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Vascular pathogenesis of gastroschisis
EDITORIAL C O R R E S P O N D E N C E
Editorial correspondence is subject to critical review and to current editorial policy in respect to publication in part or in full. Preference is given to letters related to articles published in TH~ JOURNAL, but letters on topics of current interest may be accepted if space is available. Letters are restricted to 300 words or less, and 3 supporting references.
Retinopathy of prematurity/ idiopathic fibroplasia
REFERENCE
I.
Heath P: Retrolental fibroplasia as a syndrome, Arch Ophthalmol, 44:245, 1950.
To the Editor: The term retrolental fibroplasia (RLF), taken literally, is too restrictive, since most premature infants whose retinal vessels undergo abnormal changes are not left with retinal scarring. In 1950, Heath' proposed that the name "retinopathy of prematurity" be used to encourage recognition of the fact that there is a wide spectrum of pathologic findings in the disorder. His arguments are timely; modern techniques of examination (indirect opthalmoscopy, retinal photography, and fluorescein angiography) have demonstrated that vascular manifestations are much more common, and regression more frequent, than was appreciated in the past. At a recent meeting (Bodega Bay, Calif., July, 1980) it was urged that a renewed effort be made to dislodge the established misnomer, RLF. The following scheme of terminology was proposed: a revival of Heath's label "retinopathy of prematu rity" (ROP) as the designation for the overall condition, and "idiopathic fibroplasia" (IF) as the term for the scarring complication. There are a number of justifications for the proposal: (1) the broad term emphasizes that vascular changes should be included in descriptions and studies of the disease, (2) the old-new term points to prematurity as the most common precondition of the disorder, and (3) the schema should promote awareness that factors operating to determine the infrequent switch from a reversible proliferative retinopathy to an unremitting cicatricial proccss-fibroplasia are simply unknown. (For example, the variable clinical courses in individuals cannot be predicted from the vascular changes seen on serial examinations nor from the oxygen-treatment histories.) The proposal has been sent to appropriate journals throughout the world to indicate that it is time to discard old patterns of thought about this serious affliction. A fresh attack should be mounted on the unsolved problems of ROP/IF.
Beno~it Duc, Secretary Second International R L F Conference 3961 Chermignon Switzerland
662
TheJournalofPEDIATRlCS
Vascular pathogenesis of gastroschisis To the Editor: Hoyme et al 1 have recently proposed vascular disruption with premature ablation of the distal omphalomesenteric artery, as the etiology for gastroschisis. In support o f their findings, we would fike to report the results o f a similar study. We investigated patients with intestinal atresia for associated defects of possible vascular origin. Intestinal atresia was selected because it has been shown experimentally to be secondary to a vascular pathogenesis involving at least one of the superior mesenteric artery arcades, ~ and it would be anticipated that other defects found in association might be caused by vascular disruption as well. Charts were reviewed of 18 patients ascertained wilh the diagnosis of intestinal atresia over the past ten years. Records were obtained from the Department of Medical Records at Children's Orthopedic Hospital and Medical Center, Seattle. Of special interest to the report by Hoyme et al were associated abnormalities which may be due to vascular disruption: Three of the 18 had gastroschisis; another had gallbladder abnormalities and hypoplastic cystic artery and duct; and two had structural abnormalities of the brain, including cerebellar atrophy, which could have been secondary to vascular problems.
Margot 1. Van Allen, M.S., M.D. David W. Smith, M.D/f Dysmorphology Unit Department of Pediatrics Child Development and Mental Retardation Center, and the Center for Inherited Diseases University of Washington School of Medicine Seattle, WA 98195 ]'Deceased.
Volume 98 Number 4
Editorial correspondence
REFERENCES 1.
2.
care units for plotting the postnatal growth of sick low-birthweight infants. Mean and standard deviations of the measurements were calculated, and these data were plotted as smoothed curves and superimposed on a chart of normal fetal and postnatal growth (Figure). The gestational ages of our infants at birth ranged from 28 to 32 weeks. They were appropriately grown (between the tenth and ninetieth percentile, mean-fiftieth percentile), but all had severe neonatal disease. In 27 the primary diagnosis was hyaline membrane disease requiring prolonged assisted ventilation, although many of the infants had multiple problems. Four infants with birth weights of less than 1,000 gm had recurrent apnea and bradycardia; one had erythroblastosis fetalis and one had multicystic kidney disease. Of the 22 male and 19 female infants, 40 were white and one black. All infants received 80 to 120 kcal/kg/day by the end o f the second week. Using this type of growth chart, we have been able to identify infants whose head growth has been too rapid: Similarly, this chart permits an assessment of whether adequate "catch-up growth" is occurring. We suggest that these curves may be useful for the plotting of postnatal growth of sick low-birth-weight infants and for identifying significant deviations from the
Hoyme HE, Higginbottom MC, and Jones KL: The vascular pathogenesis gastroschisis: Intrauterine interruption o f the omphalomesenteric artery, J Ptt)lA'rR 98:32, 1981. Louw J, and Barnard CN: Congenital intestinal atresia; observations on its origin, Lancet 2:1065, 1955.
Growth chart for sick premature infants To the Editor: We have previously documented the growth pattern of 41 sick preterm infants, by serial anthropometric measurements made at regular intervals from birth through the first year of life in 82% o f the initial cohort.' This was a longitudinal study in which the data were obtained from repeated measurements made on the same group of infants. We thought that it would be useful to use these data to construct a growth chart which could be used in intensive
AGE-WEEKS
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4
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kg,
kg
cm.
crn
24
24 28
32
36
40
44
48
52
56
663
60
64
68
72
76
80
R4
88
92
Figure. The diagnonal shading represents longitudinal growth of sick preterm infants, ~ 28 to 32 weeks' gestation (mean _+ 1 SD) superimposed on Babson's curve for postnatal growth in well infants:
Editorial correspondence is subject to critical review and to current editorial policy in respect to publication in part or in full. Preference is given to letters related to articles published in TH~ JOURNAL, but letters on topics of current interest may be accepted if space is available. Letters are restricted to 300 words or less, and 3 supporting references.
Retinopathy of prematurity/ idiopathic fibroplasia
REFERENCE
I.
Heath P: Retrolental fibroplasia as a syndrome, Arch Ophthalmol, 44:245, 1950.
To the Editor: The term retrolental fibroplasia (RLF), taken literally, is too restrictive, since most premature infants whose retinal vessels undergo abnormal changes are not left with retinal scarring. In 1950, Heath' proposed that the name "retinopathy of prematurity" be used to encourage recognition of the fact that there is a wide spectrum of pathologic findings in the disorder. His arguments are timely; modern techniques of examination (indirect opthalmoscopy, retinal photography, and fluorescein angiography) have demonstrated that vascular manifestations are much more common, and regression more frequent, than was appreciated in the past. At a recent meeting (Bodega Bay, Calif., July, 1980) it was urged that a renewed effort be made to dislodge the established misnomer, RLF. The following scheme of terminology was proposed: a revival of Heath's label "retinopathy of prematu rity" (ROP) as the designation for the overall condition, and "idiopathic fibroplasia" (IF) as the term for the scarring complication. There are a number of justifications for the proposal: (1) the broad term emphasizes that vascular changes should be included in descriptions and studies of the disease, (2) the old-new term points to prematurity as the most common precondition of the disorder, and (3) the schema should promote awareness that factors operating to determine the infrequent switch from a reversible proliferative retinopathy to an unremitting cicatricial proccss-fibroplasia are simply unknown. (For example, the variable clinical courses in individuals cannot be predicted from the vascular changes seen on serial examinations nor from the oxygen-treatment histories.) The proposal has been sent to appropriate journals throughout the world to indicate that it is time to discard old patterns of thought about this serious affliction. A fresh attack should be mounted on the unsolved problems of ROP/IF.
Beno~it Duc, Secretary Second International R L F Conference 3961 Chermignon Switzerland
662
TheJournalofPEDIATRlCS
Vascular pathogenesis of gastroschisis To the Editor: Hoyme et al 1 have recently proposed vascular disruption with premature ablation of the distal omphalomesenteric artery, as the etiology for gastroschisis. In support o f their findings, we would fike to report the results o f a similar study. We investigated patients with intestinal atresia for associated defects of possible vascular origin. Intestinal atresia was selected because it has been shown experimentally to be secondary to a vascular pathogenesis involving at least one of the superior mesenteric artery arcades, ~ and it would be anticipated that other defects found in association might be caused by vascular disruption as well. Charts were reviewed of 18 patients ascertained wilh the diagnosis of intestinal atresia over the past ten years. Records were obtained from the Department of Medical Records at Children's Orthopedic Hospital and Medical Center, Seattle. Of special interest to the report by Hoyme et al were associated abnormalities which may be due to vascular disruption: Three of the 18 had gastroschisis; another had gallbladder abnormalities and hypoplastic cystic artery and duct; and two had structural abnormalities of the brain, including cerebellar atrophy, which could have been secondary to vascular problems.
Margot 1. Van Allen, M.S., M.D. David W. Smith, M.D/f Dysmorphology Unit Department of Pediatrics Child Development and Mental Retardation Center, and the Center for Inherited Diseases University of Washington School of Medicine Seattle, WA 98195 ]'Deceased.
Volume 98 Number 4
Editorial correspondence
REFERENCES 1.
2.
care units for plotting the postnatal growth of sick low-birthweight infants. Mean and standard deviations of the measurements were calculated, and these data were plotted as smoothed curves and superimposed on a chart of normal fetal and postnatal growth (Figure). The gestational ages of our infants at birth ranged from 28 to 32 weeks. They were appropriately grown (between the tenth and ninetieth percentile, mean-fiftieth percentile), but all had severe neonatal disease. In 27 the primary diagnosis was hyaline membrane disease requiring prolonged assisted ventilation, although many of the infants had multiple problems. Four infants with birth weights of less than 1,000 gm had recurrent apnea and bradycardia; one had erythroblastosis fetalis and one had multicystic kidney disease. Of the 22 male and 19 female infants, 40 were white and one black. All infants received 80 to 120 kcal/kg/day by the end o f the second week. Using this type of growth chart, we have been able to identify infants whose head growth has been too rapid: Similarly, this chart permits an assessment of whether adequate "catch-up growth" is occurring. We suggest that these curves may be useful for the plotting of postnatal growth of sick low-birth-weight infants and for identifying significant deviations from the
Hoyme HE, Higginbottom MC, and Jones KL: The vascular pathogenesis gastroschisis: Intrauterine interruption o f the omphalomesenteric artery, J Ptt)lA'rR 98:32, 1981. Louw J, and Barnard CN: Congenital intestinal atresia; observations on its origin, Lancet 2:1065, 1955.
Growth chart for sick premature infants To the Editor: We have previously documented the growth pattern of 41 sick preterm infants, by serial anthropometric measurements made at regular intervals from birth through the first year of life in 82% o f the initial cohort.' This was a longitudinal study in which the data were obtained from repeated measurements made on the same group of infants. We thought that it would be useful to use these data to construct a growth chart which could be used in intensive
AGE-WEEKS
0
4
8
12
16
20
24
28
32
36
40
44
48
52
10.0
10.0 9.0
9.0
8.0
8.0
7.0
7.0
6.0
76
5.0
72
4.0
68
3.0
64
2.0
60
1.0
56
52
52
48
48
44
44
40
40
36
36
32
32
28
28
kg,
kg
cm.
crn
24
24 28
32
36
40
44
48
52
56
663
60
64
68
72
76
80
R4
88
92
Figure. The diagnonal shading represents longitudinal growth of sick preterm infants, ~ 28 to 32 weeks' gestation (mean _+ 1 SD) superimposed on Babson's curve for postnatal growth in well infants: