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Prune Belly Syndrome a Focused Physical Assessment Amanda G 10542-05a_ANC703-Woods.qxd 5/31/07 3:33 PM Page 132 LINDA MERRITT, MSN, RNC • Section Editor 3.5 HOURS Continuing Education Prune Belly Syndrome A Focused Physical Assessment Amanda G. Woods, RN, MSN, CNNP,1,2 Debra H. Brandon, RN, PhD1,3 ABSTRACT Prune belly syndrome, a rare congenital anomaly, exists almost exclusively in males and consists of genital and urinary abnormalities with partial or complete absence of abdominal wall musculature. The syndrome, caused by urethral obstruction early in development, is the result of massive bladder distention and urinary ascites, leading to degeneration of the abdominal wall musculature and failure of testicular descent. The impaired elimination of urine from the bladder leads to oligohydramnios, pulmonary hypoplasia, and Potter’s facies. The syndrome has a broad spectrum of affected anatomy with different levels of severity. The exact etiology of prune belly syndrome is unknown, although several embry- ologic theories attempt to explain the anomaly. With advances in prenatal screening techniques, the diagnosis and pos- sible treatment of prune belly syndrome can occur before birth, although controversy exists on the proper management of prune belly syndrome. This article explores the theories behind the pathophysiology and embryology of prune belly syndrome and its multisystemic effects on the newborn infant. Specific attention is paid to presentation, clinical fea- tures, head-to-toe physical assessment, family support, and nursing care of infants with prune belly syndrome. KEY WORDS: congenital absence of abdominal musculature, deficiency of abdominal musculature, Eagle-Barrett syndrome, prune belly syndrome, triad syndrome he most common cause of perinatal and infant in hydronephrosis (dilation of the urinary tract), and mortality in the United States is congenital ultimately can cause renal parenchymal damage. anomalies. Advances in prenatal screening The level of severity depends on the duration T 1 techniques have increased earlier diagnosis of con- and area of obstruction. This article focuses on genital anomalies. Obstructive uropathy, an obstruc- prune belly syndrome and discusses the underlying tion in the urinary tract, includes obstructions such obstruction. as ureteropelvic junction (UPJ), ureterovesical junc- The underlying etiology of the urinary tract obstruc- tion (UVJ), posterior urethral valves (PUV), vesi- tion and the degree of pulmonary hypoplasia influ- coureteral reflux (VUR), urethral atresia/stricture, ence the survival and outcome of infants with prune duplicating collecting system, multicystic dysplastic belly syndrome. Management of these infants depends kidney, and prune belly syndrome. Obstructions can on a functioning urinary tract system and the extent occur at different levels of the urinary tract, resulting of associated symptoms. Understanding the patho- physiology provides a foundation for nursing care of infants with prune belly syndrome and provides Address correspondence to Amanda G. Woods, Women’s accurate information to parents for making decisions Hospital of Greensboro, 801 Green Valley Road, about treatment of their infant. Although rare, anom- Greensboro, NC 27407. alies such as prune belly syndrome are often difficult E-mail: [email protected] to manage because of their complexity.2 Prune belly From 1Duke University School of Nursing, Durham, NC; syndrome, also known as Eagle-Barrett syndrome, 2Women’s Hospital, Greensboro, NC; and 3Duke University comprises a triad of anomalies that include abdom- Health System, Durham, NC. inal wall flaccidity, urologic abnormalities, and bi- Copyright © 2007 by the National Association of lateral cryptorchidism. The characteristic wrinkled, Neonatal Nurses. prune-like abdomen of the infant gives the syndrome The authors have no significant ties, financial or otherwise, to its name. In addition, cardiovascular, respiratory, any company that might have an interest in the publication orthopedic, and gastrointestinal anomalies can accom- of this educational activity. pany prune belly syndrome.3 132 Advances in Neonatal Care • Vol. 7, No. 3 • pp. 132-143 10542-05a_ANC703-Woods.qxd 5/31/07 3:33 PM Page 133 Prune Belly Syndrome: A Focused Physical Assessment 133 INCIDENCE the primitive urogenital sinus, and the posterior part becomes the anorectal canal. The urogenital sinus The incidence of prune belly syndrome is between has 3 parts. The vesicle part will form the bladder; the 1 in 29,000 to 1 in 40,000 live male births.1 Males are pelvic part will form the urethra in females and the affected 20 times more often than females, with approx- urethra in the bladder neck and prostatic urethra in imately 3% of all cases occurring in females. Infants males; and the phallic part will eventually grow born to younger mothers are at greater risk for having toward the genital tubercle.8 In infants, the urinary prune belly syndrome.4,5 The incidence of prune belly bladder is located in the abdomen, entering the pelvis syndrome in twins is 4 times greater than that found in at about 6 years of age.8 single births, at 12.2 per 100,000 live births.6 An important factor in fetal development and growth is amniotic fluid. At first, amniotic fluid comes ETIOLOGY OF PRUNE BELLY SYNDROME from maternal tissue and fluid that moves across the amniochorionic membrane from decidua parietalis The exact etiology of prune belly syndrome is un- through diffusion. Soon after, fluid moves through known; however, several theories exist to explain the the chorionic plate from the blood of the placenta syndrome.7 To understand the theories behind prune also by diffusion. Amniotic fluid is also comprised of belly syndrome, an understanding of normal geni- fluid secreted by the fetal respiratory tract, known as tourinary development is essential. Development of fetal lung fluid. Urine production begins between 9 the urogenital system is divided into the urinary and and 10 weeks of gestation and will contribute to the reproductive systems. During the third week of amniotic fluid volume by 11 weeks’ gestation.8,9 At embryonic development, a process called gastrulation 20 weeks’ gestation, fetal urine production is approx- occurs. Gastrulation is the beginning of embryonic imately 0.1 mL/minute and gradually increases to body formation by the development of 3 specific 1 mL/minute by 40 weeks’ gestation.10 During the first germ layers (ectoderm, mesoderm, and endoderm). and second trimesters, electrolytes and osmolality in These 3 germ layers will then give rise to specific fetal urine reflect maternal blood. After the third organs and tissues. One of the germ layers, the meso- trimester, sodium and chloride absorption results derm, will give rise to urinary and reproductive organs. from a more hypotonic urine and amniotic fluid.11 The urinary system begins development at approxi- Consequently, if an obstruction or arrest occurs during mately 3 weeks’ gestation and occurs before the geni- renal development, urine production is low, resulting tal system is evident, although, embryological develop- in low amniotic fluid levels (oligohydramnios). After ment between these 2 systems is closely related. Four 20 weeks’ gestation, urine excretion by the kidneys is components comprise the urinary system:8,9 responsible for approximately 90% of amniotic fluid • kidneys—primary role is urine excretion volume.1 • ureters—primary role is transport of urine from Lack of urine production or excretion leads to oligo- the kidneys to the bladder hydramnios, a decreased amount, or a lack of, amni- • bladder—primary role is storage of urine until otic fluid. This leads to a number of findings in the elimination infant. Fetal deformation caused by compression of • urethra—primary role is to carry urine from the the abdominal wall may cause characteristic facial bladder to be eliminated features and contraction of extremities. Adequate lev- The kidneys develop first through a series of 3 con- els of amniotic fluid are also fundamental for normal secutive steps. The first kidneys, called pronephroi, pulmonary development. Infants with prune belly appear between weeks 3 and 4 of gestation. They are syndrome who have not undergone antenatal med- essentially nonfunctioning kidneys represented by a ical interventions to divert urine may present with cluster of cells in the neck region and usually disap- pulmonary hypoplasia, depending on the degree of pear by the end of the fourth week of gestation. Next, urinary obstruction.12 mesonephroi develop around the fourth week of ges- Complications associated with prune belly syn- tation and serve as a temporary excretion organ until drome originate during embryonic and fetal develop- the permanent kidneys develop. The mesonephroi ment. Infants with prune belly syndrome who have begin to disappear by 8 weeks of gestation. Finally, urethral obstructions can have a patent urachus, which the metanephroi develop by the fifth week of gesta- allows elimination of urine from the body.1 Normally, tion and become the functioning permanent kidneys the urachus develops from the urogenital sinus with by approximately 9 weeks’ gestation. Initially, the the superior portion of the sinus developing into the kidneys are located in the pelvis; as the fetus grows, bladder and urachus and the inferior portion of the they gradually ascend to their proper location in the sinus developing into the urethra. During the fourth abdomen.8 to fifth months of gestation, the urachus obliterates. The bladder and urethra develop following a divi- If the urachus fails to obliterate, a fibrous cord that sion of the cloaca by the urorectal septum between 5 stretches from the bladder to the umbilicus exists and to 6 weeks of gestation. The anterior part becomes allows urine to escape through the umbilical cord. Advances in Neonatal Care • Vol. 7, No. 3 10542-05a_ANC703-Woods.qxd 5/31/07 3:33 PM Page 134 134 Woods and Brandon Other complications that can originate during embry- found in one male with an early urethral obstruction onic development include renal agenesis, horseshoe that had a large interstitial deletion located on the long kidney, duplications of the urinary tract, ectopic ureter, arm of chromosome 6 [del 6 (q15-q2.33)].
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