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At least 19 recordsLinked to original sources
PubMed2000

Prune belly syndrome.

Prune belly is a lax, wrinkled abdominal wall and frequently is associated with other anomalies. The prune belly syndrome is a specific constellation of anomalies consisting of an abdominal wall deficient in muscular tissue, dilated urinary tract, and bilateral cryptorchidism. This group of anomalies is also called the Eagle-Barrett syndrome and the triad syndrome. In about 3 of 4 patients with the prune belly syndrome, there are associated malformations of the cardiopulmonary, gastrointestinal, and orthopedic systems.

Abdominal Muscles↗
PubMedDate not supplied

Orthopaedic aspects of prune belly syndrome.

Prune belly syndrome is a relatively uncommon disorder that is characterized by intrauterine urinary obstruction associated with cryptorchisdism, oligohydramnios, and orthopaedic deformations. The oligohydramnios is believed to produce limited intrauterine space, which in turn leads to fetal compression and the resultant deformities. The deformities observed in our patients were developmental dislocation of the hip (DDH), clubfeet, metatarsus adductus, vertical talus, and congenital muscular torticollis. These deformations should be treated aggressively because children with prune belly syndrome may be expected to have a relatively normal life if their renal function is good. The one exception is that as infants these children are very susceptible to pulmonary infections because of their inability to cough. Therefore, treatment of the hip and other deformations should be delayed until the children are old enough to be able to clear pulmonary secretions easily.

Adolescent↗
PubMedDate not supplied

[Prune belly syndrome].

The prune belly syndrome consists of congenital absence or deficiency of the anterior abdominal muscle, bilateral cryptorchidism and anomalies of urogenital tract. Neurological problems are common in these patients as respiratory, orthopedic, gastrointestinal and chromosomal anomalies. We have treated 17 cases of prune belly syndrome. Mortality is 23.5%: two patients died in the neonatal period of respiratory failure; one patient, aged two months, died of septicemia in postoperative period, another, 14 years old, died of respiratory failure with normal renal function. Two patients are female without urological anomalies, one male has an incomplete form with normal urinary tract. Two patients with complete syndrome are treated conservatively. Ten patients with vesicourethral reflux or ureteral dilatation for distal obstruction are treated surgically. We performed 20 ureteral reimplantations with 16 tailoring of the ureters. In three patients ureteral reimplantation failed and in these patients we performed successfully a transureteroureterostomy. All patients have adequate complete bladder emptying without surgical procedure. Renal function is normal in seven, slightly reduced in one and reduced but stable in the others. Orchidopexy was performed successfully in eleven patients (10 Fowler-Stephens procedures). Orchiectomy with testicular prosthesis was performed in a patient 12 years old. Five patients have moderate respiratory insufficiency treated with chest physiotherapy. Urinary tract abnormalities are the most common cause of morbidity and mortality in patients with prune belly syndrome. Treatment regimen must be individualized. Respiratory problems are also frequent and is mandatory a precocious chest physiotherapy.

Adolescent↗
PubMed1987

Obstructive lesions of the lower urinary tract in the prune belly syndrome.

The prune belly syndrome is a well-recognized entity consisting of deficient abdominal musculature, cryptorchid testes, and urinary tract abnormalities most consistent with an obstructive phenomenon. However, an obstructive lesion has not been consistently identified in previously reported cases. Retrospective review of the autopsy files of The Johns Hopkins Hospital, Baltimore, identified 18 cases of prune belly syndrome occurring since 1945. In 13 cases, obstructive lesions in the lower urinary tract had been described grossly. A single case, in which the lower urinary tract had been preserved intact and subsequently serially sectioned as 8-micron sections taken at every 120 micron, demonstrated an obstruction consisting of two overriding urethral lumens connected only by a narrow channel. Reconstruction of the sections suggested that this represented a "kink" in a short segment of the prostatic urethra just above the membranous urethra, leading to the obstructive changes of bladder hypertrophy and dilatation, hydroureter, hydronephrosis, and renal dysplasia seen higher in the urinary tract. The results suggest that examination of the lower urinary tract in cases of prune belly syndrome by serial histologic sectioning of the intact unopened prostatic and penile urethra may be required to demonstrate the presence or absence of an obstructive lesion.

Female↗
PubMed1989

Pathophysiologic and anesthetic correlations of the prune-belly syndrome.

"Prune-Belly" is the name given to the disease which is characterized by a congenital wrinkled appearance of the abdomen. Usually, a triad of congenital anomalies highlights the components of the prune-belly syndrome. This triad consists of undescended testicles, abdominal musculature deficiency and urinary tract abnormalities. The previously described triad of the syndrome is by no means the total spectrum of the disease. Prune-belly syndrome is also associated with diseases of the respiratory, cardiovascular, skeletal, gastrointestinal and central nervous systems. Associated diseases of major body systems which are caused by renal and cardiopulmonary anomalies, as well as the surgical procedure to be performed, influence the plan for the administration of anesthesia. The nature of the genitourinary diseases in the prune-belly baby may obviate the need for surgical intervention as early as the neonatal period of life. Although this congenital disease occurs with some rarity, the gravity of the syndrome demands an in-depth knowledge of its pathophysiology in order to assure uncomplicated anesthetic care. Astute surveillance during preanesthetic, anesthetic, and postanesthetic management is germane to the prevention of mishaps.

Anesthesia↗
PubMed2006

Calculus in a toddler with end-stage renal disease due to prune-belly syndrome.

Prune-belly syndrome is a congenital kidney and urinary tract anomaly which may lead to end-stage renal failure (ESRF). The present case describes an infant suffering from end-stage kidney disease due to prune-belly syndrome, undergoing chronic hemodialysis, with excessive calculus deposits which disappeared following kidney transplantation. Possible explanations are discussed. The first mechanism is associated with lack of oral function which may have caused pooling of saliva around the teeth enhancing precipitations of minerals. The second possible mechanism is associated with the child's uremic state. The third mechanism could be a disturbance in calcium-phosphor metabolism. It is possible that in the present case, the gastrostomy and the electrolyte disturbances characterizing ESRF had an additive effect.

Child, Preschool↗
PubMed2004

Surgical treatment with the technique of Galveston-Luque of spine deformities associated with the prune belly syndrome.

In prune belly syndrome (PBS), spine deformities, particularly scoliosis, are the most frequent accompanying orthopedic problem (18%). We give a detailed description of two cases with prune belly-associated spine deformities. A combined anterior-posterior fusion procedure with posterior correction and stabilization according to the Galveston-Luque technique was carried out in both patients. In one patient insufficiency of the pelvic fixation developed with loss of balance. Reoperation was performed. In the second patient correct alignment and balance was achieved, breakage of both metal rods occurred over time. The PBS represents a model of how scoliosis may emerge. The lack of the abdominal musculature subsequently leads to congenial imbalance, which may cause lordo-scoliotic deformity. The operative treatment of spine deformities in the PBS in the presented technique is efficient. In great deformities balance is difficult to achieve and maintain especially in the lumbo-sacral junction.

Adolescent↗
PubMed2000

A case of prune belly syndrome.

The prune belly syndrome was first described in 1839 by Frolich. Till now about 300 cases have been reported in literature. The complete form of the syndrome is presented by a classic triad that is seen only in boys and is incompatible with life. The etiology of the syndrome is uncertain. Genetic factors, intrauterine infections, adverse mechanical factors have been implicated. The prenatal diagnosis relies on the ultrasonographic findings of oligohydramnion, renal anomalies, and non-immune fetal hydropsy. The present case concerns a newborn of male sex suffering from the most severe and rare form of the syndrome. The child died 5 minutes after his birth. The pathological examination found the patient devoid of striated muscles anterior abdominal wall, urethral atresia, hyperthrophy of the urinary bladder, bilateral hydroureter and hydronephrosis and coinciding cryptorchidism. The case indicates the PBS as one of the possible reasons for non-immune fetal hydropsy.

Abdominal Muscles↗
PubMed1988

[A surgical method of abdominal wall reconstruction in prune belly syndrome].

The "Prune Belly"-syndrome consists of a triad of anomalies: absence or hypoplastic musculature of the abdominal wall, bilateral cryptorchidism, and dilatation of the urinary tract. A surgical method of construction of the abdominal wall in such a case with a missing rectus abdominis is reported.

Abdominal Muscles↗
PubMed1991

[Prognosis of prune belly syndrome].

The Prune Belly syndrome (PBS) is unfrequent. Fourteen cases have been followed in our unit during the last 20 years. Four infants (29%) died during the first months of life, because of neonatal sepsis (2 cases) or end-stage renal failure (2 cases). Among the other 10 cases, 6 (43%) had normal glomerular filtration rate at a mean age of 10 years 6 months (6 months to 15 years), 4 had chronic renal failure, including 3 cases who developed end-stage renal failure at 8, 8 years 8 months and 17 years respectively. Resection of an urethral obstruction was performed in 2 cases. This surgical indication remains widely accepted, while the current tendency is to limit ureteral surgery in PBS. Orchidopexy was performed in 4 children, 3 of them less than 6 years 6 months of age. Fertility of these early operated children remains to be established, as all adults reported in the literature remain sterile when orchidopexy was not performed or was performed after age 6.

Abnormalities, Multiple↗
PubMed1993

The prune belly syndrome: triad syndrome.

The prune belly syndrome is described in a 2 month old male infant who presented with abdominal distension since birth to Paediatrics Department, Gonder College of Medical Science Hospital, in November 1991. The clinical and radiological presentations and theories concerning the genesis of the syndrome are briefly discussed with a review of the literature.

Ethiopia↗
PubMed1989

The outcome of renal transplantation in children with the prune belly syndrome.

Children with the prune belly syndrome are at high risk for renal failure. The effect of the prune belly syndrome on the outcome of renal transplantation was evaluated in a retrospective study in which 8 transplant recipients with this syndrome were randomly matched with 13 control, nondiabetic transplant patients. The parameters evaluated were patient and graft survival, renal function and interval until transplantation. The patients were matched by computer for age at transplantation, date of transplantation, immunosuppressive therapy used and type of donor. Data were analyzed by the Gehan test. There was no statistically significant difference in patient deaths (1 versus 3), graft survival (75 versus 69%) or graft function between patients with the prune belly syndrome and controls. Patients with the prune belly syndrome waited a shorter interval for transplantation than did controls (no statistically significant difference) because the distensible abdominal wall characteristic of the syndrome permits placement of an adult kidney in a young child. The prune belly syndrome did not adversely affect the outcome of renal transplantation in these patients.

Adolescent↗
PubMedDate not supplied

[A case of the prune belly syndrome].

Rare heterogeneous syndrome, the prune belly syndrome was first described in 1839 and associates the agenesis of the abdominal musculature with other complex malformations of the genitourinary tract and bilateral cryptorchidism. In this plurimalformative context, the main evolutive problem is represented by the chronic renal insufficiency. The work presents a new case of prune belly syndrome which through its clinical evolutive aspects joins the category of classical cases published in literature.

Humans↗
PubMed1995

[A case of Prune Belly Syndrome. Prenatal diagnosis].

Prune Belly Syndrome is a rare and complicated condition affecting the genitourinary organs and abdominal wall, it was named after the aspect of the abdomen after the bladder has been drained. In its fully developed form presents with the triad: megalocyst, abdominal muscle deficiency and cryptorchidism. We present a case of a patient with 22 weeks of gestation with ultrasonographic diagnosis of a large thoracoabdominal cyst. The delivery was by cesarean operation. We analyzed the literature.

Abnormalities, Multiple↗
PubMed2001

Bidirectional Glenn shunt in an infant with prune-belly syndrome.

The prune-belly syndrome (PBS) usually is described as a deficiency of the anterior abdominal muscle involving bilateral cryptorchidism and urinary tract malformations. In this report, we will present an eleven-month-old boy with PBS associated with a complex cardiac anomaly. A bilateral bidirectional Glenn shunt was performed with the diagnosis of isolated dextrocardia, single ventricle, pulmonary atresia, incomplete A-V septal defect, hemiazygos continuity, persistent right superior vena cava, patent ductus arteriosus-dependent pulmonary blood flow. The patient required special consideration for postoperative pulmonary care.

Abdominal Muscles↗
PubMed1990

A possible relationship between Beckwith-Wiedemann syndrome, urinary tract anomaly and prune belly syndrome.

We report here two siblings diagnosed as having Beckwith-Wiedemann syndrome. In addition to Beckwith-Wiedemann syndrome, one of the siblings was also diagnosed with prune belly syndrome. The other sibling suffered from obstructive uropathy and unilateral cryptorchidism, which are also seen in prune belly syndrome. We believe that these two cases point to a potential association between Beckwith-Wiedemann syndrome, prune belly syndrome, and urinary tract anomaly.

Adult↗
PubMed1977

The early assessment for individualized treatment in the prune belly syndrome.

Patients with prune belly syndrome present a spectrum of abnormality, both in the abdominal wall and the urinary tract. Ureteral pathology has characteristic features and the ureter may be more severely involved at the bladder end than in its upper portion. Early neonatal investigation is required to determine which patient can be treated in a conservative manner and which require neonatal reconstruction or temporary vesical or upper tract drainage.

Abdominal Muscles↗