peritonea1 neoplasma. To this end the routine procedure of measur- ing the basa1 angIe in the horseshoe triangIe as here proposed by the author wil1 serve as a means of eIiminating error and cIarifying the

fina diagnosis. NevertheIess, it is essential that the diagnosis shaI1 correspond

with the clinical condition of the patient, and to be compIete it should be based in the foIIowing roentgenographic and urographic data : (I) The visuaIization of the outIine and position of the kidneys;

(2) th e ossi e e ineation of the isthmus; (3) renaI shadows of p b1 d I caIcuIi close to the vertebra1 coIumn or overlapping it; (4) in a

biIatera1 pyeIogram, the abnorma1 rotation of the peIves; (5) the Iower calices pointing inward; (6) the “flower vase” position

of the ureters; (7) the “bottIe neck” shape at the ureteropeIvic junction; and (8) the “pathognomonic pyeIographic horseshoe triangle ” with its minimum basa1 angIe hovering around 20’. A consideration of al1 these points in the diagnosis wiI1 definiteIy reveal

the presence of the cIinica1 entity of horseshoe kidney disease. In Iooking over this series of cases, one cannot fail to be struck

by the fact, that, of the group of rg patients diagnosed preopera- tiveIy, practicaIIy every one came in with an erroneous diagnosis, and 12 of this group had aIready been operated upon elsewhere for various abdomina1 conditions, before our diagnosis of horseshoe kidney was made, thus bringing out the paramount importance and necessity of a correct preoperative diagnosis.

The treatment of this condition in acute cases shouId be medica and uroIogica1, but Iater on, when the patient has recuperated from the acute symptoms and compIete invaIidism, and before any further pathoIogy deveIops, it shouId aIways be surgica1, estabIishing the division and separation of the isthmus of the fused organ by renaI symphysiotomy. This operative procedure shouId be foIIowed at the same time by a nephropexy or suspension of one haIf of the organ on the side where the operation has been performed. Care should also be taken to free the ureter and peIvis from bands of adhesions and aberrant blood vessels, in order to restore ureteric and pelvic dynamic physiological function and to secure a better drainage.

CONCLUSIONS 371

AI1 in ah, the resuhs obtained in the clinica management of this series of cases have been most gratifying, particuIarIy when uroIogica1 treatment has been SystematicaIIy carried out in the form of diIatation of the ureters and kidney peIvis Iavage and in the acute cases when the indweIIing uretera catheter has been used, thus

saving patients from uremia and death. Of the rg cases diagnosed preoperativeIy, 15 (79 per cent)

were suffering with pyelitis and pyeIonephritis infection due mainIy to urinary stasis and Iack of proper drainage, which are obviousIy the chief etioIogica1 factors in horseshoe kidney disease and represent the beginning of its most common complication.

XIII. CONCLUSIONS

I. Horseshoe kidney disease is physioIogicaIIy, anatomicaIIy,

pathoIogicaIIy and surgicaIIy of sufficient frequency to warrant its medica recognition as a new cIinica1 entity of paramount

importance. 2, The disease is produced mainIy by the unique position of the

anomaIous organ in the middIe of the abdomen and in the retro-

peritonea1 space, Iying where it not onIy makes pressure upon the aorta, vena cava, soIar pIexus and other anatomic structures, but aIso, where by its incarcerated position, it is prevented from performing its own norma ph ysioIogic function.

It is cIinicaIIy characterized by the presence of the horseshoe kidney syndrome, nameIy, (a) nephralgia or pain in the middle

of the abdomen referred to the epigastrium or umbiIica1 region; (b) gastrointestina1 disorders with a Iong history of marked chronic constipation, and (c) Iong-standing intermittent attacks of urinary

disturbances (see TabIe IV of the text). 3. Every individua1 who is born with this type of renaI fusion

is potentiaIIy suffering from a certain degree of horseshoe kidney disease (chronic, acute or subacute).

4. Two types of horseshoe kidney must be considered, the symmetric and the asymmetric. In the symmetric type, the fusion of the two organs is made by the union of the Iower or the upper poIes by an isthmus of renaI parenchyma, with a concavity facing upward or downward, and with the connecting isthmus Iying in the midIine across the vertebra1 coIumn, in front of the great abdomina1


vessels and adherent to the parietaI Iayer of the peritoneum. This anomaIy of renaI fusion constitutes the typica anatomic structure

which is characteristic of the true type of horseshoe kidney disease. In the asymmetric type the two kidneys are united in various other abnorma1 forms and occupy an ectopic position. This very unusua1 renaI anomaIy, which as a ruIe is pIaced Iow down in the bony peIvis or at one side of the spinal coIumn, is in reaIity not of the true horseshoe type, and wiI1 be discussed Iater in another paper. Of the first type of symmetric renaI fusion or renal symphysis, 25 cases are here reported.

5. The etioIogica1 factors in the fusion of the two kidneys, whiIe undetermined, appear to date from the first weeks of embryonic Iife when the two kidney bIastemata are imbedded and pIaced

together topographicaIIy within the preaortic mesoderma1 membrane of the mesonephros, whereupon, probabIy for Iack of migration and rotation, the two organs unite to constitute the semicircuIar horseshoe renaI mass before they have a chance to ascend into the normal position in the Iumbar fossae of the aduIt.

6. AI1 of the cases in this series were found to have various degrees of pathoIogy. TweIve of the patients had been operated upon eIsewhere for various abdomina1 conditions under erroneous diagnoses, without obtaining symptomatic reIief and before our diagnosis of horseshoe kidney was made. Two deaths occurred in this series, one from uremia and one from a pneumonia compIication, but both probabIy as the resuIt of horseshoe kidney disease of Iong standing. Four other deaths have been reported, of patients in whom horseshoe kidney was found at autopsy, and in whom it can be fairIy assumed that the kidney anomaIy pIayed an important part in the concomitant pathoIogy and the causation of death. In onIy I case, found at necropsy, were the two haIves of the organ united by the upper poIe, which constitutes a very rare instance.

7. Of the 25 cases here reported, rg were diagnosed preopera- tiveIy by uroIogic and radio-urographic examination, 2 were discovered at operation and 4 at autopsy. Of the rg cases that received uroIogic and urographic examination, the diagnosis was correctIy made in aI1, leading to the concIusion that when the patients are submitted to a compIete and accurate examination the diagnosis can be estabIished in IOO per cent of the cases.

CONCLUSIONS 373

8. Most of the pathology found is due to the high impIantation of the ureters and to the ventraI position and inward rotation of the peIves with the Iower caIices extending transversejy into the renaI isthmus, a condition which is obviousIy responsibIe for the Iack of norma drainage and which Ieads uItimateIy to urinary stasis, thereby causing continuous irritation and infection, with histo- pathoIogica1 evidence of chronic pyelitis and pyelonephritis, nephritis and perinephritis and in some instances the formation of hydronephrosis, pyonephrosis or even fata uremia (see TabIes I, II and III).

9. The weight, pressure and reIative fixation of the incarcerated

organ, pIus the continuous tension caused by the intra-abdomina1 pressure, interfere with the dynamic physioIogic function of the entire excretory apparatus and become responsibIe for the chronicity or exacerbation of a11 the abdomina1 symptoms and for the chronic stagnation of the intestina1 as we11 as of the urinary tract.

IO. The diagnosis of this condition must be based in the first

pIace on the suggestive signs and symptoms and shouId then be confirmed by both roentgenographic and urographic examination, with the main dependence pIaced on the biIatera1 pyelogram, obtained either by instrumental pyeIography or by intravenous injection of uroseIectan.

I I. Other important points in the diagnosis are the comparative estimation of renaI function and the determination of concomitant or associated pathoIogica1 Iesions, in order to estabIish the appropiate operative treatment.

12. The graphic points for estabIishing a correct diagnosis of horseshoe kidney are as foIIows: (I) The visuaIization and perfect outIine of the position of the kidneys; (2) the possibIe deIineation of the isthmus by the roentgen ray; (3) renaI shadows of caIcuIi cIose to the vertebra1 coIumn or overIapping it; (4) in a biIatera1 pyeIogram, the abnorma1 rotation of the peIves; (5) the Iower caIices pointing inward and toward the midIine; (6) the “Aower vase” position of the ureters; (7) the “bottIe neck” shape at the uretero- peIvic junction, as if the ureters were Ieaving the peIves from behind; (8) the “pathognomonic pyeIographic horseshoe triangIe,” with its minimum basa1 angIe of approximateIy 20” which, taken in com- bination with a11 these other points, wiI1 revea1 the diagnosis of the clinica entity of horseshoe kidney disease (see Fig. 39).


13. The treatment of this condition must be considered from

three different standpoints, nameIy (a) medica1, (b) uroIogica1 and (c) surgica1.

(a) In the medica treatment when acute symptoms are present, rest in bed, forced Auid, urinary antiseptics, cathartics, high coIonic irrigations and intravenous infusion of nolma1 saIine soIutions are

indicated. (b) The uroIogica1 treatment consists mainIy in securing drainage

and in reIief of infection by cystoscopic treatments, kidney peIvis Iavage and the method of the indweIIing uretera catheters.

(c) It appears that the idea1 treatment for this condition, as

soon as the patient is reIieved from the acute symptoms, is surgica1 intervention. Two types of cases must be considered: (I) Cases in which some concomitant associated pathoIogy is present, and in which some type of surgica1 intervention may be required as in any other pathoIogica1 Iesions of the kidneys. ObviousIy one-haIf of the horseshoe organ must have enough function to maintain Iife, particuIarIy when heminephrectomy is the operation to be carried out. (2) Cases in which no visuaIized pathoIogy is present, but stiI1 the horseshoe syndrome is cIinicaIIy evident. Here renaI symphysiotomy for the division of the isthmus of the fused kidney shouId be the operative procedure of choice, foIIowed by routine nephropexy, nephroIysis and UreteroIysis.

14. There can be no doubt that those unfortunate individuaIs who have been born with horseshoe renaI deformities have the same cIaim on the corrective possibiIities offered by modern surgica1 procedure as those suffering with any other congenita1 surgica1 maIformation of the human body under the dominion of the surgeon’s knife.

In view of the fact that horseshoe kidney is universaIIy recognized as a predisposing cause of disease, giving rise to clinica symptoms, pain and suffering, and as a ruIe to concomitant pathoIogy, it appears that the soIution of the cIinica1 probIem of horseshoe kidney disease must be found in the extraperitonea1 operation of renaI symphysiotomy or symphysiectomy, whenever possibIe. This operation is a conservative and preventive procedure that finds its justification in the assurance it offers of a good prognosis, finaIIy reIieving symptoms and achieving permanent cure.

REFERENCES 375

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(The End)

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Clinical management of the horseshoe kidney: Part III (Conclusion)

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