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Congenital Absence of the Lumbosacral Spine
Vol. 101, Mar. Printed in U.S.A.
TnE J"ouRNAL OF UROLOGY
Copyright © 1969 by The Williams & Wilkins Co.
CONGENITAL ABSENCE OF THE LU:i'vIBOSACRAL SPINE CARL K. PEARLMAN
AND
ERNEST BORS
From the Urology Division, Orange County Medical Center; the Spinal Cord Injury Service, Long Beach Veterans Administration Hospital and the Department of Surgery, University of California, Los Angeles, California
Congenital absence of the lumbar spine with a long survival is rare. A case is herein reported of a young man, now 18 years old, completely independent and fully employed, who had this congenital anomaly and whom ,Ye have observed for the past 10 years. vVe could find only 13 cases previously reported. 1- 5 CASE RBPORT
M. P. was first seen by us in July 1957 when he was 8 years old. The patient had complete absence of the lumbosacral spine below the 12th thoracic vertebra. His father, n 100 per cent disabled veteran, had contracted poliomyelitis leaving residual weakness in both arms. His mother is a diabetic. The patient had complete urinary incontinence and his feces was impacted. A simple colostomy had been done elsewhere on January 14, 1954 and had been converted into a double loop colostomy on July 9. A distal loop prolapse had been reduced conservatively on September 24, and a recurrent prolapse had been corrected by excision of 6 inches of the distal loop on October 1. A prolapse of the proximal loop necessitated its reduction by laparotomy on :viarch 11, 1955. An ileocecal intussusception had to be reduced Accepted for publication April 1, 1968. Russell, H. E. and Aitken, G. T.: Congenital absence of the sacrum and lumbar vertebrae with prosthetic management. A survey of the literature and presentation of five cases. J. Bone Joint Surg., 45-A: 501-508, 1963. 2 Pirkey, E. L. and Purcell, J. H.: A.genesis of lumbosacral vertebrae. A report of two cases in living infants. Radiology, 69: 726-729, 1957. • Blume!, J., Evans, E. B. and Eggers, G. vV. N.: Partial and complete agenesis of malformation of the sacrum with associated anomalies. Etiologic and clinical study with special reference to heredity: a preliminary report. J. Bone Joint Surg., 41-A: 497-518, 1959. 4 Rusnak, S. L. arid Driscoll, S. G.: Congenital spinal anomalies in infants of diabetic mothers. Pediatrics, 35: 989-995, 1965. 5 Frantz, C. H. and Aitken, G. T.: Complete absence of the lumbar spine and sacrum. J. Bone Joint Surg., 49-A: 1531-1540, 1967. 1
by a laparotomy on April 9. A recurrent ileocecal intussusception with evisceration of the intus-suscepted bowel through the proximal stoma of the colostomy on May 10 required surgical correction and an attempt had been made to affix the right colon to the right gutter. \Vhen first seen by us the boy had urinary incontinence. A Cunningham clamp had been used temporarily but had to be discontinued because of a penile pressure sore. The patient had a vague sensation of fullness when the bladder was filled and emptied some urine by forceful abdominal straining. His mother had to go to school 3 times in the morning and afternoon to change the boy's diapers. The patient appeared to be a bright young boy of normal appearance from the waist up. However, he sat in the typical Buddha position with the atrophic apvendages of his lower legs folded under him, using the feet as a cushion. He had popliteal webs, a large thoracic diameter discrepant from a small pelvic diameter and kyphosis of the thoracic spine (fig. 1, A). He had bilateral varalytic talipes equinovarus and a double barrel right upper quadrant colostomy. The penis was small with meatal stenosis which was corrected by meatotomy. Good renal function was noted on excretory urography, although malrotation of the right kidney was present (fig. 1, B). Cystoscopy was impeded by bladder spasticity during filling. No reflux was seen with delayed cystography. Following a suprapubic cystostomy on December 9, 1957 the patient continued to have leakage through the urethra. Because of this leakage the vesical neck was closed on June 13, 1958. However, even a Robinson catheter secured by a shield was extruded as was the Foley bag before the Robinson was used. Urinary infection was controlled with chemotherapy. A small bowel intussusception developed on February 28, 1959 which was corrected surgically. At this time the double barrel colostomy was converted to a terminal single loop colostomy
374
COXGEKI'l'AL ABSENCE OF LUMBOSACRAL 8PlNE
F1G, 1 A, x-ra 0- of spine revealR normal nrnnber of thoracic vertebrae, H, excretory nroµ;rarn n t, :ig:e
FIG,
2, Location of stornas
and :3 to 4 inches ol' Lninsver~c colon were resected diRtall:r to tlrn colo~torny, For the past 8 seam the colostomy has worked welL To soln' the urinary difficulties resulti1,1g from the unsatisfactory function of the SUJJrnpubic rathctN, a sigrnoiclocystoplasty was done on December 7, 1959, The anastomosis 1nts in isoperistaltic direction and the stoma was placed in the left lower abdominal quadrant, Therethe patient has two stomas, one on the right for feces and one on the left for urine (fig, 2), Special appliances lmd to be made because of
the 1ia1Tcm- illtcrnpacc beb-cen the rib cage a11d the peh-is. The skin smrouuding the left colm,, tomy was revised on January 28, J 963 heca11s" of kerntosis, Since then tbere have been no ('011, plaints rC'gardi11g either colostomy, On :\foy 12, 1966 an ice water test \\'as 11eµ;a tin!, Xo reflux was found 011 ry~togrnphy; howeyer, there was some narrowness of i,he aw.1stomotic site between the bladder and the; colonit: conduit (fig, 3, A_), An excretory urogram 011 September 15, 1967 shm,-ecl no essential frnm previous findings (fig, 3, B) The hlo()(l meu,
376
PEARLMAN AND BORS
FIG. 3 .•4-, colocystograrn at age 18. B, excretory urograrn at age 18
nitrogen (BUN) was 11.5 mg. per cent and creati:nine was 0.7 mg. per cent. The penis was 4.3 cm. in length and 2 cm. in width. The testes ,vere still small, although they had increased in size. The patient had pubic hair; he hud no erections. Neurological examination on December 14 showed cranial nerves and reflex activity of the upper extremities within physiological limits and the lower extremities completely flaccid, without deep tendon reflex responses and without pathologic toe signs. They were contracted at the hips and knees. Sensution of touch was preserved throughout the body, including the lower extremities, saddle area and genitalia. Deep pressure ,;rns perceived but was not painful at the toes and heelcords. There seemed to be little, if any, kinesthesia in the toes. Vibrator.1' sensation was retained in the entire body ·when it ,ms tested by bone conduction but was absent below Ll ,vhen it was tested in the skin. There was analgesia below Ll. Rectal examination was interpreted as painful, although the external anal sphincter ,ms flaccid and the bulbocavernosus and cough reflexes could not be elicited. DISCUSSION
Of the 13 patients with agenesis of the spine below Tll, Tl2, Ll and L2, 1- 4 9 \\-ere boys, 2
were girls and the sex of two was not mentioned. Of the 9 boys, five were living at 9 months, 8, 9, 11 and 15}~ years and 1 girl was alive 8 years; to this we add our patient with agenesis below Tl2 as the sixth surviving male patient, now 18 years of age. Of the 2 girls with agenesis below Ll, one survived for 63 hours and the other is alive at 8 years. Two children of unreported sex survived for 9 months and 3 years. The survival rate is better when agenesis of the spine occurs at lower levels, L4 rtnd below, as is reflected in the 8 cases of Blumel and associates (3 boys and 5 girls, all survivecl). 3 The etiology is unknown, rtlthough dirtbetes of the mother seems to play a role. Insulin injections into the chick embryo produced vertebral changes when done within the first 2 clays of incubation. 6 Malformation of the spine produced in rabbits by insulill could be counteracted by riboflavin. 4 The clinical picture which we observed cor-· responded with the description in the literature. Characteristic mermaid or siren deformity of the legs was present which produces a Buddha or frog-like sitting position. The ribs left the vertebrae at right angle (90 degree) instead of the acute angle with a caudad 6 Duraiswami, P. K.: Insulin-induced skeletal abnormalities in developing chickens. Brit. Med. J., 2: 384--390, 1950.
CONGENITAL ABSENCE OF LUMBOSACRAL SPINE
course. A similar difference of angulation is found in nerve roots of patients with a short filum terminale or with a tethered spinal cord syndrome. Occasionally agenesis of the spine is associated with a meningomyelocele but this was not the case in our patient. Neurologically, the discrepancy of motor versus sensory deficit was of interest which resembled the picture of an anterior cord syndrome with chief involvement of the lateral columns, i.e. motor paralysis and analgesia but preservation of other modalities, with one important exception, namely, the total flaccidity of paralysis. How much this is caused by the proximity of a nutrient artery which branches off the aorta and enters the spinal canal between the 11th and 12th thoracic vertebrae is merely conjectural. This difference in motor and sensory innervation has been described. With a lesion at this level visceral deficit should be expected. This included bladder, bowel and sexual function in our patient, who showed signs of a lower motor neuron lesion not too different from that of patients with traumatic conus terminalis and/or cauda equina lesions. Such patients empty their bladders by strain and only about 25 per cent of them retain exclusively psychic erections.7 In cases of low (sacral) spinal malformation upper motor neuron lesions are not infrequent. Was the first colostomy really necessary in our patient or could it have been avoided by proper management, as done in patients with spinal cord lesions from trauma or disease? Bowel dysfunction has not been the indication for colostomy in any of the 2,300 traumatic paraplegics at the Long Beach Veterans Hospital. Neither has any of the more than 100 patients with myelodysplasia seen in consultation at the Orange County Medical Center, Harbor County Hospital and the Childrens Hospital had a colostomy. Had this patient been managed without the primary colostomy he would have been not only spared the many interventions which entailed from complications, but the operating surgeon would not have been saddled with the dilemma of what diversional procedure to use. The ileal bladder is presently fashionable as the method 7 Bors, E. and Comarr, A. E.: Neurological disturbances of sexual function with special reference to 529 patients with spinal cord injury. Urol. Survey, 10: 191-222, 1960.
377
of choice in uncontrollable urinary incontinence caused by myelodysplasia, especially in female subjects. This operation was considered for our patient but rejected because of two difficulties: 1) the presence of adhesions caused by several former laparotomies and 2) the location of the stoma of the colostomy in a place where an ileal bladder is usually placed. This then logically led to the decision to use the defunctionalized colon for the cystosigmoidostomy. This procedure had the advantage of preserving the competence of the vesicoureteral junction, preventing reflux and renal infection to which congenitally malformed kidneys are known to be prone. Our operation proved to be a success but it does not pretend to be the method of choice for patients with this affliction, when they are managed properly (i.e. conservatively) in regard to their bowel dysfunction. It is a moot question as to the outcome had a conservative route been taken; but for the sake of professional discussion let us consider a few points of a therapeutic approach which we would choose today if we were presented with such a patient without colostomy in early life. The first would be bowel regulation with bulkforming substances, lubrication of the intestines and stimulation of the defecation reflex with suppositories or enemas of glycerin, mineral oil and water in equal parts. The urinary incontinence would have been treated with bladder hygiene, i.e. regularly spaced intervals of drinking a set amount of fluid and by the attempt to empty the bladder by either Crede pressure or by abdominal straining, since the innervation of the abdominal musculature was intact prior to colostomy. Incontinence, accompanying myelodysplasia or its allied conditions of a short filum terminale or spinal malformations, is more amenable to conservative therapy in boys than girls since a useful female urinal has yet to be invented. The shortness of a penis can be corrected by plastic methods so that a condom urinal or its equivalent in children, a finger cot, can be applied.8 Cunningham clamps must be padded with cotton or thin tissue paper and frequently opened if decubitus is to be avoided; these appliances are used only exceptionally today in order to correct the incontinence of neurologic etiology with absent penile sensation. 8 Bors, E.: Scrotal skin plasty to elongate the penis. J. Urol., 87: 419--421, 1962.
378
PEARLMAN AND BORS
The case herein reported shows what is wellknown today, that suprapubic cystostomy will not keep a patient who has a lower motor neuron lesion dry. The much maligned urethral catheter is preferable, especially when applied in its whistle-tip form and properly managed (changed frequently). Foley bag catheters will irritate the bladder causing small segmental, uncoordinated contractions which can be reduced, however, by banthine and/or atropine. We have failed to apply these drugs or topical anesthesia to contain this type of bladder response to the suprapubic catheter. By virtue of entering the viscus through normally innervated structures the catheter was, perhaps, more irritating than a urethral catheter, which would have traversed denervated, although incompletely deafferentated, parts of the body. The ultimate goal, without the primary colostomy, would have been the same as the one finally achieved, namely, to render the patient free of a catheter and of urinary infection. There
can be no doubt that the primary colostomy interfered with the abdominal straining power or Crede pressure, essential for bladder rehabilitation in patients with lower motor neuron lesions. Therefore, our case shows the complicating events which can be started by one wrong indication and, also, that utilization of the defunctionalized sigmoid for cystocolostomy can be useful under these extraordinary circumstances. SUMMARY
A case of congenital absence of the lumbosacral spine is presented. This is the fourteenth case of this severe malformation reported in the literature. Our patient is now 18 years old; he has been followed for 10 years. Urinary incontinence was eventually solved by surgical closure of the vesical neck and a colocystostomy. The literature is reviewed and the general management of this type of myelodysplasia is discussed with regard to bowel and bladder function.
TnE J"ouRNAL OF UROLOGY
Copyright © 1969 by The Williams & Wilkins Co.
CONGENITAL ABSENCE OF THE LU:i'vIBOSACRAL SPINE CARL K. PEARLMAN
AND
ERNEST BORS
From the Urology Division, Orange County Medical Center; the Spinal Cord Injury Service, Long Beach Veterans Administration Hospital and the Department of Surgery, University of California, Los Angeles, California
Congenital absence of the lumbar spine with a long survival is rare. A case is herein reported of a young man, now 18 years old, completely independent and fully employed, who had this congenital anomaly and whom ,Ye have observed for the past 10 years. vVe could find only 13 cases previously reported. 1- 5 CASE RBPORT
M. P. was first seen by us in July 1957 when he was 8 years old. The patient had complete absence of the lumbosacral spine below the 12th thoracic vertebra. His father, n 100 per cent disabled veteran, had contracted poliomyelitis leaving residual weakness in both arms. His mother is a diabetic. The patient had complete urinary incontinence and his feces was impacted. A simple colostomy had been done elsewhere on January 14, 1954 and had been converted into a double loop colostomy on July 9. A distal loop prolapse had been reduced conservatively on September 24, and a recurrent prolapse had been corrected by excision of 6 inches of the distal loop on October 1. A prolapse of the proximal loop necessitated its reduction by laparotomy on :viarch 11, 1955. An ileocecal intussusception had to be reduced Accepted for publication April 1, 1968. Russell, H. E. and Aitken, G. T.: Congenital absence of the sacrum and lumbar vertebrae with prosthetic management. A survey of the literature and presentation of five cases. J. Bone Joint Surg., 45-A: 501-508, 1963. 2 Pirkey, E. L. and Purcell, J. H.: A.genesis of lumbosacral vertebrae. A report of two cases in living infants. Radiology, 69: 726-729, 1957. • Blume!, J., Evans, E. B. and Eggers, G. vV. N.: Partial and complete agenesis of malformation of the sacrum with associated anomalies. Etiologic and clinical study with special reference to heredity: a preliminary report. J. Bone Joint Surg., 41-A: 497-518, 1959. 4 Rusnak, S. L. arid Driscoll, S. G.: Congenital spinal anomalies in infants of diabetic mothers. Pediatrics, 35: 989-995, 1965. 5 Frantz, C. H. and Aitken, G. T.: Complete absence of the lumbar spine and sacrum. J. Bone Joint Surg., 49-A: 1531-1540, 1967. 1
by a laparotomy on April 9. A recurrent ileocecal intussusception with evisceration of the intus-suscepted bowel through the proximal stoma of the colostomy on May 10 required surgical correction and an attempt had been made to affix the right colon to the right gutter. \Vhen first seen by us the boy had urinary incontinence. A Cunningham clamp had been used temporarily but had to be discontinued because of a penile pressure sore. The patient had a vague sensation of fullness when the bladder was filled and emptied some urine by forceful abdominal straining. His mother had to go to school 3 times in the morning and afternoon to change the boy's diapers. The patient appeared to be a bright young boy of normal appearance from the waist up. However, he sat in the typical Buddha position with the atrophic apvendages of his lower legs folded under him, using the feet as a cushion. He had popliteal webs, a large thoracic diameter discrepant from a small pelvic diameter and kyphosis of the thoracic spine (fig. 1, A). He had bilateral varalytic talipes equinovarus and a double barrel right upper quadrant colostomy. The penis was small with meatal stenosis which was corrected by meatotomy. Good renal function was noted on excretory urography, although malrotation of the right kidney was present (fig. 1, B). Cystoscopy was impeded by bladder spasticity during filling. No reflux was seen with delayed cystography. Following a suprapubic cystostomy on December 9, 1957 the patient continued to have leakage through the urethra. Because of this leakage the vesical neck was closed on June 13, 1958. However, even a Robinson catheter secured by a shield was extruded as was the Foley bag before the Robinson was used. Urinary infection was controlled with chemotherapy. A small bowel intussusception developed on February 28, 1959 which was corrected surgically. At this time the double barrel colostomy was converted to a terminal single loop colostomy
374
COXGEKI'l'AL ABSENCE OF LUMBOSACRAL 8PlNE
F1G, 1 A, x-ra 0- of spine revealR normal nrnnber of thoracic vertebrae, H, excretory nroµ;rarn n t, :ig:e
FIG,
2, Location of stornas
and :3 to 4 inches ol' Lninsver~c colon were resected diRtall:r to tlrn colo~torny, For the past 8 seam the colostomy has worked welL To soln' the urinary difficulties resulti1,1g from the unsatisfactory function of the SUJJrnpubic rathctN, a sigrnoiclocystoplasty was done on December 7, 1959, The anastomosis 1nts in isoperistaltic direction and the stoma was placed in the left lower abdominal quadrant, Therethe patient has two stomas, one on the right for feces and one on the left for urine (fig, 2), Special appliances lmd to be made because of
the 1ia1Tcm- illtcrnpacc beb-cen the rib cage a11d the peh-is. The skin smrouuding the left colm,, tomy was revised on January 28, J 963 heca11s" of kerntosis, Since then tbere have been no ('011, plaints rC'gardi11g either colostomy, On :\foy 12, 1966 an ice water test \\'as 11eµ;a tin!, Xo reflux was found 011 ry~togrnphy; howeyer, there was some narrowness of i,he aw.1stomotic site between the bladder and the; colonit: conduit (fig, 3, A_), An excretory urogram 011 September 15, 1967 shm,-ecl no essential frnm previous findings (fig, 3, B) The hlo()(l meu,
376
PEARLMAN AND BORS
FIG. 3 .•4-, colocystograrn at age 18. B, excretory urograrn at age 18
nitrogen (BUN) was 11.5 mg. per cent and creati:nine was 0.7 mg. per cent. The penis was 4.3 cm. in length and 2 cm. in width. The testes ,vere still small, although they had increased in size. The patient had pubic hair; he hud no erections. Neurological examination on December 14 showed cranial nerves and reflex activity of the upper extremities within physiological limits and the lower extremities completely flaccid, without deep tendon reflex responses and without pathologic toe signs. They were contracted at the hips and knees. Sensution of touch was preserved throughout the body, including the lower extremities, saddle area and genitalia. Deep pressure ,;rns perceived but was not painful at the toes and heelcords. There seemed to be little, if any, kinesthesia in the toes. Vibrator.1' sensation was retained in the entire body ·when it ,ms tested by bone conduction but was absent below Ll ,vhen it was tested in the skin. There was analgesia below Ll. Rectal examination was interpreted as painful, although the external anal sphincter ,ms flaccid and the bulbocavernosus and cough reflexes could not be elicited. DISCUSSION
Of the 13 patients with agenesis of the spine below Tll, Tl2, Ll and L2, 1- 4 9 \\-ere boys, 2
were girls and the sex of two was not mentioned. Of the 9 boys, five were living at 9 months, 8, 9, 11 and 15}~ years and 1 girl was alive 8 years; to this we add our patient with agenesis below Tl2 as the sixth surviving male patient, now 18 years of age. Of the 2 girls with agenesis below Ll, one survived for 63 hours and the other is alive at 8 years. Two children of unreported sex survived for 9 months and 3 years. The survival rate is better when agenesis of the spine occurs at lower levels, L4 rtnd below, as is reflected in the 8 cases of Blumel and associates (3 boys and 5 girls, all survivecl). 3 The etiology is unknown, rtlthough dirtbetes of the mother seems to play a role. Insulin injections into the chick embryo produced vertebral changes when done within the first 2 clays of incubation. 6 Malformation of the spine produced in rabbits by insulill could be counteracted by riboflavin. 4 The clinical picture which we observed cor-· responded with the description in the literature. Characteristic mermaid or siren deformity of the legs was present which produces a Buddha or frog-like sitting position. The ribs left the vertebrae at right angle (90 degree) instead of the acute angle with a caudad 6 Duraiswami, P. K.: Insulin-induced skeletal abnormalities in developing chickens. Brit. Med. J., 2: 384--390, 1950.
CONGENITAL ABSENCE OF LUMBOSACRAL SPINE
course. A similar difference of angulation is found in nerve roots of patients with a short filum terminale or with a tethered spinal cord syndrome. Occasionally agenesis of the spine is associated with a meningomyelocele but this was not the case in our patient. Neurologically, the discrepancy of motor versus sensory deficit was of interest which resembled the picture of an anterior cord syndrome with chief involvement of the lateral columns, i.e. motor paralysis and analgesia but preservation of other modalities, with one important exception, namely, the total flaccidity of paralysis. How much this is caused by the proximity of a nutrient artery which branches off the aorta and enters the spinal canal between the 11th and 12th thoracic vertebrae is merely conjectural. This difference in motor and sensory innervation has been described. With a lesion at this level visceral deficit should be expected. This included bladder, bowel and sexual function in our patient, who showed signs of a lower motor neuron lesion not too different from that of patients with traumatic conus terminalis and/or cauda equina lesions. Such patients empty their bladders by strain and only about 25 per cent of them retain exclusively psychic erections.7 In cases of low (sacral) spinal malformation upper motor neuron lesions are not infrequent. Was the first colostomy really necessary in our patient or could it have been avoided by proper management, as done in patients with spinal cord lesions from trauma or disease? Bowel dysfunction has not been the indication for colostomy in any of the 2,300 traumatic paraplegics at the Long Beach Veterans Hospital. Neither has any of the more than 100 patients with myelodysplasia seen in consultation at the Orange County Medical Center, Harbor County Hospital and the Childrens Hospital had a colostomy. Had this patient been managed without the primary colostomy he would have been not only spared the many interventions which entailed from complications, but the operating surgeon would not have been saddled with the dilemma of what diversional procedure to use. The ileal bladder is presently fashionable as the method 7 Bors, E. and Comarr, A. E.: Neurological disturbances of sexual function with special reference to 529 patients with spinal cord injury. Urol. Survey, 10: 191-222, 1960.
377
of choice in uncontrollable urinary incontinence caused by myelodysplasia, especially in female subjects. This operation was considered for our patient but rejected because of two difficulties: 1) the presence of adhesions caused by several former laparotomies and 2) the location of the stoma of the colostomy in a place where an ileal bladder is usually placed. This then logically led to the decision to use the defunctionalized colon for the cystosigmoidostomy. This procedure had the advantage of preserving the competence of the vesicoureteral junction, preventing reflux and renal infection to which congenitally malformed kidneys are known to be prone. Our operation proved to be a success but it does not pretend to be the method of choice for patients with this affliction, when they are managed properly (i.e. conservatively) in regard to their bowel dysfunction. It is a moot question as to the outcome had a conservative route been taken; but for the sake of professional discussion let us consider a few points of a therapeutic approach which we would choose today if we were presented with such a patient without colostomy in early life. The first would be bowel regulation with bulkforming substances, lubrication of the intestines and stimulation of the defecation reflex with suppositories or enemas of glycerin, mineral oil and water in equal parts. The urinary incontinence would have been treated with bladder hygiene, i.e. regularly spaced intervals of drinking a set amount of fluid and by the attempt to empty the bladder by either Crede pressure or by abdominal straining, since the innervation of the abdominal musculature was intact prior to colostomy. Incontinence, accompanying myelodysplasia or its allied conditions of a short filum terminale or spinal malformations, is more amenable to conservative therapy in boys than girls since a useful female urinal has yet to be invented. The shortness of a penis can be corrected by plastic methods so that a condom urinal or its equivalent in children, a finger cot, can be applied.8 Cunningham clamps must be padded with cotton or thin tissue paper and frequently opened if decubitus is to be avoided; these appliances are used only exceptionally today in order to correct the incontinence of neurologic etiology with absent penile sensation. 8 Bors, E.: Scrotal skin plasty to elongate the penis. J. Urol., 87: 419--421, 1962.
378
PEARLMAN AND BORS
The case herein reported shows what is wellknown today, that suprapubic cystostomy will not keep a patient who has a lower motor neuron lesion dry. The much maligned urethral catheter is preferable, especially when applied in its whistle-tip form and properly managed (changed frequently). Foley bag catheters will irritate the bladder causing small segmental, uncoordinated contractions which can be reduced, however, by banthine and/or atropine. We have failed to apply these drugs or topical anesthesia to contain this type of bladder response to the suprapubic catheter. By virtue of entering the viscus through normally innervated structures the catheter was, perhaps, more irritating than a urethral catheter, which would have traversed denervated, although incompletely deafferentated, parts of the body. The ultimate goal, without the primary colostomy, would have been the same as the one finally achieved, namely, to render the patient free of a catheter and of urinary infection. There
can be no doubt that the primary colostomy interfered with the abdominal straining power or Crede pressure, essential for bladder rehabilitation in patients with lower motor neuron lesions. Therefore, our case shows the complicating events which can be started by one wrong indication and, also, that utilization of the defunctionalized sigmoid for cystocolostomy can be useful under these extraordinary circumstances. SUMMARY
A case of congenital absence of the lumbosacral spine is presented. This is the fourteenth case of this severe malformation reported in the literature. Our patient is now 18 years old; he has been followed for 10 years. Urinary incontinence was eventually solved by surgical closure of the vesical neck and a colocystostomy. The literature is reviewed and the general management of this type of myelodysplasia is discussed with regard to bowel and bladder function.