Dental treatment of patients with prune belly syndrome.

BACKGROUND: Prune belly syndrome (PBS), also known as Eagle-Barrett syndrome (EGBRS), is a rare congenital disease characterized by deficiency or absence of abdominal wall muscles, urological abnormalities, and bilateral cryptorchidism. TYPES OF STUDIES REVIEWED: A review of literature was done using four search engines (PubMed, Google Scholar, Scopus, Science Direct) and keywords (individually and in combinations): prune belly syndrome, PBS, Eagle-Barrett syndrome, dental manifestation, clinical manifestation, and psychological aspects. The search was run with no language restrictions and covered the 1965-2021 time period. RESULTS: The search yielded a large number of articles. The vast majority were dealing with a variety of treatments. PBS is a multisystem disease with a variable spectrum ranging from mild cases to infant mortality. Comorbidities of PBS (63% gastrointestinal, 65% orthopedic, and 49% cardiopulmonary) present challenges for treatment. PBS affects quality of life of patients and caregivers. We selected and summarized published information that is relevant to oral health and dental care. CONCLUSIONS AND PRACTICAL IMPLICATIONS: Providing information to dental practitioners will improve their understanding of PBS. It will help them to better treat patients with PBS and it will encourage more dental providers to welcome patients with PBS into their dental clinics.

Modern management of and update on prune belly syndrome.

Prune belly syndrome (PBS) is characterized by the triad of abdominal flaccidity, a variable degree of urinary tract involvement and cryptorchidism. Most cases of PBS are sporadic and have a normal karyotype, with 95% patients being male. In the last decade, mutations in known genes that regulate embryonic genitourinary myogenesis have been identified and with increasing knowledge of these critical genes involved in bladder maldevelopment, advances can be made in genetic counseling. A multidisciplinary approach is necessary and individualization of care is recommended according to phenotypic severity. Some patients require abdominal and urinary tract reconstruction while others require as little as bilateral orchiopexies. Major treatment objectives are: preservation of renal function and upper urinary tract; polyuria management; adequate bladder emptying; improvement of corporal image and quality of life; preservation of fertility and adequate sexual function. Long-term surveillance of the urinary tract is essential up to adulthood, because functional dynamics can change over time.

Respiratory support in the absence of abdominal muscles: A case study of ventilatory management in prune belly syndrome.

Prune belly syndrome (PBS) results in a total lack of abdominal musculature. Abdominal muscles have an important function during inspiration and expiration. This puts the patient at risk for respiratory complications since they have a very limited ability to cough up secretions. Patients in an intensive care unit (ICU) with PBS who receive mechanical ventilation are at even greater risk for respiratory complications. We review the function of the abdominal muscles in breathing and delineate why they are important in the ICU. We include an illustrative case of a long-term ventilated patient with PBS and offer respiratory management options.

Síndrome del abdomen en ciruela pasa / Prune belly syndrome

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Management of Congenital Urethral Strictures In Infants. Case Series.

PURPOSE: Infra-vesical obstruction is uncommon in infants and generally due to urethral valves. Congenital urethral strictures (CUS), instead, defined as a concentric narrowing of the urethral lumen, are exceedingly rare in infants. MATERIALS AND METHODS: We reviewed our experience with 7 patients treated at our institution for CUS

Diastasis recti abdominis - a review of treatment methods.

Diastasis recti abdominis is a condition in which both rectus abdominis muscles disintegrate to the sides, this being ac-companied by the extension of the linea alba tissue and bulging of the abdominal wall. DRA may result in the herniation of the abdominal viscera, but it is not a hernia per se. DRA is common in the female population during pregnancy and in the postpartum period. There is a scant knowledge on the prevalence, risk factors, prevention or management of the abovemen-tioned condition. The aim of this paper is to present the methods of DRA treatment based on the results of recent studies.

Three cases of prune belly syndrome at the Lagos State University Teaching Hospital, Ikeja.

Prune belly syndrome (PBS) is a rare congenital disorder affecting 2.5 to 3.8/100,000 live births worldwide. Our objective of this report is to describe clinical manifestation, laboratory, and radiological characteristics of PBS in our patients, to highlight the limitations to offering appropriate patient care due to parents demanding discharge against medical advice and the need to increase the awareness regarding this rare disease. We report three cases; all referred after birth with lax abdominal wall, congenital anomalies of kidney, and urinary tract. One of the patients had an absent right foot. They all had cryptorchidism, and in one, there was deranged renal function. The reported cases had both medical and radiological interventions to varying degrees. They all had an abdominal ultrasound which revealed varying degrees of hydronephrosis, hydroureters, and bladder changes. Voiding cystourethrogram showed vesicoureteric reflux in one of the reported cases. Urinary tract infections were appropriately treated with antibiotics based on sensitivity. PBS management in our setting remains a challenge because of strong cultural beliefs, and high rate of discharge against medical advice. Focus should be on parent education, early diagnosis, and multidisciplinary management approach.

Unusual presentation of prune belly syndrome: a case report.

BACKGROUND: Prune belly syndrome is a rare congenital malformation of unknown etiology, with the following triad of findings: abdominal muscle wall weakness, undescended testes, and urinary tract abnormalities. In most cases, detection of prune belly syndrome occurs during neonatal or infancy period. In this case report, we describe a 12-year-old boy from Ethiopia with the triad of findings of prune belly syndrome along with skeletal malformations. We are unaware of any previous report of prune belly syndrome in Ethiopia. CASE PRESENTATION: A 12-year-old Amhara boy from the Northwest Gondar Amhara regional state presented to our referral hospital with a complaint of swelling over his left flank for the past 3 months. Maternal pregnancy course and medical history were noncontributory, and he had an attended birth at a health center. He has seven siblings, none of whom had similar symptoms. On examination he had a distended abdomen, asymmetric with bulging left flank, visible horizontal line, upward umbilical slit, and absent rectus abdominis muscles. His abdomen was soft with a tender cystic, bimanually palpable mass on the left flank measuring 13 × 11 cm. Both testes were undescended and he also has developmental dysplasia of the hips. An abdominal ultrasound revealed a large cystic mass in his left kidney area with echo debris and a hip X-ray showed bilateral developmental dysplasia of the hip. Intraoperative findings were cystic left kidney, both testes were intraperitoneal, tortuous left renal vein, enlarged bladder reaching above umbilicus, and left megaureter. INTERVENTIONS: bilateral orchidectomy and left nephrectomy were done. He was given intravenously administered antibiotics for treatment of pyelonephritis and discharged home with an appointment for follow up and possible abdominoplasty. CONCLUSIONS: In the current report delayed presentation contributed to testicular atrophy and decision for orchidectomy. Furthermore, he will be at potential risk for sex hormone abnormality. Therefore, diagnosis of prune belly syndrome in resource-limited settings requires a high index of suspicion. We recommend further research to determine the optimal management and early diagnosis of prune belly syndrome in resource-limited settings.

Corset Usage for Gastrointestinal and Respiratory Problems in a Newborn with Prune Belly Syndrome.

Prune Belly syndrome (PBS), comprises a triad of anomalies that include abdominal wall flaccidity, urologic anomalies and bilateral cryptorchidism in males. The abdominal musculature hypoplasia predisposes to respiratory problems, respiratory infections secondary to impaired cough mechanism, and cause chronic constipation secondary to ineffective valsalva ability. Here, the authors present a newborn baby with Prune Belly syndrome who had respiratory and gastrointestinal problems which resolved after corset use. To the authors knowledge, this is the first case of corset usage in the treatment of PBS in a newborn infant.

[Diastasis Recti Abdominis and Pregnancy]. / Rektusdiastase und Schwangerschaft.

Diastasis recti abdominis during pregnancy is a frequent phenomenon with a prevalence of 30­70%. It is associated with functional and cosmetic limitations. Gold standard in diagnosis during pregnancy is the transabdominal ultrasonography. The most frequent localization is in the periumbilical region and persistence postpartum is found in about 60% of cases. Either conservative or surgical treatments seem to be effective, but relapse rates, especially after surgical therapy, are unclear. It is a problem that no standard values of diastasis recti are given, no evidence-based therapy schemes are implemented and only rare and insufficient studies exist.

Normal live births after intracytoplasmic sperm injection in a man with the rare condition of Eagle-Barrett syndrome (prune-belly syndrome).

OBJECTIVE: To report the first live births of male infants resulting from intracytoplasmic sperm injection (ICSI) using spermatozoa from a man with Eagle-Barrett syndrome (EBS). DESIGN: Case report. SETTING: Assisted conception unit within a private hospital. PATIENT(S): An infertile couple. INTERVENTIONS: An infertile couple received repeated treatment with ICSI. MAIN OUTCOME MEASURE(S): Clinical pregnancy and a normal live birth. RESULT(S): In 2008, after microinjection of ten oocytes, the transfer of a single expanded blastocyst led to the premature birth of a morphologically normal male infant at 18 weeks' gestation. This outcome followed preterm rupture of membranes and possible cervical incompetence. In 2009, after microinjection of six oocytes, transfer of a single 5-cell embryo led to a singleton pregnancy, with emergency cervical cerclage being performed at 21 weeks. A healthy male infant was born at 30 weeks, with no evidence of EBS, by lower-segment cesarean section for breech presentation and premature labor. In 2012, after elective laparoscopic placement of cervical suture, microinjection of ten oocytes and transfer of a single 4-cell embryo led to a singleton pregnancy with a healthy male infant, with no evidence of EBS, being born by cesarean section at 38 weeks. CONCLUSION(S): This report suggests that EBS is not transmitted to male offspring via ICSI.

A case of ultrasound-guided prenatal diagnosis of prune belly syndrome in Papua New Guinea--implications for management.

BACKGROUND: Prune belly syndrome is a rare congenital malformation of unknown aetiology and is characterised by abnormalities of the urinary tract, a deficiency of abdominal musculature and bilateral cryptorchidism in males. We report a case of prune belly syndrome from Papua New Guinea, which was suspected on pregnancy ultrasound scan and confirmed upon delivery. CASE PRESENTATION: A 26-year-old married woman, Gravida 3 Para 2, presented to antenatal clinic in Madang, Papua New Guinea, at 21(+5) weeks' gestation by dates. She was well with no past medical or family history of note. She gave consent to participate in a clinical trial on prevention of malaria in pregnancy and underwent repeated ultrasound examinations which revealed a live fetus with persistent megacystis and anhydramnios. Both mother and clinicians agreed on conservative management of the congenital abnormality. The mother spontaneously delivered a male fetus weighing 2010 grams at 34 weeks' gestation with grossly abnormal genitalia including cryptorchidism, penile aplasia and an absent urethral meatus, absent abdominal muscles and hypoplastic lungs. The infant passed away two hours after delivery. This report discusses the implications of prenatal detection of severe congenital abnormalities in PNG. CONCLUSION: This first, formally reported, case of prune belly syndrome from a resource-limited setting in the Oceania region highlights the importance of identifying and documenting congenital abnormalities. Women undergoing antenatal ultrasound examinations must be carefully counseled on the purpose and the limitations of the scan. The increasing use of obstetric ultrasound in PNG will inevitably result in a rise in prenatal detection of congenital abnormalities. This will need to be met with adequate training, referral mechanisms and better knowledge of women's attitudes and beliefs on birth defects and ultrasound. National medicolegal guidance regarding induced abortion and resuscitation of a fetus with severe congenital abnormalities may be required.

Familial prune belly syndrome in a Nigerian family.

A case of Prune Belly Syndrome in an infant, the second in a middle class family with both parents in their late thirties, is presented because of its rarity. Constraints in the management are discussed and relevant literature reviewed. This is intended to awaken interest and sharpen indices of suspicion that would facilitate early diagnosis, enhance management, and mitigate prejudices.

Urethral hydrodistension for management of urethral hypoplasia in prune belly syndrome: long-term results.

PURPOSE: The aim of the study was to evaluate the efficacy and safety of urethral hydrodistension for management of urethral hypoplasia in prune belly syndrome (PBS). METHODS: During a 10-year period, 7 infants with PBS and urethral hypoplasia presented either with open urachus or surgically created urinary diversion referred to our hospital. Five milliliters of normal saline was pushed via a 22-gauge plastic angiocatheter into the urethra with simultaneous finger pressure on the perineum to occlude the proximal urethra that was repeated with higher volumes of the solution (up to 20 mL). The procedure was continued until a 6F or 8F feeding tube catheter confirmed the urethral patency. Hydrodistension was repeated in 3-month intervals till complete patency was confirmed by imaging. RESULTS: Median age of the infants was 6 (1-8) months. All urethral hydrodistension were successful after 1 to 3 sessions. Follow-up imaging studies showed significant improvement in all patients except one. Natural and surgically created urinary diversions were closed in 6 infants. CONCLUSIONS: The hydrodistension create an equal and constant pressure into the urethral wall without any urethral damage. This technique can be considered along with the other available methods for management of urethral hypoplasia in selected cases of PBS.

Intraosseous route as alternative access for infusion therapy.

The safe and effective use of intraosseous (IO) access is evolving from its historical use only for emergent access for pediatric patients to use in adult trauma patients and, more recently, for adult inpatients. Current data and research support the use of IO route for infusion in patients of any age as a safe, rapid, and equally effective alternative to intravenous access. The article provides an overview of IO access indications, care, and management; describes therapies administered via IO access; and discusses the expanding use of IO access into areas within hospitals during nonemergent clinical situations.

Contemporary epidemiology and characterization of newborn males with prune belly syndrome.

OBJECTIVES: Prune belly syndrome (PBS) is a rare condition characterized by the congenital absence or deficiency of the abdominal wall musculature, with associated abnormalities of the genitourinary tract, including hydronephrosis and cryptorchidism. Few population-based epidemiology or mortality data are available. METHODS: We retrospectively reviewed the Kids' Inpatient Database to evaluate PBS among newborn infants during their initial hospitalization in 2000, 2003, and 2006. The International Classification of Diseases, Ninth Revision, Clinical Modification codes were used to identify patients and to determine the comorbidity status. The PBS incidence, demographics, comorbid conditions, and disposition were assessed. RESULTS: A total of 133 newborn male infants diagnosed with PBS were identified of 1,420,991 live male births, for a weighted incidence estimate of 3.8 cases/100,000 live births. Of the newborns with PBS, 50% were white, 31% black, and 10% were Hispanic. In-hospital mortality was high (39 of 133, 29%). Of the 133 patients with PBS, 55 (41%) were discharged home and 39 (29%) required inpatient transfer or home nursing care. Fifty-seven patients (43%) were born premature; 56% of the PBS deaths occurred in premature infants. Mechanical ventilation was required in 64 newborns (48%), and 33 (24%) had coexisting congenital cardiovascular anomalies. Renal failure was uncommon, occurring in only 5 newborns (4%); none required dialysis. Only 13 patients (10%) underwent urinary diversion (vesicostomy or ureterostomy). CONCLUSIONS: The incidence of PBS was 3.8 cases/100,000 live births. Despite advances in care for children with PBS, this condition continues to be associated with high perinatal mortality, likely related to the associated prematurity and pulmonary complications. Renal failure was rare, as was immediate urinary diversion.

Síndrome de Prune Belly / Prune Belly Syndrome

Información sobre este síndrome congénito, en el que se presenta agenesia, atrofia o hipoplasia de los músculos de la pared abdominal, anomalías en el sistema urinario, criptorquidia bilateral, y otras malformaciones secundarias. Se considera a la ecografía el primer método de diagnóstico por imágenes que se debe efectuar inmediatamente al nacimiento, y a la vez se destaca la importancia del diagnóstico prenatal precoz.