Surgical and endoscopic treatment of bladder exstrophy-epispadias complex in a female dog

Brittani Jones Department of Surgery, the Animal Medical Center, 510 E 62nd St, New York, NY 10065.

Search for other papers by Brittani Jones in
Current site
Google Scholar
PubMed
Close
 DVM
,
Allyson C. Berent Department of Interventional Radiology, the Animal Medical Center, 510 E 62nd St, New York, NY 10065.

Search for other papers by Allyson C. Berent in
Current site
Google Scholar
PubMed
Close
 DVM
,
Chick W. Weisse Department of Interventional Radiology, the Animal Medical Center, 510 E 62nd St, New York, NY 10065.

Search for other papers by Chick W. Weisse in
Current site
Google Scholar
PubMed
Close
 VMD
,
Robert Hart Department of Surgery, the Animal Medical Center, 510 E 62nd St, New York, NY 10065.

Search for other papers by Robert Hart in
Current site
Google Scholar
PubMed
Close
 DVM
,
Leilani Alvarez Department of Rehabilitation, the Animal Medical Center, 510 E 62nd St, New York, NY 10065.

Search for other papers by Leilani Alvarez in
Current site
Google Scholar
PubMed
Close
 DVM
,
Anthony Fischetti Department of Diagnostic Imaging, the Animal Medical Center, 510 E 62nd St, New York, NY 10065.

Search for other papers by Anthony Fischetti in
Current site
Google Scholar
PubMed
Close
 DVM
,
B. David Horn Division of Orthopedics, Department of Surgery, Children's Hospital of Philadelphia, 3401 Civic Center Blvd, Philadelphia, PA 19104.

Search for other papers by B. David Horn in
Current site
Google Scholar
PubMed
Close
 MD
, and
Douglas Canning Division of Urology, Department of Surgery, Children's Hospital of Philadelphia, 3401 Civic Center Blvd, Philadelphia, PA 19104.

Search for other papers by Douglas Canning in
Current site
Google Scholar
PubMed
Close
 MD

Click on author name to view affiliation information

Abstract

CASE DESCRIPTION A 14-week-old 7.7-kg (16.9-lb) sexually intact female Golden Retriever was evaluated because of urine dripping from the caudoventral aspect of the abdomen.

CLINICAL FINDINGS Ultrasonography, radiography, excretory CT urography, and vaginocystourethroscopy were performed. Results indicated eversion of the bladder through the ventral abdominal wall with exposure of the ureterovesicular junctions, pubic diastasis, and an open vulva and clitoral fossa. Clinical findings were suggestive of bladder exstrophy, a rare congenital anomaly.

TREATMENT AND OUTCOME The dog was anesthetized and bilateral ileal osteotomies were performed. Two ureteral catheters were passed retrograde into the renal pelves under fluoroscopic guidance. The lateral margins of the bladder, bladder neck, and urethra were surgically separated from the abdominal wall, and the bladder was closed, forming a hollow viscus. The symphysis pubis was closed on midline with horizontal mattress sutures. The defects in the vestibule and clitoral fossa were closed. Lastly, the iliac osteotomies were stabilized. The dog was initially incontinent with right hind limb sciatic neuropraxia and developed pyelonephritis. Over time, the dog became continent with full return to orthopedic and neurologic function, but had recurrent urinary tract infections, developed renal azotemia likely associated with chronic pyelonephritis, and ultimately was euthanized 3.5 years after surgery because of end-stage kidney disease.

CLINICAL RELEVANCE Bladder exstrophy and epispadias is a treatable but rare congenital abnormality. The procedure described could be considered for treatment of this condition, but care should be taken to monitor for urinary tract infections and ascending pyelonephritis.

Abstract

CASE DESCRIPTION A 14-week-old 7.7-kg (16.9-lb) sexually intact female Golden Retriever was evaluated because of urine dripping from the caudoventral aspect of the abdomen.

CLINICAL FINDINGS Ultrasonography, radiography, excretory CT urography, and vaginocystourethroscopy were performed. Results indicated eversion of the bladder through the ventral abdominal wall with exposure of the ureterovesicular junctions, pubic diastasis, and an open vulva and clitoral fossa. Clinical findings were suggestive of bladder exstrophy, a rare congenital anomaly.

TREATMENT AND OUTCOME The dog was anesthetized and bilateral ileal osteotomies were performed. Two ureteral catheters were passed retrograde into the renal pelves under fluoroscopic guidance. The lateral margins of the bladder, bladder neck, and urethra were surgically separated from the abdominal wall, and the bladder was closed, forming a hollow viscus. The symphysis pubis was closed on midline with horizontal mattress sutures. The defects in the vestibule and clitoral fossa were closed. Lastly, the iliac osteotomies were stabilized. The dog was initially incontinent with right hind limb sciatic neuropraxia and developed pyelonephritis. Over time, the dog became continent with full return to orthopedic and neurologic function, but had recurrent urinary tract infections, developed renal azotemia likely associated with chronic pyelonephritis, and ultimately was euthanized 3.5 years after surgery because of end-stage kidney disease.

CLINICAL RELEVANCE Bladder exstrophy and epispadias is a treatable but rare congenital abnormality. The procedure described could be considered for treatment of this condition, but care should be taken to monitor for urinary tract infections and ascending pyelonephritis.

A 14-week-old 7.7-kg (16.9-lb) sexually intact female Golden Retriever was referred to the Animal Medical Center for evaluation of incontinence and urine dripping from the caudoventral aspect of the abdomen since birth. Initially, a cyst-like structure had been noticed in the umbilical region, but at approximately 10 weeks of age, this cyst-like structure reportedly ruptured and began leaking urine. The dog had also had an abnormal gait since birth, described as a “bunny hopping” type of gait with abduction of the hip joints. The dog was otherwise considered healthy.

On physical examination, the dog (body condition score, 3/5) had evidence of bladder exstrophy, with the intraluminal surface (mucosa) of the bladder visible along the ventral midline of the abdomen. Both UVJs could be seen just cranial to the urethra, with jets of urine emptying onto the ventral abdominal wall (Figure 1). The patient's linea alba was split from the umbilicus caudally to the level of the pubis, where the mucosa of the dorsal bladder wall was adhered to the proximal portion of the urethra. The urethra was patent and was a closed cylindrical tube. The clitoral fossa and vestibule were open, exposing the clitoris, vestibule, and vaginal opening. Both hind limbs were also abducted, and there was no palpable connection between the left and right pubic bones. The remainder of the dog's physical examination findings were unremarkable. A CBC revealed normocytic, hypochromic, nonregenerative anemia (Hct, 29.7% [reference range, 37% to 55%]; reticulocytes, 87,000 reticulocytes/μL [reference range, 10,000 to 110,000 reticulocytes/μL]). The only serum biochemical abnormality was hyperphosphatemia (8.6 mg/dL; reference range, 2.1 to 6.3 mg/dL).

Figure 1—
Figure 1—

Preoperative images of a 14-week-old 7.7-kg (16.9-lb) sexually intact female Golden Retriever evaluated because of urine dripping from the ventral aspect of the abdomen. A—Examination of the ventral aspect of the abdomen revealed bladder exstrophy, with the intraluminal (mucosal) surface of the bladder visible along the ventral midline and exposure of the ureteral openings (yellow arrows) and bladder neck (red arrow) and open vestibule (black arrow) and clitoral fossa (orange arrow). B—Insertion of an endoscope indicated that the urethra was patent. C—On a lateral radiographic image, there is no evidence of a bladder within the pelvis. D—On a ventrodorsal radiographic image, the pubic bones and ischia are malformed and do not converge on the midline. Less than 50% coverage of the femoral heads by the acetabula is present bilaterally, but secondary arthritic changes are not apparent. E—Computed tomographic reconstruction illustrating the pelvic malformation and pubic diastasis. F—Lateral, postcontrast CT image obtained during excretory urography illustrating the left ureter (red arrow) as it exits onto the ventral body wall with accumulation of contrast medium on the ventral body wall (yellow arrow). The right ureter ended in a similar fashion.

Citation: Journal of the American Veterinary Medical Association 252, 6; 10.2460/javma.252.6.732

Transabdominal ultrasonography showed mild bilateral renal pelvic dilation (renal pelvis diameter, 2.5 to 4.0 mm) with bilateral hydroureter (diameter, 4 mm) to the level of the UVJs. The architecture of the kidneys was otherwise normal. Ultrasonographic imaging of the ventral body wall and urinary bladder indicated that the rectus abdominus muscles were normally located on the midline near the xiphoid process, but that the linea alba widened caudally. Caudal to the umbilicus, the linea alba had a focus of hypoechoic material believed to represent urothelium opposed to skin. The exposed mucosa extended caudally. The ureters ended in the exposed mucosa superficially at this level. The trigone of the bladder was followed to a thick-walled urethra that appeared to be a complete tube. Shadowing representing the floor of the bony pelvis was not seen. The urethra extended to the perineum. The uterus could not be identified. The overall interpretation of abdominal ultrasonographic findings was that there was a caudal abdominal wall malformation with external exposure of the urinary bladder and UVJs consistent with an exstrophy variant.

Pelvic radiographs were obtained and demonstrated a malformed pubic symphysis and ischia, with failure of the ischia to converge on midline. Radiographic findings were consistent with pubic diastasis, defined as separation of the pubic symphysis without fracture. There was < 50% coverage of the femoral head by the acetabula bilaterally

Computed tomography with double-contrast excretory urography (0.63-mm and 2.5-mm slice thicknesses) showed mildly asymmetric dilation of the ureters, which was especially pronounced distally as the ureters opened onto the ventral body wall. There was contrast accumulation on the ventral external body wall, and no urinary bladder was identified (Figure 1).

Vaginocystourethroscopy was performed (Figure 1) with a 2.7-mm integrated 30° rigid cystoscopea to help clarify the urogenital anatomy. This showed an intact urethra that measured 4 cm in length, a persistent paramesonephric remnant with an intact vagina, and an open vestibule and clitoral fossa resulting from a lack of ventral midline skin fusion at the vulva. Most notable was exposure of the intraluminal surface of the dorsal aspect of the bladder through the body wall because of failure of the ventral surface of the bladder to create a bladder luminal structure. Instead, the bladder body was fused to the ventral abdominal wall at the linea alba. In view of these findings, the diagnosis was bladder exstrophy with epispadias. No specific treatments were offered at this time, while potential options for bladder and pelvis reconstruction were investigated.

Approximately 3.5 months later, when the dog was 28 weeks old, surgery was performed to reconstruct the urinary and orthopedic defects. The treatment team included a pediatric urologist (DC), a veterinary internist with expertise in interventional endoscopy and endourology (ACB), a veterinary surgeon with expertise in soft tissue surgery and interventional radiology (CWW), a pediatric orthopedic surgeon (BDH), and a veterinary surgeon with expertise in orthopedic surgery (RH).

The dog was premedicated with oxymorphone (0.1 mg/kg [0.045 mg/lb], IM), maropitant citrate (1 mg/kg [0.45 mg/lb], IM), and glycopyrrolate (0.01 mg/kg [0.0045 mg/lb], IM). Anesthesia was induced with propofol (3 mg/kg [1.4 mg/lb], IV) and midazolam (0.5 mg/kg [0.23 mg/lb], IV). Anesthesia was maintained with isoflurane in oxygen delivered via an endotracheal tube. A lumbosacral epidural injection of ketamine (0.19 mg/kg [0.09 mg/lb]), dexmetomidine (0.1 mg/kg), morphine (0.05 mg/kg [0.023 mg/lb]), and bupivacaine (0.29 mg/kg [0.13 mg/lb]) was given preoperatively in the subarachnoid space. A fentanyl CRI (0.2 to 0.5 μg/kg/min [0.09 to 0.23 μg/lb/min], IV) was administered throughout surgery. Ampicillin sodium-sulbactam sodium (22 mg/kg [10 mg/lb], IV) was given at the time of anesthetic induction and then every 90 minutes during anesthesia. Hair was clipped from the level of the xiphoid caudally, including both hip regions, the entire ventral surface of the abdomen, and both hind limbs. The ventral aspect of the abdomen, both hip regions, the proximal aspects of the hind limbs, and the clitoral fossa and vestibule were aseptically prepared, and both hind limbs were secured in a hanging position to maintain sterility.

With the patient in dorsal recumbency, a 2.7-mm integrated 30° rigid cystoscope was advanced into the vestibule. There was a dorsoventral, vestibulovaginal remnant present that covered 75% of the vaginal opening, consistent with a persistent paramesonephric remnant. The urethral opening appeared dorsoventrally flattened and wider than usual. The urethra itself appeared grossly normal, measured approximately 5 cm in length, and emerged ventrally into the exstrophic bladder. Ureteral openings were noted approximately 1 cm cranial to the urethrovesicular junction. The left UVJ was positioned slightly more cranially than the right. A suspected extension, or flap, of bladder mucosa was noted to the right and caudal to the primary bladder region.

The dog was repositioned in right lateral recumbency, and a lateral approach to the left ilium was performed. The midportion of the ilium was thickened and malformed. Hohmann retractors were placed medial to the ilium to protect the sciatic nerve and other underlying structures. A linear iliac osteotomy was made perpendicular to the ilio-ischial axis of the pelvis. The site was packed with sterile sponges soaked with saline (0.9% NaCl) solution, and the skin was stapled. The patient was repositioned in left lateral recumbency, and the identical procedure was performed on the right side.

The patient was then rotated back into dorsal recumbency. A 5F red rubber catheter was passed retrograde into each UVJ, and retrograde ureteropyelography was performed with fluoroscopic guidance.b Each catheter was advanced into its respective renal pelvis over a 0.035-inch hydrophilic angle-tipped guidewire.c Next, the guidewire was passed antegrade from the trigone, into the urethra and out the vulva. The tip of each ureteral red rubber catheter was cut off, and the remaining ends of the red rubber catheters were advanced in the same path over the guidewire to maintain urethral patency and cannulation. The guidewire was removed. The red rubber catheters were then sutured to the external aspect of the vulva with 3–0 polyamide in a finger-trap pattern and to the bladder mucosa at the level of the UVJ with 3–0 chromic gut in a finger-trap pattern. Both catheters were observed to be draining urine from the renal pelves.

The lateral margins of the bladder urothelium were marked with a sterile marking pen, including the previously identified right-sided flap of additional bladder mucosa (Figure 2). An incision was made through the linea alba approximately 3 cm cranial to the externalized bladder apex and then continued around the rim of the bladder mucosa at the junction of the mucosa and skin. The detrusor muscle was identified deep to the transitional epithelium. The bladder was dissected from surrounding fascia with a combination of blunt and sharp dissection extending from the cranial margin on the left and right to the perivesical fascial attachments to the pubic symphysis on each side. A 5F nonlocking-loop pigtail catheterd was then placed in the urinary bladder through the urethral lumen and secured to the vulva by means of an antegrade catheterization approach, over a 0.035-inch angle-tipped hydrophilic guidewire. This catheter was secured to the vulva with 3–0 polyamide in a finger-trap pattern and to the bladder mucosa with 3–0 chromic gut. The bladder edges were deeply mobilized proximally and distally to provide placement of the bladder neck deep within the reconstructed pelvic canal. The bladder and bladder neck were then closed in a full-thickness simple interrupted pattern with 4–0 poliglecaprone 25 suture. Once the entire bladder wall was closed, it was leak tested with the pigtail urethral catheter. The reproductive tract was identified and appeared normal. Through a ventral midline laparotomy, routine ovariectomy was performed.

Figure 2—
Figure 2—

Intraoperative and postoperative images of the patient in Figure 1. A—Bladder mucosa (black arrow) has been traced with a marking pen, including a right extension flap of mucosa (yellow arrow). Ureteral catheters are in place (white arrows). The dog's head is to the top of the picture. B—Approximation of the margins of the mobilized pubic symphysis with size 1 polydioxanone (yellow arrow) placed through the symphyseal cartilage in a horizontal mattress pattern. C—Appearance of the body wall and vulva following closure. A 5F nonlocking loop pigtail urethral catheter (yellow arrow) is seen exiting the vulva along with the ureteral catheters (white arrows). Notice that the bladder is no longer exposed through body wall. D—Immediate postoperative ventrodorsal radiographic view showing closer approximation of the ischia (yellow arrow), appropriate positioning of lateral and ventral cortical iliac plates bilaterally, and the urethral catheter. E—Lateral radiographic image obtained 13 days after surgery showing the bilateral ureteral stents (black arrows), indwelling urethral catheter (cut to the length of the patient's urethra; yellow arrow), and appropriate positioning of the iliac plates.

Citation: Journal of the American Veterinary Medical Association 252, 6; 10.2460/javma.252.6.732

Next, the margins of the mobilized pubic symphysis were reduced, although an approximately 1.5-cm gap remained, and held in place with 3 horizontal mattress sutures of size 1 polydioxanone in the symphyseal cartilage and periosteum (Figure 2).

A closed suction draine was placed in the abdomen to monitor for urine leakage postoperatively. The linea alba and reconstructed midline were closed with size 0 polydioxanone in a continuous suture pattern, ensuring that the urethra was dorsal to the pubic sutures apposing the pubic symphysis. The ventral vestibular defect was closed over the clitoral fossa and overlying vulva with 3–0 poliglecaprone 25 in a simple interrupted pattern in 2 layers. The subcutaneous tissues were closed with 2–0 polydioxanone in a continuous subcutaneous pattern. Intradermal tissues were closed with 3–0 poliglecaprone 25 in a continuous subcuticular pattern. The skin was apposed with 3–0 polyamide in an interrupted cruciate pattern (Figure 2).

The patient was then repositioned in right lateral recumbency, and the previous surgery site was reopened. The distal iliac fragment, which had translated medially during mobilization of the pubic symphysis, was partially reduced, achieving 20% to 30% apposition of bone at the osteotomy. A 7-hole 2.7-mm locking compression platef was contoured and applied to the lateral cortex of the ilium with a combination of cortical and locking screws. A second 5-hole 2.7-mm locking compression platef was contoured and applied to the ventral cortex of the ilium with a combination of cortical and locking screws. The musculature was opposed with 2–0 polydioxanone in a simple continuous pattern. The subcutaneous and intradermal layers were closed with 2–0 polydioxanone in a simple continuous pattern. The skin was apposed with 3–0 polyamide in a simple interrupted pattern. The patient was positioned in left lateral recumbency, and the identical procedure was performed on the right ilium. Postoperative radiography of the abdomen and pelvis (Figure 2) confirmed successful placement of ureteral catheters and pelvic bone plates. Postoperatively, the patient received CRIs of fentanyl (2 μg/kg/h [0.9 μg/lb/h], IV), lidocaine (20 μg/kg/min [9.1 μg/lb/min], IV), and ketamine (0.2 mg/kg/h [0.09 mg/lb/h], IV) along with cefazolin (22 mg/kg, IV, q 12 h), pantoprazole (1 mg/kg, IV, q 24 h), and maintenance isotonic fluids. The patient was hospitalized for 14 days for postoperative care, monitoring (renal panel, CBC, serum biochemical profile, abdominal ultrasonography, and radiography), and daily physical rehabilitation.

Twenty-four hours after surgery, a fentanyl patch (25 μg/h) was applied. Carprofen (1.7 mg/kg [0.77 mg/lb], PO, q 12 h) was added to the treatment regimen, and the fentanyl, lidocaine, and ketamine CRIs were decreased 12 hours later. During this time, the patient was laterally recumbent and nonambulatory in both hind limbs. The urethral catheter was producing urine and the closed suction drain was producing 0.3 to 0.5 mL/kg/h (0.14 to 0.23 mL/lb/h) throughout the day. The second day after surgery, abdominal ultrasonography was performed, which showed no evidence of progressive renal pelvic dilation, the presence of 2 ureteral catheters with mild ureteral dilation around the catheters, and an empty urinary bladder within the abdomen with a urinary bladder catheter in place. Three days after surgery, the left hind limb was neurologically normal and weight-bearing. Lateral toe sensation and a withdrawal reflex were absent in the right hind limb, and sciatic nerve dysfunction was diagnosed, likely associated with neuropraxia secondary to surgical inflammation. Rehabilitation was aggressively pursued during hospitalization and continued at home. During the immediate postoperative period, rehabilitation was performed twice daily for the first 7 days and consisted of passive range-of-motion exercises for the stifle and tarsal joints bilaterally (5 repetitions with a 2-second hold for each joint); recumbent gentle joint approximations for the tarsal, stifle, and hip joints (2 sets of 10 repetitions for each joint); and massage (10-minute duration focusing primarily on edematous regions of the hind limbs). This period of rehabilitation also included once-daily therapeutic laser therapyg,h (4 J/cm2) applied to the edematous tarsal regions and tail base and to the surgical incisions (2 J/cm2). Class IV laser therapyi (6 W; total, 492 J) was also performed once daily for 3 consecutive days in the ventral abdominal region and was then continued twice weekly until discharge. The laser therapy duration varied from 1 to 3 min/site depending on the size of the area treated and power of the laser.

Forty-eight hours after surgery, the fentanyl, lidocaine, and ketamine CRIs were discontinued, and treatment with methadone (0.1 mg/kg, IV, q 4 to 6 h as needed) was started. Seventy-two hours after surgery, the patient was switched from injectable to oral medications. These included cephalexin monohydrate (23 mg/kg [10 mg/lb], PO, q 12 h) and tramadol (2.3 mg/kg [1.0 mg/lb], PO, q 8 h). The fentanyl patch was replaced, and carprofen administration was continued as previously prescribed. Treatment with miconazole lotion was also started because of development of yeast otitis externa, diagnosed on the basis of cytologic examination of swab specimens. On day 7, the fentanyl patch was removed. Weight-bearing exercises were initiated and included assisted standing (isometric hold for 10 to 15 seconds as tolerated up to 3 times), neurosensory stimulation to the paws of the hind limbs with foot-to-floor exercises (3 sets of 10 repetitions for each hind limb), standing weight shifts and rhythmic stabilizations (3 sets of 5 pulses to each side), joint approximations (2 sets of 10 repetitions for each joint), and assisted walking with harness support (2 sets of 5 minutes with a 1- to 2-minute rest session between sets). In addition, dry-needle acupuncture and electroacupuncture were performed twice weekly (20-minute sessions). On day 8, both ureteral catheters were removed, and the following day, the urethral catheter was removed. Aerobic culture of a swab specimen from the patient's vestibule yielded Pseudomonas aeruginosa, and treatment with marbofloxacin (4.6 mg/kg [2.1 mg/lb], PO, q 24 h) was initiated.

On day 10, the patient developed a fever (39.7°C [103.4°F]). Focused ultrasonography of the urinary tract showed severe bilateral hydroureter (right, 0.6 to 1.0 cm; left, 0.5 to 1.5 cm), moderate hydronephrosis bilaterally (transverse measurement: right kidney, 1.5 cm; left kidney, 1.5 cm), a ureterocele (2.3 × 3.3 × 3.5 cm) at the UVJ, and focal narrowing of the left ureter with 5 to 7 tiny hyperechoic foci in the region of the wall surrounded by hyerpechoic fat. A CBC revealed band neutrophila (absolute neutrophil count, 19,635 neutrophils/μL [reference range, 3,000 to 11,500 neutrophils/μL]; band neutrophil count, 924 band neutrophils/μL [reference range, 0 to 300 band neutrophils/μL]) and nonregenerative anemia (Hct, 30.3%; absolute reticulocytes, 82,000 reticulocytes/μL). Serum biochemical analyses showed mild azotemia (BUN, 35 mg/dL [reference range, 7 to 27 mg/dL]; creatinine, 1.9 mg/dL [reference range, 0.4 to 1.8 mg/dL]) and persistent hyperphosphatemia (8.8 mg/dL). Because of concerns that swelling of the urinary bladder might be causing obstruction of both UVJs, a urinary bladder catheter was placed with a closed-collection system. The following day, bilateral, 22-cm-long, 4.7F double ureteral stentsj were placed with endoscopic and fluoroscopic guidance, as described.1 A temporary indwelling Foley urethral catheter cut to the length of the urethra was placed to maintain urethral patency and sutured to the vestibule to maintain it in place. The balloon was not inflated. Within 12 hours, the fever and azotemia resolved (BUN, 27 mg/dL; creatinine, 1.4 mg/dL). Follow-up focused ultrasonography of the urinary tract 2 days after ureteral stent replacement showed correct placement of the ureteral stents, improvement in the renal pelvic dilation bilaterally to baseline values (transverse measurement: left kidney, 0.25 cm; right kidney, 0.4 cm), and minimal ureteral dilation. The patient was maintained on marbofloxacin (4.6 mg/kg, PO, q 24 h) and cephalexin monohydrate (23 mg/kg, PO, q 8 h). On day 13, pelvic radiographs were obtained to evaluate alignment of the iliac bone plates and pelvis because of improved, yet persistent, right-sided neuropraxia. Radiographs showed good alignment of the pelvis.

The patient was discharged 14 days after surgery; treatment at that time consisted of marbofloxacin (4.6 mg/kg, PO, q 24 h) for 5 days, miconazole nitrate (5 drops to both ears, q 12 h) for otitis externa, cephalexin monohydrate (23 mg/kg, PO, q 8 h) for 14 days because of superficial pyoderma, and a hydrogel wound care spray for application to the abdominal incision every 8 hours. The urethral catheter was left in place with the cut end exiting the urethra in the vestibule. This allowed the dog to remain incontinent and maintain urinary bladder decompression. Continued rehabilitation and exercise restriction for the next 6 to 8 weeks were recommended. Home exercises included twice daily passive range-of-motion exercises for both hind limbs; joint approximations for the tarsal, stifle, and hip joints; sensory paw stimulation; foot-to-floor exercises; assisted standing; and assisted walking. The dog was maintained in a urinary diaper to keep the vulva clean and covered. Dilute chlorhexidine solution was used at home twice daily to clean the vulva and vestibule by syringe infusion into the vestibule. The owner was instructed to have the patient's abdominal sutures removed in 2 days, have renal biochemical parameters assessed in 1 and 2 weeks, have a urine sample collected for bacterial culture in 2 and 6 weeks, and have abdominal ultrasonography repeated in 2 and 6 weeks. Additionally, follow-up pelvic radiographs were obtained at 6 weeks, at which time the urethral catheter was removed.

On a recheck examination 6 weeks after surgery (4 weeks after discharge), the patient was ambulatory in all 4 limbs but still knuckling in the right hind limb. Left tarsal swelling was present. Superficial pain was absent but deep pain sensation was present in the lateral aspect of the right hind limb. The patient also had restricted hip joint extension bilaterally with mild discomfort during extension. Administration of vitamin E (18.4 U/kg [8.36 U/lb], PO, q 24 h) was recommended to help promote repair of the sciatic nerve damage. The patient was fitted with a custom bootk for the right hind limb to assist with dorsiflexion and prevent knuckling. A urine specimen was obtained by means of cystocentesis and submitted for bacterial culture, which yielded Eshcherichia coli and an Enterococcus sp. A brief ultrasound examination revealed moderate hydronephrosis bilaterally (left renal pelvis, 1.5 cm; right renal pelvis, 1.2 cm). No hydroureter was noted, and both ureteral stents were properly positioned. An abdominal wall hernia was noted and described as a 5- to 8-cm-long band of thick fibrous tissue on the ventral midline in the normal location of the linea alba (along the surgical incision). This band of tissue was separating both rectus abdominal muscles and the subcutaneous tissue from the abdominal cavity. No entrapment of organs or fat was noted.

Seven weeks after surgery, the local veterinarian removed the patient's indwelling urethral catheter. The patient was subsequently reported to be posturing to urinate with a small stream of urine being produced but was persistently incontinent with a continence score of 2 of 10, on the basis of a previously reported scoring system.2 Eight weeks after surgery, the patient underwent pelvic radiography, abdominal ultrasonography, cystoscopy, and bilateral ureteral stent exchange. The dog was persistently incontinent. The owner reported that the right sciatic nerve function was static, with continued signs of neuropraxia. Pelvic radiography showed that the osteotomy sites were healing well. Focused ultrasonography of the urinary tract showed improved bilateral pyelectasia (right, 0.7 cm; left, 0.4 cm), with mildly thickened ureteral walls bilaterally and a urinary bladder with a slightly irregular wall. Abdominal radiography showed that the ureteral stents were in an appropriate location. Cystoscopy revealed a persistent paramesonephric remnant, short urethra, and small bladder. A white film was noted coating the mucosa of the bladder, and mucopuruluent debris was seen in the bladder. Owing to development of a resistant urinary tract infection and concern for possible biofilm formation on the ureteral stents, both ureteral stents were exchanged under cystoscopic and fluoroscopic guidance. The debris was also removed from the bladder. A urine sample submitted for bacterial culture yielded P aeruginosa and E coli. Sulfadimethoxine-ormetoprim (27.5 mg/kg [12.5 mg/lb], PO, q 12 h for 14 days) was prescribed on the basis of results of susceptibility testing.

Physical examination and bacterial culture of a urine sample were repeated 15 weeks after surgery and showed persistent Enterococcus infection. Because the patient was not having clinical signs and there was no evidence of pyuria, no treatment was initiated. At this time, the owner reported that the patient was still having some incontinence between urinations and when lying down, but was continent for longer periods of time with some voluntary control (continence score, 5/10). Examination showed continued right-sided neuropraxia of the sciatic nerve and hyperextension of the left tarsal joint. The patient was fitted with a custom bootk for the left hind limb to prevent hyperextension. Also at this time, the patient was started on phenylpropanolamine hydrochloridel (1.5 mg/kg [0.68 mg/lb], PO, q 8 h).

Twenty-three weeks after surgery, the patient was examined for stent removal. The owner reported a dramatic improvement in walking, with no appreciable neurologic dysfunction. On physical examination, the patient's sciatic nerve dysfunction had resolved, and the gait at a walk and a run was assessed as normal. The urinary incontinence had improved with phenylpropanolamine hydrochloride administration (continence score, 7/10). A urine sample had been submitted for bacterial culture 1 week earlier by the regular veterinarian and had yielded Staphylococcus intermedius and an Enterococcus sp, and the regular veterinarian had prescribed chloramphenicol (at an unknown dosage). The patient was anesthetized and positioned in dorsal recumbency. The vulva was clipped, aseptically prepared, and flushed with diluted chlorhexidine solution. Routine cystourethroscopy was performed. Findings included diffuse proliferative papules throughout the vestibule and opening of the clitoral fossa into the vestibule. During the procedure, the vaginal septum was lasered open with a diode laserm at 20 W as previously reported.3 A slightly short and wide urethra with papules proximally was noted. Both ureteral stents were removed with gentle traction, using a grasping instrumentn inserted through the working channel of the endoscope. Both UVJs appeared anatomically normal. The urinary bladder looked healed and intact with normal mucosa along the entire surface. The stents were submitted for bacterial culture and susceptibility testing, which yielded the same Enterococcus and hemolytic Staphylococcus spp previously cultured, and chloramphenicol (46.3 mg/kg [21.0 mg/lb], PO, q 8 h for 6 weeks) was prescribed.

Electronic and telephone follow-up was continued with the patient's regular veterinarian as well as bacterial culture of serial urine samples obtained via cystocentesis. It was recommended that bacterial culture be performed every 3 months for 2 years and then every 6 months thereafter, unless culture results were positive, in which case more frequent culture would be needed depending on the treatment plan. At 25 weeks after surgery, the regular veterinarian performed ultrasonography of the urinary tract, which showed improvement in both renal pelvic dimensions with no hydroureter. Results of repeated clinicopathologic testing were within reference limits, and results of bacterial culture of a urine sample were negative. At this time, treatment with estriolo (0.09 mg/kg [0.04 mg/lb], PO, q 24 h) was started and treatment with phenylpropanolamine was continued because of improved, yet persistent, urinary incontinence (continence score, 7/10). At 1.6 years after surgery, the patient was reported to be completely continent with a continence score of 10/10. At this time, the dog was maintained on phenylpropanolamine (1 mg/kg, PO, q 8 h) and estriol (0.04 mg/kg [0.02 mg/lb], PO, q 24 h).

In the time after the patient's last discharge from the Animal Medical Center, multiple urinary tract infections were documented by means of repeated cystocentesis by the regular veterinarian. A variety of bacteria were cultured, and all responded to appropriate antimicrobial treatment selected on the basis of susceptibility test results. Despite these infections, the patient had clinical signs consistent with a urinary tract infection only once, approximately 1 year and 7 months after surgery. The patient was empirically treated with gentamicin sulfate (1 mg/kg), SC, q 24 for 21 days) and penicillin V potassium (10 mg/kg [4.5 mg/lb], PO, q 8 h for 14 days) by the regular veterinarian. Bacterial culture yielded E coli susceptible to trimethoprim-sulfadiazine; therefore, treatment with gentamicin and penicillin was discontinued, and treatment with trimethoprim-sulfadiazine (29 mg/kg [13 mg/lb], PO, q 24 h) was begun.

Approximately 2.1 years after surgery, the dog was reevaluated because of chronic urinary tract infections. A CBC, serum biochemical profile, urinalysis, urine bacterial culture, abdominal ultrasonography, and abdominal radiography were performed. At that time, the dog was neurologically normal. There was no evidence of urinary incontinence, and the dog was reported to have a continence score of 10/10. Abdominal ultrasonography showed greatly improved pelvic dilation in both kidneys (< 0.4 mm each), ureteral wall thickening (left greater than right), and renal asymmetry with architectural changes consistent with chronic renal disease. The urinary bladder was a normal size. The ventral abdominal wall defect was similar to that reported shortly after surgery, but this time, the continuous fibrous band was seen to be adhered to the ventral wall of the urinary bladder. Radiographs of the pelvis and abdomen showed an intra-abdominal urinary bladder, healed osteotomy sites with fractured bone plates bilaterally, and some pelvic inlet narrowing (Figure 3). The urinalysis showed a large number of bacteria (> 40 bacteria/hpf) with a urine specific gravity of 1.016 and WBC count of 6 to 10 WBCs/hpf (reference range, 0 to 5 WBCs/hpf). Urine bacterial culture yielded Proteus vulgaris and P aeruginosa. Clinicopathologic testing showed high BUN (54 mg/dL; reference range, 9 to 31 mg/dL), creatinine (2.4 mg/dL; reference range, 0.5 to 1.5 mg/dL), and symmetric dimethylarginine (15 μg/dL; reference range, 0 to 14 g/dL) concentrations. Enrofloxacin (20 mg/kg [9.4 mg/lb], PO, q 24 h) was prescribed on the basis of results of susceptibility testing. Ten days later, bacterial culture of another urine sample obtained by cystocentesis yielded P aeruginosa despite high-dose enrofloxacin treatment. Thus, meropenem (12 mg/kg [5.45 mg/lb], SC, q 24 h) was added to the enrofloxacin regimen. Ten days later, results of urine bacterial culture were negative and the creatinine concentration was 2.2 mg/dL. The urinary bladder was assessed ultrasonographically before and after urination and was found to have poor function with a moderately high residual volume. A trial course of bethanecol (0.4 mg/kg [0.18 mg/lb], PO, q 8 h) was initiated, and bladder size was monitored over the next 12 weeks. However, bladder size after urination did not change. Therefore, bethanechol administration was discontinued. The dog remained without clinical signs of urinary tract infection or azotemia for 337 days.

Figure 3—
Figure 3—

Follow-up radiographic and ultrasonographic images obtained 2 years after surgery for the dog in Figure 1. A—Ventrodorsal radiographic image showing healing of the osteotomy sites with fracture of the bone plates bilaterally (yellow arrows). The degree of pelvic inlet narrowing is similar to that seen on previous examinations. B—Lateral radiographic image showing a moderately sized urinary bladder (black arrow) and fractured iliac plates. C—Abdominal ultrasonographic image showing a moderately sized bladder and normal-appearing urethra (red arrow).

Citation: Journal of the American Veterinary Medical Association 252, 6; 10.2460/javma.252.6.732

At 1,262 days after surgery, the dog was examined because of a 10-day history of lethargy, anorexia, and urinary incontinence. At this time, clinicopathologic testing revealed severe azotemia (BUN, > 140 mg/dL; creatinine, 12.2 mg/dL). A urinalysis performed 10 days earlier by the regular veterinarian had revealed bacteriuria, and treatment with meropenem had been reinstituted at the previous dosage. On examination at the Animal Medical Center, a urine sample was obtained for bacterial culture. Ultrasonography revealed no evidence of ureteral obstruction, but the urinary bladder was moderately full despite normal urination. At this time, results of urine bacterial culture were negative. After 10 days of IV fluid therapy and meropenem administration, the serum creatinine concentration had not improved, and the dog was euthanized. Necropsy revealed marked asymmetry of the kidneys, with the left kidney substantially smaller than the right. There was turbid fluid within the left renal pelvis and linear white foci in the renal parenchyma suggestive of pyelonephritis. Consistent with this finding was erythema and expansion of the apical bladder mucosa with marked cloudy urine suggestive of an active inflammatory process. Microscopic findings included neutrophilic, lymphoplasmacytic, histiocytic pyelonephritis with neutrophilic tubulitis and microabcesses and regional tubular loss, necrosis, degeneration, and regeneration (left kidney more severely affected than the right) along with glomerulosclerosis with multifocal, radiating, interstitial fibrosis and multifocal basement membrane and glomerular mineralization (left kidney more severely affected than the right). Additionally, the bladder, uterine horns, vagina, and both ureters had various degrees of chronic, diffuse lymphoplasmacytic, histiocytic, neutrophilic inflammation. Overall, these findings were most consistent with a chronic, ongoing inflammatory process originating and ascending from the lower urinary tract and causing chronic kidney disease.

Discussion

Bladder exstrophy is a rare congenital deformity of the bladder, pelvic floor, pelvis, and abdominal wall that has been reported in humans, a monkey, a dog, a goat, and a cat.4–7 This condition is most commonly reported in humans, although it is considered rare,8 and is associated with urinary incontinence, anatomic defects, and gait abnormalities. Bladder exstrophy is part of a spectrum of pelvic and abdominal fusion abnormalities known as the exstrophy-epispadias complex. The International Clearinghouse for Birth Defects Monitoring Systems9 has estimated that the bladder exstrophy-epispadias complex occurs in 3.3 of 100,000 human births. This complex can be as simple as distal epispadias alone, characterized as opening of the urethra dorsally and failure to form a complete tube.10 A moderate form of the complex is classic bladder exstrophy. This variant involves a defect in the abdominal wall with exposure of an open bladder and urethra11 as well as failure of fusion of the abdominal muscles, pelvic ring, and pelvic floor muscles.10 In human females with this congenital condition, there are incomplete labia minora, a nontubularized urethra, a bifid clitoris, and a flat mons pubis.12 Inguinal hernias also commonly occur in affected humans.13 Additionally, the pelvic abnormalities typically include a mean external rotation of 12 degrees of the posterior aspect of the pelvis bilaterally, 18 degrees of external rotation of the anterior aspect of the pelvis, retroversion of the acetabula, and 30% shortening of the pubic rami, leading to progressive diastasis of the pubic rami.14 The dog of the present report had classic bladder exstrophy that seemed to spare the urethra, but included complete exstrophy of the bladder and bladder neck. The dog's abnormalities include an extruded bladder that was visible caudal to the umbilicus, an open vestibule and clitoral fossa, and pubic diastasis. Few studies have been performed to evaluate a genetic link for exstrophy-epispadias complex, although loci for the exstrophy-epispadias complex were found in 7 separate chromosomes with susceptibility genes in a variety of regions.15

To our knowledge, there has been only 1 previous report5 of bladder exstrophy in a dog. In that report, an 8-month-old female English Bulldog was treated solely with dissection of the bladder from the body wall followed by closure of the bladder and body wall. However, the dog developed overwhelming azotemia secondary to pyelonephritis and was euthanized 3 months after surgery. This is in contrast to the dog described in the present report, for which treatment consisted of bilateral ilial osteotomies, insertion of ureteral and urethral catheters followed by ureteral stents and catheters, and repeated urine bacterial culture to monitor for infection. The dog had recurrent and chronic urinary tract infections, which were carefully treated in an effort to prevent pyelonephritis and progressive renal dysfunction over the long term. Additionally, because of the risk of swelling-induced ureteral obstruction after surgery, ureteral catheters and stents and a urethral catheter were used to maintain urine outflow.

The developmental origin of classic bladder exstrophy, part of the exstrophy-epispadias complex, is currently under debate. Most theories suggest there is failure of mesenchymal cells to migrate medially8 between the endoderm and ectoderm, causing premature rupture of the cloacal membrane.16 The cloacal membrane is necessary for formation of the lower abdominal muscles and pelvic bones. We hypothesize that, in the dog described in the present report, there was premature rupture of the cloacal membrane centrally resulting in the described defects.17

The surgical repair technique described in the present report was similar to the approach used in human patients. An important part of the technique was the use of bilateral iliac osteotomies to provide approximation of the pelvis and facilitate successful reconstruction of the urinary bladder, genitalia, and pelvis. This ultimately resulted in resolution of the patient's lameness and urinary incontinence while also allowing creation of an internalized urinary tract.

In humans, a variety of techniques and treatment approaches can be used for treatment of bladder exstrophy-epispadias complex; however, staged and complete primary repair of bladder exstrophy are the 2 most common. Staged repair of bladder exstrophy involves 3 steps, consisting of repair of the bladder, abdominal wall, and pelvis initially, followed by correction of epispadias at a separate time and then bladder neck reconstruction and ureteral reimplantation at a later date. This approach results in a 70% continence rate with minimal complications. Complete primary repair of exstrophy involves repair of the bladder and abdominal wall and correction of epispadias during a single surgical episode. This results in a continence rate similar to that reported for staged repair, but often requires a second surgery in males for correction of hypospadias and continence.18 In our patient, we repaired the bladder, external genitalia, and pelvis during a single surgical episode, similar to the complete primary repair technique described for human patients.

There are many options for treatment of the pelvic deformity in human patients with bladder exstrophy. All have the goal of closing the pelvic ring to decrease tension on the abdominal wall and improve future reconstruction of the urinary system and genitalia.19 The most common method involves bilateral anterior innominate and vertical iliac osteotomies.20 With this method, there is less risk for bladder dehiscence and prolapse.20 Other types of osteotomies that have been used include bilateral osteotomy of the superior pubic ramus and diagonal osteotomy of the iliac wing. In our patient, we performed bilateral iliac linear osteotomies, which resolved the abnormal gait and decreased the width of the pubic diastasis, but did result in temporary unilateral neuropraxia.

Some of the most common complications associated with osteotomies were described in a retrospective study by Sponseller et al.19 In that study, 82 children underwent an anterior innominate osteotomy with or without a posterior osteotomy. The most common complication was transient left femoral nerve palsy (7 patients) that resolved fully by 12 weeks after surgery and required bed rest for 6 to 8 weeks. There was also delayed union of osteotomy sites (3 patients), superficial infection at ileofemoral incisions (1 patient), pin infection (1 patient), transient motor palsy of the right peroneal nerve (1 patient), and transient right thigh abductor weakness (1 patient).19 In another study21 of 178 patients with bladder exstrophy in which repair involved an orthopedic procedure, injuries to the femoral nerve (8 patients), peroneal nerve (2 patients), sciatic nerve (2 patients), and superior gluteal nerve (1 patient) were documented. Overall, 9 patients had spontaneous recovery, which included all of the patients with femoral nerve injuries (in 7 patients, recovery took 4 months, and in 1, recovery took 11 months). The remaining patients had limited recovery.21 The dog described in the present report developed sciatic neuropraxia after surgery. This could have happened for a variety of reasons, such as retraction of the soft tissues; injury from the saw, drill, tap, or screws; or compression or stretching of the nerve due to rotation of the pelvic bones. Careful dissection around the sciatic notch during surgery should decrease the incidence of sciatic nerve palsy, although even with careful dissection in the dog described in the present report, neuropraxia still occurred. This patient's neuropraxia had resolved completely by 23 weeks after surgery.

Complications that occurred in our patient in association with primary bladder closure were urinary incontinence, ureteral obstruction, pyelonephritis, and recurrent urinary tract infections. Among all the complications reported in human patients undergoing repair of bladder exstrophy, persistent urinary incontinence resulting from either too loose a bladder neck reconstruction or bladder outlet obstruction is the most important.22 In fact, the definition of successful repair of exstrophy-epispadias complex in human patients usually involves achievement of continence. In a retrospective study23 of 67 male patients treated with staged repair of bladder exstrophy, 70% achieved continence, defined as voiding through the urethra with dry periods > 3 hours without bladder augmentation or intermittent catheterization. Mean time to achieve continence during the day was 14 months, and mean time to achieve continence at night was 23 months. Bladder capacity at the time of bladder neck reconstruction was predictive of whether continence was achieved as well as the time required to achieve continence.23 In a retrospective study24 of 41 females, 74% of patients were continent day and night without augmentation or catheterization after staged repair of bladder exstrophy. Urinary incontinence improved immediately after surgery but took over a year to resolve completely.24 These times to achieve continence were consistent with the outcome for our patient.

The dog of the present report experienced bilateral ureteral obstructions due to UVJ compression. Ureterovesicular junction compression could have occurred because of pelvic compartment syndrome or bladder outlet obstruction when the bladder was closed and internalized. Temporary ureteral stenting was performed while the bladder outlet healed, which resolved the obstruction. In turn, as the bladder enlarged, the UVJ compression resolved. In a recent multi-institutional study25 consisting of 16 human patients with bladder exstrophy repaired with the complete primary repair technique, 2 female patients developed urethral obstruction and 3 patients developed pyelonephritis. Ureteral obstructions also are a common complication with exstrophy repair.22 In humans, if urethral outflow obstruction is documented, then intermittent overnight catheter drainage (deemed clean intermittent catheterization)7,25–28 can be used to help preserve renal health; however, this approach is not possible in dogs because of their internalized urethral orifice and the need for sedation and risk of contamination during intermittent catheterization. However, placement of a cystostomy tube may be an alternative.

Our patient also developed pyelonephritis that we suspect was due to exteriorized ureteral and urethral catheters that were necessary after surgery to maintain ureteral and urethral patency and allow the bladder time to heal. It is common for children who have undergone exstrophy repair to also have exteriorized ureteral catheters; however, instead of a urethral catheter, a suprapubic catheter is used to drain the bladder.29 Once all catheters were removed, culture of urine from the renal pelvis revealed P aeruginosa infection in our patient. In humans, it is recommended to culture the urine before removing the urethral catheter.22 In the dog of the present report, an already existing infection was likely potentiated once the catheters were removed and stagnancy occurred secondary to urinary outflow obstruction. The pyelonephritis resolved with antimicrobial treatment and placement of bilateral ureteral stents and a urethral catheter for drainage.

Despite the success with urinary continence and return to full neurologic and orthopedic soundness in the patient described in the present report, concern remained because of chronic bacterial colonization and potential gentamicin toxicosis, and there was evidence of renal compromise with the increased creatinine concentration. Several urinary tract infections were documented in the short and long term after surgery, even though the patient only displayed clinical signs once (approx 1 year and 7 months after surgery), until the patient was documented to be in end-stage renal failure. There are several possible reasons for the development of chronic urinary tract infections in our patient, including a widened and short urethra, antimicrobial resistance, low urine specific gravity, urine stagnancy in the urinary bladder owing to poor detrusor muscle function, and altered urothelial defense mechanisms. The patient's clinical signs resolved after initiation of treatment with gentamicin at an appropriate dosage for an extended period of time, but this ultimately resulted in long-term renal injury. Performing urinalyses to look for evidence of casts or serum biochemical testing to monitor renal biochemical parameters was not performed in this patient during aminoglycoside administration. Had evidence of increasing creatinine concentration or urinary casts been identified, administration of the aminoglycoside could have been stopped sooner, potentially avoiding nephrotoxicosis. Five months later, the patient had mild azotemia with a creatinine concentration of 2.4 mg/dL. Additional potential causes of azotemia in this patient were chronic urinary tract infections, pyelonephritis, and urethral or ureteral obstruction. Evaluation of the bladder size before and after voiding initially showed that there was minimal stagnancy of urine in the bladder after voiding, but over time it was determined that the dog was unable to fully empty the urinary bladder. Ultrasonographically, the ureters and renal pelves were minimally dilated, making obstruction an unlikely cause of the azotemia. In a study30 comparing functional and electrolyte effects during and 20 days after a 10-day course of gentamicin at a low (7 mg/kg/d [3.2 mg/lb/d]) or high (30 mg/kg/d [13.6 mg/lb/d]) dosage, tubulointerstitial nephritis occurred with both the low and high dosages. Decreased urinary concentration and glomerular filtration rate as well as glucosuria, hypokalemia, and hypocalcemia were seen in the high-dosage treatment group. Avoiding aminoglycoside treatment in dogs, especially those with urinary tract compromise, is recommended. If aminoglycoside treatment is necessary, then careful monitoring is essential.

Necropsy findings for the dog in the present report suggested that pyelonephritis and chronic kidney disease secondary to chronic urinary tract infections were the cause of the patient's severe azotemia and ultimate death. In the dog of this report, the biggest concern related to chronic urinary tract infections was urine retention in the bladder and the resulting urine stagnancy. Administration of parasympathomimetics, such as bethanechol, at an earlier time could potentially have aided in the management of the chronic urinary tract infections and ultimate renal compromise. Placement of a cystostomy tube could have been considered to keep the urinary bladder empty, but this likely would have exacerbated the chronic urinary tract infections. Additionally, it was almost a year between the patient's last recheck ultrasound examination and urine bacterial culture and reexamination at our hospital. Increasing the frequency of diagnostic monitoring may have allowed for earlier identification of urinary tract infections, preventing renal disease.

Bladder exstrophy-epispadias complex is a rare congenital deformity that can result in genitourinary and musculoskeletal defects with various degrees of severity. The most common variant, classic bladder exstrophy, involves a defect in the abdominal wall exposing an open bladder and urethra, pubic diastasis, and epispadias.29,31 Achieving continence is the main goal in repairing bladder exstrophy-epispadias in patients. In humans, successful surgical repair requires tension-free closure, an adequate outflow passage for urine, secure placement of stents, proper incision care with minimal infection and movement,22 and adequate postoperative physical therapy. Achieving adequate bladder capacity prior to bladder neck reconstruction as well as having successful primary closure is paramount in developing continence. Repair of bladder exstrophy and epispadias was successful in the dog described in the present report, and the technique used can be considered as an option for the treatment of this deformity in dogs. To our knowledge, the present report is the first to describe successful surgical correction in a dog with bladder exstrophy with continence and long-term clinical outcome.

Acknowledgments

No third-party funding or support was received in connection with this study or the writing or publication of this manuscript. The authors declare that there were no conflicts of interest.

ABBREVIATIONS

CRI

Continuous rate infusion

UVJ

Ureterovesicular junction

Footnotes

a.

Richard Wolf Medical Instruments Corp, Vernon Hills, Ill.

b.

ISO-C fluoroscopy C-arm, Siemens, Malvern, Pa.

c.

0.035-inch, 150-cm, angled/regular taper standard hydrophilic Weasel Wire, Infiniti Medical, Menlo Park, Calif.

d.

5F, 30-cm pigtail drainage catheter set without safety string lock, Infiniti Medical, Menlo Park, Calif.

e.

Jackson-Pratt, Cardinal Health, New York, NY.

f.

Synthes Inc, West Chester, Pa.

g.

Respond 2400XL, Respond Systems Inc, Branford, Conn.

h.

Multiwave Locked System Mphi Vet, Cutting Edge Veterinary Lasers, Fairport, NY.

i.

Companion Therapy Laser CTC-12, Lite Cure LLC, Newark, Del.

j.

InLay Optima 4F × 22- to 32-cm multilength ureteral stent kit, Bard, Covington, Ga.

k.

Thera-Paw Inc, Lebanon, NJ.

l.

Proin, PRN Pharmacal, Pensacola, Fla.

m.

600-μm diode laser fiber and 25-W diode laser, Lumenis Inc, Santa Clara, Calif.

m.

Captura three-prong grasper, Cook Medical, Bloomington, Ind.

o.

Incurin, Merck Animal Health, Summit, NJ.

References

  • 1. Kuntz JA, Berent AC, Weisse CW, et al. Double pigtail ureteral stenting and renal pelvic lavage for renal-sparing treatment of obstructive pyonephrosis in dogs: 13 cases (2008–2012). J Am Vet Med Assoc 2015;246:216225.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 2. Berent AC, Weisse CW, Mayhew PD, et al. A prospective study evaluating the cystoscopic guided laser ablation of ureteral ectopia in 30 dogs. J Am Vet Med Assoc 2012;240:716725.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 3. Burdick S, Berent AC, Weisse C, et al. Endoscopic-guided laser ablation of vestibulovaginal septal remnants in dogs: 36 cases (2007–2011). J Am Vet Med Assoc 2014;244:944949.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 4. Stec AA, Hommer R, Walker LC, et al. Classic bladder exstrophy in a nonhuman primate: a comparative analysis. Urology 2002;59:180183.

  • 5. Hobson HP, Ader PL. Exstrophy of the bladder in a dog. J Am Anim Hosp Assoc 1979;15:103107.

  • 6. Athanasiou LV, Papadakis SM, Polizopoulou ZS. Spontaneous exstrophy-epispadias complex in a goat-kid. Vet Q 2012;32:117119.

  • 7. Geoffroy Saint-Hillaire I. Extroversion de la vessie. In: Geoffroy Saint-Hillaire I, ed. Histoire génèrale et particulière des anomalies de l'organisation chez l'homme et les animaux: des monstruosités, des variétiés et vices de conformation. Vol 1. Paris: J-B Bailliere, 1832;387391.

    • Search Google Scholar
    • Export Citation
  • 8. Tekes A, Ertan G, Solaiyappan M, et al. 2D and 3D MRI features of classic bladder exstrophy. Clin Radiol 2014;69:e223e229.

  • 9. International Clearinghouse for Birth Defects Monitoring Systems. Epidemiology of bladder exstrophy and epispadias: a communication from the International Clearinghouse for Birth Defects Monitoring Systems. Teratology 1987;36:221227.

    • Search Google Scholar
    • Export Citation
  • 10. Stec AA. Embryology and bony and pelvic floor anatomy in the bladder exstrophy-epispadias complex. Semin Pediatr Surg 2011;20:6670.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 11. Inouye BM, Tourchi A, Di carlo HN, et al. Modern management of the exstrophy-epispadias complex. Surg Res Pract 2014;2014:19.

  • 12. Frimberger D. Diagnosis and management of epispadias. Semin Pediatr Surg 2011;20:8590.

  • 13. Connolly JA, Peppas DS, Jeffs RD, et al. Prevalence and repair of inguinal hernias in children with bladder exstrophy. J Urol 1995;154:19001901.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 14. Sponseller PD, Bisson LJ, Gearhart JP, et al. The anatomy of the pelvis in the exstrophy complex. J Bone Joint Surg Am 1995;77:177189.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 15. Ludwig M, Rüschendorf F, Saar K, et al. Genome-wide linkage scan for bladder exstrophy-epispadias complex. Birth Defects Res A Clin Mol Teratol 2009;85:174178.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 16. Ambrose SS, O'Brien DP. Surgical embryology of the exstrophy-epispadias complex. Surg Clin North Am 1974;54:13791390.

  • 17. Gearhart JP, Mathews RI. Exstrophy-epispadias complex. In: Wein AJ, Kavoussi LR, Novick AC, et al, eds. Campbell-Walsh urology. Vol 4. 10th ed. Philadelphia: Elsevier, 2012;33273335.

    • Search Google Scholar
    • Export Citation
  • 18. Massanyi EZ, Gearhart JP, Kost-byerly S. Perioperative management of classic bladder exstrophy. Res Rep Urol 2013;5:6775.

  • 19. Sponseller PD, Jani MM, Jeffs RD, et al. Anterior innominate osteotomy in repair of bladder exstrophy. J Bone Joint Surg Am 2001;83:184193.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 20. Gearhart JP, Forschner DC, Jeffs RD, et al. A combined vertical and horizontal pelvic osteotomy approach for primary and secondary repair of bladder exstrophy. J Urol 1996;155:689693.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 21. Okubadejo GO, Sponseller PD, Gearhart JP. Complications in orthopedic management of exstrophy. J Pediatr Orthop 2003;23:522528.

  • 22. Hernandez DJ, Purves T, Gearhart JP. Complications of surgical reconstruction of the exstrophy-epispadias complex. J Pediatr Urol 2008;4:460466.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 23. Baird AD, Nelson CP, Gearhart JP. Modern staged repair of bladder exstrophy: a contemporary series. J Pediatr Urol 2007;3:311315.

  • 24. Purves JT, Baird AD, Gearhart JP. The modern staged repair of bladder exstrophy in the female: a contemporary series. J Pediatr Urol 2008;4:150153.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 25. Borer JG, Vasquez E, Canning DA, et al. An initial report of a novel multi-institutional bladder exstrophy consortium: a collaboration focused on primary surgery and subsequent care. J Urol 2015;193:18021807.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 26. Koff SA, Gigaz M, Jayanthi V. Nocturnal bladder emptying: a simple technique for reversing urinary tract deterioration in children with neurogenic bladder. J Urol 2005;174:16291631.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 27. Montané B, Abitbol C, Seeherunvog W, et al. Beneficial effects of continuous overnight catheter drainage in children with polyuric renal failure. BJU Int 2003;92:447451.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 28. Nguyen MT, Pavlock CL, Zderic SA, et al. Overnight catheter drainage in children with poorly compliant bladders improves post-obstructive diuresis and urinary incontinence. J Urol 2005;174:16331636.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 29. Woo LL, Thomas JC, Brock JW. Bladder and cloacal exstrophy. In: Coran AG, Adzick NS, Caldamone AA, et al, eds. Pediatric surgery. 7th ed. Philadelphia: Elsevier, 2012;15151517.

    • Search Google Scholar
    • Export Citation
  • 30. Cronin RE, Bulger RE, Southern P, et al. Natural history of aminoglycoside nephrotoxicity in the dog. J Lab Clin Med 1980;95:463474.

  • 31. Inouye BM, Massanyi EZ, Di carlo H, et al. Modern management of bladder exstrophy repair. Curr Urol Rep 2013;14:359365.

All Time Past Year Past 30 Days
Abstract Views 299 0 0
Full Text Views 1371 1100 223
PDF Downloads 499 207 19
Advertisement