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January 1995, Vol 149, No. 1 >
Article | January 1995

A Descriptive Study of Prune Belly in New York State, 1983 to 1989 FREE

Charlotte M. Druschel, MD, MPH
Arch Pediatr Adolesc Med. 1995;149(1):70-76. doi:10.1001/archpedi.1995.02170130072017.
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Objective:  To determine the prevalence and spectrum of prune belly in a defined population.

Design:  Population-based descriptive study using New York State's Congenital Malformations Registry.

Setting:  The Congenital Malformations Registry is a statewide registry of children diagnosed as having congenital anomalies before the age of 2 years.

Patients:  Infants with the diagnosis of prune-belly syndrome born during the years 1983 to 1989 to women who were New York State residents and verified by medical record review.

Main Outcome Measures:  The live birth prevalence of prune belly for the total population and for population subgroups, such as race, sex, plurality, and maternal age. The occurrence of other malformations with prune belly.

Results:  Sixty cases of prune belly were ascertained (50 male and 10 female). The live birth prevalence was 3.2 per 100 000 and declined over the time period. The prevalence was higher in males, 5.1 per 100 000, than females, 1.1; and higher in blacks, 5.8, than whites, 2.6. The live birth prevalence of prune belly in twins, 12.2 per 100 000, was four times higher than that found in singletons, 3.0. More than 36 (60%) of patients died, most in the first week. Forty-two (70%) of patients had one of the commonly described associated defects; pulmonary hypoplasia was the most common. Almost one third of patients had defects other than those typically associated with prune belly.

Conclusions:  Twins, blacks, and children born to younger mothers appear to be at higher risk. Mortality remains high, especially early with many deaths due to pulmonary hypoplasia. Further studies should include stillborns and terminated pregnancies.(Arch Pediatr Adolesc Med. 1995;149:70-76)

REFERENCES

1 +
Pagon RA, Smith DW, Shepard TH.  Urethral obstruction malformation complex: a cause of abdominal muscle deficiency and the 'prune belly.' J Pediatr . 1979;;94:900-906.
2 +
Nakayama DK, Harrison MR, Chinn DH, de Lorimier AA.  The pathogenesis of prune belly . AJDC . 1984;;138:834-836.
3 +
Wigger HJ, Blanc WA.  The prune belly syndrome . Pathol Annu . 1977;;12:17-39.
4 +
Jones KJ. Smith's Recognizable Patterns of Human Malformation . Philadelphia, Pa: WB Saunders Co; 1988;:562-565.
5 +
Beluffi G, Brokensha C, Kozlowski K, et al.  Congenital cystic adenomatoid malformation of the lung: presentation of 16 cases . Fortschr Rontgenstr . 1989;; 150;523-530.
6 +
Kuruvilla AC, Kesler KR, Williams JW, McGee MJ.  Congenital cystic adenomatoid malformation of the lung associated with prune belly syndrome . J Pediatr Surg . 1987;;22:370-371.
7 +
Woodard JR.  The prune belly syndrome . Urol Clin North Am . 1978;;5:75-93.
8 +
Graham JM. Smith's Recognizable Patterns of Human Deformation . Philadelphia, Pa: WB Saunders Co; 1988;:102-105.
9 +
Baird PA, MacDonald EC.  An epidemiologic study of congenital malformations of the anterior abdominal wall in more than half a million consecutive live births . Am J Hum Genet . 1981;;33:470-478.
10 +
New York State Department of Health. Congenital Malformations Registry Annual Report, 1983 Birth Cohort . Albany, NY: Congenital Malformations Registry; 1983;.
11 +
Winer-Muram HT, Tonkin ILD.  The spectrum of heterotaxic syndromes . Radiol Clin North Am . 1989;;27:1147-1170.
12 +
Kleinbaum DG, Kupper LL, Muller KE. Applied Regression Analysis and Other Multivariable Methods . Boston, Mass: PWS-Kent; 1988;:497-519.
13 +
Edmonds LD, Layde PM, James LM, Flynt JW, Erickson JD, Oakley GP.  Congenital malformations surveillance: two American systems . Int J Epidemiol . 1981;;10:247-252.
14 +
Baird PA.  Measuring birth defects and handicapping disorders in the population: the British Columbia Health Surveillance Registry . Can Med Assoc J . 1987;; 136:109-111.
15 +
Moore RM, Jeng LL, Kaczmarek RG, Placek PJ.  Use of diagnostic imaging procedures and fetal monitoring devices in the care of pregnant women . Public Health Rep . 1990;;105:471-475.
16 +
Burke EC, Shin MH, Kelalis PP.  Prune belly syndrome: clinical findings and survival . AJDC . 1969;;117:668-671.
17 +
Geary DF, MacLusky IB, Churchill BM, McLorie G.  A broader spectrum of abnormalities in the prune belly syndrome . J Urol . 1986;;135:324-326.
18 +
Woodhouse CRJ, Ransley PG, Innes-Williams D.  Prune belly syndrome—report of 47 cases . Arch Dis Child . 1982;;57:856-859.
19 +
Welch KJ, Kearney GP.  Abdominal musculature deficiency syndrome: prune belly . J Urol . 1974;;111:693-700.
20 +
Hoagland MH, Hutchins GM.  Obstructive lesions of the lower urinary tract in the prune belly syndrome . Arch Pathol Lab Med . 1987;;111:154-156.
21 +
Ives EJ.  The abdominal muscle deficiency triad syndrome—experience with 10 cases . In: Bergsma D, ed. The Clinical Delineation of Birth Defects . Baltimore, Md: Williams & Wilkins for the National Foundation—March of Dimes BD:OAS; 1974;;10:127-135.
22 +
Petersen DS, Fish L, Cass AS.  Twins with congenital deficiency of abdominal musculature . J Urol . 1972;;107:670-672.
23 +
Garlinger P, Ott J.  Prune belly syndrome: possible genetic implications . Clin Cytogenet Genet . 1974;;10:173-180.
24 +
Chitkara U, Berkowitz RL.  Multiple gestations . In: Gabbe SG, Niebyl JR, Simpson JL, eds. Obstetrics: Normal and Problem Pregnancies . New York, NY: Churchill Livingstone Inc; 1991;;881-921.
25 +
Schinzel AAGL, Smith DW, Miller JR.  Monozygotic twinning and structural defects . J Pediatr . 1979;;95:921-930.
26 +
Van Allen Ml.  Fetal vascular disruptions: mechanisms and some resulting birth defects . Pediatr Ann . 1981;;10:219-233.
27 +
Van Allen Ml, Smith DW, Shepard TH.  Twin reversed arterial profusion (TRAP) sequence: a study of 14 twin pregnancies with acardius . Semin Perinatol . 1983;; 7:285-293.
28 +
Lubinsky MS.  Female pseudohermaphroditism and associated anomalies . Am J Med Genet . 1980;;6:123-136.
29 +
Lubinsky M, Doyle K, Trunca C.  The association of a prune belly with Turner's syndrome . AJDC . 1980;;134:1171-1172.
30 +
McKeown CME, Donnai D.  Prune belly in trisomy 13 . Prenat Diagn . 1986;;6: 379-381.
31 +
Frydman M, Magenis RE, Mohandas TK, Kaback MM.  Chromosome abnormalities in infants with prune belly anomaly: association with trisomy 18 . Am J Med Genet . 1983;;15:145-148.
32 +
Scarbrough PR, Files B, Carroll AJ, Quinlan RW, Finley SC, Finley WH.  Interstitial deletion of chromosome 1 [del(1)(q25q32)] in an infant with prune belly sequence . Prenat Diagn . 1988;;8:169-174.
33 +
Amacker EA, Grass FS, Hickey DE, Hisley JC.  Brief clinical report: an association of prune belly anomaly with trisomy 21 . Am J Med Genet . 1986;;23:919-923.
34 +
Goldbaum G, Daling J, Milham S.  Risk factors for gastroschisis . Teratology . 1990;;42:397-403.
35 +
Roeper PJ, Harris J, Lee G, Neutra R.  Secular rates and correlates for gastroschisis in California (1968-1977) . Teratology . 1987;;35:203-210.
36 +
Martinez-Frias ML, Salvador J, Prieto L, Zaplana J.  Epidemiological study of gastroschisis and omphalocele in Spain . Teratology . 1984;;29:377-382.
37 +
Hemminki K, Saloniemi I, Kyyronen P, Kekomaki M.  Gastroschisis and omphalocele in Finland in the 1970's: prevalence at birth and its correlates . J Epidemiol Community Health . 1982;;36:289-293.
38 +
Czeizel A, Vitez M.  Etiological study of omphalocele . Hum Genet . 1981;;58:390-395.
39 +
Fraser FC, Cziezel A, Hanson C.  Increased frequency of neural tube defects in sibs of children with other malformations . Lancet . 1982;;2:144-145.
40 +
MRC Vitamin Study Research Group.  Prevention of neural tube defects: results of the Medical Research Council Vitamin Study . Lancet . 1991;;338:131-137.
41 +
Centers for Disease Control and Prevention.  Recommendations for the use of folic acid to reduce the number of cases of spina bifida and other neural tube defects . MMWR Morb Mortal Wkly Rep . 1992;;41(No. (RR-14) ):1-7.

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Country-Specific Mortality and Growth Failure in Infancy and Yound Children and Association With Material Stature

Use interactive graphics and maps to view and sort country-specific infant and early dhildhood mortality and growth failure data and their association with maternal

References

1 +
Pagon RA, Smith DW, Shepard TH.  Urethral obstruction malformation complex: a cause of abdominal muscle deficiency and the 'prune belly.' J Pediatr . 1979;;94:900-906.
2 +
Nakayama DK, Harrison MR, Chinn DH, de Lorimier AA.  The pathogenesis of prune belly . AJDC . 1984;;138:834-836.
3 +
Wigger HJ, Blanc WA.  The prune belly syndrome . Pathol Annu . 1977;;12:17-39.
4 +
Jones KJ. Smith's Recognizable Patterns of Human Malformation . Philadelphia, Pa: WB Saunders Co; 1988;:562-565.
5 +
Beluffi G, Brokensha C, Kozlowski K, et al.  Congenital cystic adenomatoid malformation of the lung: presentation of 16 cases . Fortschr Rontgenstr . 1989;; 150;523-530.
6 +
Kuruvilla AC, Kesler KR, Williams JW, McGee MJ.  Congenital cystic adenomatoid malformation of the lung associated with prune belly syndrome . J Pediatr Surg . 1987;;22:370-371.
7 +
Woodard JR.  The prune belly syndrome . Urol Clin North Am . 1978;;5:75-93.
8 +
Graham JM. Smith's Recognizable Patterns of Human Deformation . Philadelphia, Pa: WB Saunders Co; 1988;:102-105.
9 +
Baird PA, MacDonald EC.  An epidemiologic study of congenital malformations of the anterior abdominal wall in more than half a million consecutive live births . Am J Hum Genet . 1981;;33:470-478.
10 +
New York State Department of Health. Congenital Malformations Registry Annual Report, 1983 Birth Cohort . Albany, NY: Congenital Malformations Registry; 1983;.
11 +
Winer-Muram HT, Tonkin ILD.  The spectrum of heterotaxic syndromes . Radiol Clin North Am . 1989;;27:1147-1170.
12 +
Kleinbaum DG, Kupper LL, Muller KE. Applied Regression Analysis and Other Multivariable Methods . Boston, Mass: PWS-Kent; 1988;:497-519.
13 +
Edmonds LD, Layde PM, James LM, Flynt JW, Erickson JD, Oakley GP.  Congenital malformations surveillance: two American systems . Int J Epidemiol . 1981;;10:247-252.
14 +
Baird PA.  Measuring birth defects and handicapping disorders in the population: the British Columbia Health Surveillance Registry . Can Med Assoc J . 1987;; 136:109-111.
15 +
Moore RM, Jeng LL, Kaczmarek RG, Placek PJ.  Use of diagnostic imaging procedures and fetal monitoring devices in the care of pregnant women . Public Health Rep . 1990;;105:471-475.
16 +
Burke EC, Shin MH, Kelalis PP.  Prune belly syndrome: clinical findings and survival . AJDC . 1969;;117:668-671.
17 +
Geary DF, MacLusky IB, Churchill BM, McLorie G.  A broader spectrum of abnormalities in the prune belly syndrome . J Urol . 1986;;135:324-326.
18 +
Woodhouse CRJ, Ransley PG, Innes-Williams D.  Prune belly syndrome—report of 47 cases . Arch Dis Child . 1982;;57:856-859.
19 +
Welch KJ, Kearney GP.  Abdominal musculature deficiency syndrome: prune belly . J Urol . 1974;;111:693-700.
20 +
Hoagland MH, Hutchins GM.  Obstructive lesions of the lower urinary tract in the prune belly syndrome . Arch Pathol Lab Med . 1987;;111:154-156.
21 +
Ives EJ.  The abdominal muscle deficiency triad syndrome—experience with 10 cases . In: Bergsma D, ed. The Clinical Delineation of Birth Defects . Baltimore, Md: Williams & Wilkins for the National Foundation—March of Dimes BD:OAS; 1974;;10:127-135.
22 +
Petersen DS, Fish L, Cass AS.  Twins with congenital deficiency of abdominal musculature . J Urol . 1972;;107:670-672.
23 +
Garlinger P, Ott J.  Prune belly syndrome: possible genetic implications . Clin Cytogenet Genet . 1974;;10:173-180.
24 +
Chitkara U, Berkowitz RL.  Multiple gestations . In: Gabbe SG, Niebyl JR, Simpson JL, eds. Obstetrics: Normal and Problem Pregnancies . New York, NY: Churchill Livingstone Inc; 1991;;881-921.
25 +
Schinzel AAGL, Smith DW, Miller JR.  Monozygotic twinning and structural defects . J Pediatr . 1979;;95:921-930.
26 +
Van Allen Ml.  Fetal vascular disruptions: mechanisms and some resulting birth defects . Pediatr Ann . 1981;;10:219-233.
27 +
Van Allen Ml, Smith DW, Shepard TH.  Twin reversed arterial profusion (TRAP) sequence: a study of 14 twin pregnancies with acardius . Semin Perinatol . 1983;; 7:285-293.
28 +
Lubinsky MS.  Female pseudohermaphroditism and associated anomalies . Am J Med Genet . 1980;;6:123-136.
29 +
Lubinsky M, Doyle K, Trunca C.  The association of a prune belly with Turner's syndrome . AJDC . 1980;;134:1171-1172.
30 +
McKeown CME, Donnai D.  Prune belly in trisomy 13 . Prenat Diagn . 1986;;6: 379-381.
31 +
Frydman M, Magenis RE, Mohandas TK, Kaback MM.  Chromosome abnormalities in infants with prune belly anomaly: association with trisomy 18 . Am J Med Genet . 1983;;15:145-148.
32 +
Scarbrough PR, Files B, Carroll AJ, Quinlan RW, Finley SC, Finley WH.  Interstitial deletion of chromosome 1 [del(1)(q25q32)] in an infant with prune belly sequence . Prenat Diagn . 1988;;8:169-174.
33 +
Amacker EA, Grass FS, Hickey DE, Hisley JC.  Brief clinical report: an association of prune belly anomaly with trisomy 21 . Am J Med Genet . 1986;;23:919-923.
34 +
Goldbaum G, Daling J, Milham S.  Risk factors for gastroschisis . Teratology . 1990;;42:397-403.
35 +
Roeper PJ, Harris J, Lee G, Neutra R.  Secular rates and correlates for gastroschisis in California (1968-1977) . Teratology . 1987;;35:203-210.
36 +
Martinez-Frias ML, Salvador J, Prieto L, Zaplana J.  Epidemiological study of gastroschisis and omphalocele in Spain . Teratology . 1984;;29:377-382.
37 +
Hemminki K, Saloniemi I, Kyyronen P, Kekomaki M.  Gastroschisis and omphalocele in Finland in the 1970's: prevalence at birth and its correlates . J Epidemiol Community Health . 1982;;36:289-293.
38 +
Czeizel A, Vitez M.  Etiological study of omphalocele . Hum Genet . 1981;;58:390-395.
39 +
Fraser FC, Cziezel A, Hanson C.  Increased frequency of neural tube defects in sibs of children with other malformations . Lancet . 1982;;2:144-145.
40 +
MRC Vitamin Study Research Group.  Prevention of neural tube defects: results of the Medical Research Council Vitamin Study . Lancet . 1991;;338:131-137.
41 +
Centers for Disease Control and Prevention.  Recommendations for the use of folic acid to reduce the number of cases of spina bifida and other neural tube defects . MMWR Morb Mortal Wkly Rep . 1992;;41(No. (RR-14) ):1-7.

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