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12 year old female with urinary bladder and right renal agenesis. Contrast enhanced CT abdomen in portal phase shows empty right renal fossa (arrow) and normally enhancing orthotopic left kidney. Protocol: Philips Brilliance 64 slice CT 200 mAs, 120 kV, 1.5 mm slice thickness, 45 ml Omnipaque.
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Complete agenesis of urinary bladder is an extremely rare anomaly with only a few live cases reported so far. In most of the instances death occurs early as it may be incompatible with life. Here we report a case of adolescent female with urinary bladder and unilateral renal agenesis, who presented with a rather unusual presentation of incontinence...
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Citations
... The pathophysiology of bladder agenesis is unknown but has been attributed to urogenital sinus maldevelopment at weeks five to seven of embryogenesis [5,6]. In normal development, this embryonic period is defined by the division of the cloaca into the anorectal canal posteriorly and the urogenital sinus anteriorly. ...
Bladder agenesis is an exceedingly rare congenital anomaly with an estimated incidence of 1 in 600,000 [1,2]. Patients born with bladder agenesis carry a poor prognosis due to its association with renal impairment and defects of other organ systems, including the reproductive tract, vascular system, and neurologic system [2-4]. About 90% of those born with bladder agenesis are female, with an estimated female-to-male ratio of 30:1 [1,2]. Herein, we present two cases of bladder agenesis and one case of bladder dysgenesis in female patients; two underwent successful ileal neobladder creation and one underwent incontinent colonic conduit creation.
... The prenatal presentation was due to oligohydramnios, and the definitive diagnosis was after birth. (2) Late presentation: beyond the first year of life, usually with incontinence [2,3,5,11,15,18,37,40,52,53], urinary tract infection (UTI) [1,6,30,34,43], or both [13,27,30,33,35,42]. ...
... An aberrant vascular artery connecting the right internal iliac artery to the left external iliac artery has been documented in two cases by Lowrey et al., in addition to an absent left common iliac artery in one subject [50]. Indiran et al. reported a case in which the subject had a high bifurcation of the aorta with the right external iliac artery, giving rise to what seems to be the posterior trunk of the right internal iliac artery in association with right developmental dysplasia of the hip (DDH) and gluteal muscular atrophy [35]. In a follow-up to Rodin et al.'s report, a more detailed description of the vascular anomaly was mapped, starting below the level of the superior mesenteric artery, where the abdominal aorta branches into two vessels; the smaller of the two is thought to be the distal portion of the abdominal aorta, and the larger vessel is thought to be the aberrant abdominal umbilical artery, continuing to the left as the iliac and common femoral artery. ...
... Lower limb deformities: Equinovarus deformities (clubfeet) [1,29], left foot agenesis, remnant extremity [20], rocker bottom feet [29], DDH [29,34,35,52]; one with gluteal and thigh muscle atrophy [35] and another with genu valgum [29], bilateral inguinal hernia [26], and absent fibula (hemimelia) [56]. ...
Bladder agenesis is a rare congenital deformity characterized by the absence of the bladder. It is primarily observed in postmortem dissections of stillbirths rather than live births. The condition is often associated with other congenital anomalies, leading to the hypothesis that most affected fetuses do not survive to term. However, the exact cause and specific associated anomalies remain unclear and poorly described in the literature. The limited mention of bladder agenesis in textbooks and literature underscores the importance of creating a comprehensive source for future research in this field. Therefore, our objective is to collect and analyze data on bladder agenesis, focusing on associated anomalies and potential causes, to enhance our understanding of the condition.
We conducted a thorough review of reports collected from three databases, Google Scholar, PubMed, and Science Direct, last searched on July 30, 2023, starting with 327 reports. Excluding duplicates and records written in languages other than English, veterinary studies, irrelevant reports, or stillbirths. Inclusion criteria were the following: cases must have proven bladder agenesis, not hypoplasia, and must have most of the information, including the age of diagnosis, presenting symptoms, gender, associated anomalies, and management or outcome of the patient. A quality assessment was conducted according to the Joanna Briggs Institute checklist for case reports. A total of 65 case reports from 56 articles were included in the review.
Through our manual analysis, we documented a wild array of malformations associated with bladder agenesis. Among the reports reviewed, 93% exhibited urinary system malformations beside bladder agenesis, 77% were found to have reproductive malformations, 44% had gastrointestinal anomalies, 38% showed musculoskeletal malformations, 28% had cardiac malformations, and another 28% had vascular anomalies. The overall mortality rate was 38%, with a higher rate of 74% for males compared to 20% for females. By collating and analyzing those case reports, we aim to contribute to a better understanding of bladder agenesis and its associated anomalies, facilitating further investigations and advancements in the field.
... Contrast enhanced CT abdomen with arterial, portal, and excretory phase images could identify different kinds of urinary tract anomalies, such as renal agenesis, as well as other associated intra-abdominal anomalies [11] . Besides, renal agenesis can be confirmed by ultrasound, with empty renal fossae and absent bladder filling [12] . ...
... In addition, the lack of recognisable renal arteries on color Doppler would strongly support the suspicion of renal agenesis [13] . Regarding the work-up of ectopic ureter, a renal ultrasound and/or computerized tomography is essential for the diagnosis [11] . ...
Congenital anomalies of the kidney and the urinary tract such as renal agenesis and ec-topic ureter have complex development. These anomalies have variable presentations and associations. In this report, we highlight the case of a young man with congenital renal age-nesis presenting for a urinary tract infection. Abdominal and pelvic computed tomography imaging revealed the rare association of renal agenesis with contralateral ectopic ureter and subsequent hydroureteronephrosis. A urinary tract infection can be the presenting complication of such association, and a long follow-up is needed to anticipate the management.
... While urological anomalies are among the most common congenital defects [1], bladder agenesis is an extremely rare congenital anomaly, with approximately 74 cases described with only 25 cases of live birth reported in the literature written in English until 2020 [2]. e live-birth patients are often diagnosed in infancy or early childhood, presenting with other anomalies, urinary incontinence, and recurrent urinary tract infection (UTI) [3]. ...
... During the literature review, we found few other publications that addressed lower urinary tract reconstruction and attained continence in patients with bladder agenesis; Kasat et al. [3] managed a case of a five-year-old child with bladder agenesis and ectopic ureter opening into the vestibule using a continent ileocecal pouch (Penn pouch) with the Mitrofanoff principle [3]. 2 Advances in Urology ...
Background:
Agenesis of the bladder and urethra is a rare congenital anomaly, with a very few living cases reported in the literature so far. Case Presentation. We are reporting two female patients (3 and 6 years old) with bladder and urethral agenesis who presented with urinary incontinence. In both patients, magnetic resonant imaging (MRI) revealed a case of bladder and urethral agenesis with normal ureters draining into the vagina. Patients underwent a neobladder and conduit creation surgery. The neobladder was constructed from the whole cecum and a part of the ascending colon, followed by an anastomose of the ureters into the neobladder in a nonrefluxing fashion; the appendix was used simultaneously as a continent catheterizable conduit. The two patients attained urinary continence postoperatively.
Conclusion:
We reported two cases of bladder agenesis, and for the first time, we have performed neobladder creation surgery using the cecum and ascending colon. One-year follow-up did not reveal any complications.
... Urinary bladder agenesis is one of the rarest urinary tract anomalies, with a reported incidence of 1 in 600,000patients [1]. Only 64 cases have been reported worldwide, with a significant female predominance, where it occurs thirty times more often in girls than in boys [2][3][4][5]. Only 25 live births have been reported, as the condition is usually associated with other severe malformations that are incompatible with life 6 . ...
... Only 25 live births have been reported, as the condition is usually associated with other severe malformations that are incompatible with life 6 . Reported associated anomalies include those of the urogenital, gastrointestinal, vascular and musculoskeletal systems [2,6]. The cause of urinary bladder agenesis has been attributed to urogenital sinus injury at weeks 5-7 of embryogenesis [4]. ...
... There is female predominance, where girls are affected thirty times more than boys; reported cases have all been females except for four males [9][10][11][12]. This predominance is explained by the anatomy of the reproductive system of females that enables them to maintain urine drainage, as opposed to male patients who are usually stillborn or die early [2]. Male neonates with absent bladder can survive only if urine drainage is allowed through ectopic ureteric insertion into the rectum or a patent urachus [3,11,13]. ...
Background:
Urinary bladder agenesis is a very rare congenital anomaly with very few cases reported in the literature.
Case presentation:
We report a one-month-old baby presenting with ambiguous genitalia and recurrent urinary tract infections. Her clinical course was complicated by renal impairment. Magnetic resonant imaging (MRI) revealed a diagnosis of bladder agenesis with bilateral ectopic insertion of the ureters into the vagina, associated with several other anomalies. The patient underwent bilateral high anterior ureterostomies in an hospital abroad at 5.5 months of age. She then developed ureteral necrosis that had to be corrected with left pyeloplasty and by placing a left nephrostomy tube for drainage. Eventually, the patient's renal function declined, and she developed chronic kidney disease (CKD).The case with its imaging findings and pathogenesis as well as a review of the literature are presented.
Conclusions:
Urinary bladder agenesis is a rare congenital condition that can be associated with multiple anomalies. Early diagnosis and therapeutic intervention can prevent progression to chronic kidney disease.
... 3 Over 90% of the reported cases of bladder agenesis have been female neonates, 3 and a 30:1 female-to-male ratio has been recorded. 4 We report a case of one twin live infant without oligohydramnios who was found to have bladder agenesis, ectopic ureters and a multicystic dysplastic horseshoe pelvic kidney. ...
... It has been predicted that ureteric drainage into the female genital tract allows for passage of urine and hence helps maintains renal function. 4 Bladder agenesis is thought to be compatible with life in a male neonate only if the ureters ectopically drain into the rectum or a patent urachus. 5 Our patient's radiographic findings were consistent with the absence of a bladder, and ectopic ureters were found to be most likely terminating into the seminal vesicles, if not a blind pouch. ...
A monochorionic-diamniotic twin baby presented with intrauterine growth restriction and anuria. The baby was found to have bladder agenesis, a pelvic dysplastic horseshoe kidney, vertebral anomalies, a ventricular septal defect and facial dysmorphisms. It was surprising to find no abnormalities in amniotic fluid indices prenatally, suggesting the possibility of urine output that declined as the pregnancy proceeded. Some degree of twin-to-twin transfusion of amniotic fluid was also possible, which could have rescued the oligohydramnios known to be associated with kidney and urinary tract abnormalities. It was also notable that there was no abnormality in respiratory function, especially since further investigations revealed close to no kidney function. The intrauterine growth restriction (IUGR) along with the multiple anomalies found made the baby unsuitable for dialysis and transplant, and the decision of transition to palliative care was made.
We report a case of a solitary kidney and a single-system entopic ureter draining into the vestibule in an infant with a Müllerian duct defect. Due to the absence of the upper vagina and uterus, an extremely underdeveloped bladder masqueraded as the vagina, and bladder agenesis was suspected preoperatively. Urinary continence was achieved using staged bladder surgery without augmentation or urinary diversion.
Ultrasound is the imaging modality of choice for assessment of the urinary tract in children, particularly in the workup of hydronephrosis, urinary tract infection, kidney masses, and bladder abnormalities. Other important applications include the evaluation of stone disease, kidney failure, renovascular disease, and kidney transplants. This chapter reviews the development and anatomy of the urinary tract followed by an overview of ultrasound imaging features of urinary tract disorders in children. Brief discussions of clinical presentation and treatment approach are also included.
Bladder agenesis is a rare congenital anomaly infrequently reported in the literature, with an incidence of 1/600,000 patients.¹ Commonly associated with other fatal malformations, the condition is often incompatible with life. Prior reports estimate that over 90% of living children born with this malformation are female, owing to renal preservation resulting from low pressure drainage of urine into the vagina, uterus, and vestibule.²,³ Herein we report a rare case of an infant male born with penoscrotal transposition and end stage renal disease secondary to bilateral cystic renal dysplasia found to have concurrent bladder agenesis and bilateral ureteral ectopia.
ABSTRACT
Nutrition during illness is an important part of recovering patients in the hospital and necessary
appropriate together medical treatment. At present nursing care using nutrition have two methods, first
is a feeding bypassing the usual process of enteral feeding and second is parenteral nutrition using by
intravenous route. If nutrition energy are inadequate calories, parenteral nutrition will be necessarily for
patient. The purpose of this article needs to be stress that the majority of nurses have nutrition assessment
and nursing practice by using nursing process approach. In addition the article point to practice nurse’s
awareness and showed precede nutritional intervention strategies. Therefore nurse should emphasize
in the planning step of nursing process lead to quality and effectiveness of nursing care for patients,
prevention adverse effects and complications of parenteral nutrition support, including preventing of
diversely affect every major organ system and may result in death.
