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Gastroschisis and extreme prematurity: a report of two survivors
Journal of Pediatric Surgery (2011) 46, 1274–1276
www.elsevier.com/locate/jpedsurg
Gastroschisis and extreme prematurity: a report of two survivors Sidney Johnson, Sarah Kimball ⁎ Hawai'i Pacific Health and Kapiolani Medical Center for Women and Children, Mililani, HI 96789, USA Received 15 November 2010; revised 29 January 2011; accepted 17 February 2011
Key words: Gastroschisis; Extreme; Prematurity; Premature; Preterm
Abstract Extreme prematurity is not commonly associated with gastroschisis, and there are no reports of babies surviving with both gastroschisis and extreme prematurity. We report 2 such cases. © 2011 Elsevier Inc. All rights reserved.
Over the past decade, there has been a notable increase in the incidence of gastroschisis (GS). Although, it is well known that babies with GS are often born prematurely, the gestational age of preterm GS babies is generally between 34 to 36 weeks [1]. Extreme prematurity is not commonly associated with GS, and there are no reports of babies surviving with both GS and extreme prematurity. We report 2 such cases. Given the increasing incidence of both extreme prematurity and GS, the outcomes of these cases are important because the association may be encountered more commonly in the future.
1. Case histories 1.1. Case 1 An infant boy with prenatally diagnosed GS was born to an 18-year-old mother. The infant was delivered by an emergency cesarean section at 24-weeks gestational age ⁎ Corresponding author. Tel.: +1 808 782 6232. E-mail address: [email protected] (S. Kimball). 0022-3468/$ – see front matter © 2011 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2011.02.057
because of premature rupture of membranes and a prolapsed umbilical cord. Birth weight was 760 g and his Apgar scores were 5 and 7 at 1 and 5 minutes, respectively. The GS defect was typical with herniated small bowel, colon, and left testicle. After intubation in the delivery room, the herniated GS contents were placed in a 3-cm silastic silo bag. During the next 2 days, the GS was reduced in the neonatal intensive care unit (NICU). Final closure and repair of the defect were completed without complications on the third day of life. The fascia was closed primarily and without undue tension; there were no signs of compartment syndrome postoperatively. The baby's early NICU course was thereafter remarkable for issues that are common in extreme prematurity. He was extubated on the sixth day of life and placed on continuous positive airway pressure (CPAP) and caffeine for respiratory support. Over the ensuing 2 months, he developed total parenteral nutrition (TPN)–associated hyperbilirubinemia, hyponatremia, and hypertriglyceridemia in addition to physiologic anemia, mild thrombocytopenia, and transient hypothyroidism. Because of the persistently bilious orogastric tube output, gastric decompression was needed for nearly a month. Thereafter, trophic feeds were slowly advanced using continuous nasal-jejunal tube feeds. Early feeding
Gastroschisis and extreme prematurity: two survivors difficulty was typical for GS with episodic emesis that resolved over time. An upper gastrointestinal at 40 days of life showed normal gastric emptying with slow transit through the gastrointestinal tract. Shortly thereafter, he was transferred from the NICU. He remained hospitalized to manage his emesis, reflux, and transition to full oral feeds. Hyperalimentation was discontinued at 95 days of life. He underwent right inguinal herniorrhaphy at 102 days and was discharged 5 days later with a weight of 3.717 kg. The left undescended testicle was addressed at a later date.
1.2. Case 2 An infant girl was born to an 18-year old gravida 1 para 0 at 24-weeks gestation with a weight of 747 g. She was delivered by an emergency cesarean section secondary to premature rupture of membranes (N7 days premature rupture of membranes), nonreassuring fetal heart tracings, and possible placental abruption. The GS defect was diagnosed at birth, and the baby was immediately intubated. After intubation, the GS contents were wrapped in sterile, moist gauze, and the baby was taken to the newborn intensive care unit where a spring-loaded silastic silo bag was placed. At the time of the initial silo placement a microperforation was noted in one of the loops of bowel. At 10 hours of life, the silo bag was removed because of worsening metabolic acidosis, hypotension, and concerns of abdominal compartment syndrome possibly secondary to premature compression of the silo contents. As it turned out, there was no compartment syndrome; rather, the baby's status was complicated by Escherichia coli sepsis owing to chorioamnionitis from the premature rupture of membranes. A Tegaderm occlusive dressing was placed over the abdomen, and the baby gradually improved with fluid resuscitation and appropriate antibiotics. The microperforation was managed nonsurgically because of its proximity to the GS defect. The enteral contents were allowed to drain from the defect with the assumption that the bowel would form a chronic fistula/stoma at the GS defect. As it turned out, the fistula closed completely within a few days. Because of her critical ventilatory status, the GS defect was never surgically closed, eventually closing completely with simple wound care. She required an OG tube for decompression for the first 44 days of life. This patient had a complicated hospital course with many difficulties typical of extreme prematurity including bronchopulmonary dysplasia, bacterial pneumonia, urinary sepsis, patent ductus arteriosis requiring ligation, prethreshold retinopathy of prematurity and bilateral grade 2 intraventricular hemorrhage. She also had GS-related problems including TPN-associated cholestasis and reflux. These resolved without surgical intervention. The baby was eventually discharged at 148 days of age at a weight of 3.546 kg. She was breastfeeding normally with supplemental nasogastric gavage feeds as needed. Her
1275 oxygen requirements related to her bronchopulmonary dysplasia and pneumonia were slowly weaned over the next few months. Evaluation at 8 months of age found a thriving infant with slight developmental delay, no signs of cerebral palsy, and no umbilical hernia at the GS site.
2. Discussion The general association between, and outcomes of, babies with GS and mild to moderate prematurity are well known [2,3]. In babies with GS, delivery before 37 weeks of gestation, elective or nonelective, is associated with higher rates of sepsis, longer lengths of stay, greater time to full enteral feeds, and overall higher health care costs compared with term neonates with GS [4,5]. Preterm infants with GS between 34 and 36 weeks of gestation also have a higher risk of recorded morbidities including sepsis, intestinal perforation, intestinal stricture, necrotizing enterocolitis, short gut syndrome, and cholestasis [4,6]. These infants spend a mean of 38 days on TPN and have a mean length of stay of 54 days [4]. Infants with GS born less than 37 weeks of gestation weighing less than 2500 g have an increased risk of neonatal death compared with term GS infants with larger birth weights [7]. Because of these data, it was unclear to us if the cumulative morbidities of GS and prematurity would be overwhelming in a baby with extreme prematurity. To our knowledge, there are no published reports of GS in extremely preterm infants (24-27 weeks of gestational age). In retrospect, we believe that most of the long-term morbidity we encountered in these patients is strongly associated with the extreme prematurity rather than the GS. The GS added some initial complicating factors that were managed with standard surgical measures. The slow progression of full enteral feeds was probably owing to a combination of both the inherent dysmotility associated with gastroschisis and the immaturity of the gut in extreme prematurity. It is important to view these cases within the greater context of extreme prematurity. The mortality of extremely preterm infants varies between 41.5% and 80.5%, and the risks of extreme prematurity are well described [8-11]. The cause of death for extremely low-birth-weight infants (501-1000 g) who die at less than 12 hours of age involves general immaturity of all organ systems, whereas infant deaths between 12 hours to 120 days and after 120 days are owing to respiratory distress syndrome and bronchopulmonary dysplasia, respectively [12]. In survivors, the major neonatal complications that occur both in infants 1000 g or lesser and infants born before 27 weeks of gestation include intraventricular hemorrhage, periventricular leucomalasia, retinopathy of prematurity, severe brain injury, growth failure, patent ductus arteriosus, necrotizing enterocolitis, and oxygen dependence [13]. The risk of severe illness increases as gestational age drops below 26\weeks [14]. At 24 weeks of gestation, the
1276 risk of severe complications including neurologic damage, oxygen requirements, or discharge with ileostomy is 51%; this decreases to 33% at 25 weeks. The hospital stay in severe preterm infants weighing 751 to 1000 g ranges between 66 and 108 days [9]. It has been suggested that a large degree of survival variation in extreme prematurity is owing to the variable practices that occur in maternity and neonatal units [8]. Most deaths among less than 1000 g birth weight infants occur in the first 3 days of life [9]. Infants who do not receive aggressive obstetric and neonatal intervention have an increased risk for early neonatal death [15]. The Vermont Oxford Network found that most extremely low-birth-weight infants who received delivery room resuscitation survive without major short-term morbidity. Nevertheless, both pediatricians and obstetricians underestimate the rates of survival and freedom from handicap in preterm infants from 24 to 35 weeks of gestation and are less likely to intervene with aggressive management [10,15]. We found that a similar bias existed toward both of our patients. In the first case, it was assumed the outcome would be universally fatal; and resuscitation efforts were questioned until it was suggested that little harm could come from trying. The bias against resuscitation in the second baby was softened somewhat by the experience with the first patient. In our limited experience, we found that combination of GS and severe prematurity did not seem to have long-term morbidity above what might be expected with severe prematurity alone.
3. Conclusion To our knowledge, this is the first report of survival in 2 patients with both GS and extreme prematurity. Because of the increasing frequency of GS and its association with prematurity, it is possible that this association with extreme prematurity will become more common in the future. In our experience, the GS defect can add some initial complicating factors that can be managed with standard surgical measures;
S. Johnson, S. Kimball the long-term morbidity of babies with both GS and extreme prematurity may be similar to that of gestational age– matched preterm infants.
References [1] Krause H, et al. Congenital abdominal wall defects—an analysis of prevalence and operative management by means of gastroschisis and omphalocele. Zentralbl Chir 2009;134(6):524-31. [2] Skarsgard E, et al. Canadian pediatric surgical network: a populationbased pediatric surgery network and database for analyzing surgical birth defects. The first 100 cases of gastroschisis. J Pediatr Surg 2008;43(1):30-4; discussion 34. [3] Boutros J, et al. Is timing everything? The influence of gestational age, birth weight, route, and intent of delivery on outcome in gastroschisis. J Pediatr Surg 2009;44(5):912-7. [4] Maramreddy H, et al. Delivery of gastroschisis patients before 37 weeks of gestation is associated with increased morbidities. J Pediatr Surg 2009;44:1360-6. [5] Molik KA, et al. Gastroschisis: a plea for risk categorization. J Pediatr Surg 2001;36(1):51-5. [6] Snyder CL. Outcome analysis for gastroschisis. J Pediatr Surg 1999;34 (8):1253-6. [7] Vilela PC, et al. Risk factors for adverse outcome of newborns with gastroschisis in a Brazilian hospital. J Pediatr Surg 2001;36:559-64. [8] Matta N, young D, Boulton R, et al. Survival of extremely preterm infants. Arch Dis Child Fetal Neonatal Ed 2010;95:F151-2. [9] Lemons JA, Bauer CR, Oh W, et al. Very-low-birth-weight (VLBW) outcomes of the NICHD Neonatal Research Network, January 1995 through December 1996. Pediatrics 2001;107:e1. [10] Haywood JL, Goldenberg RL, Bronstein J, et al. Comparison of perceived and actual rates of survival and freedom from handicap in premature infants. Am J Obstet Gynecol 1994;171:432-9. [11] Lausman A, et al. Gastroschisis: what is the average gestational age of spontaneous delivery? J Pediatr Surg 2007;42(11):1816-21. [12] Shankaran S, et al. Risk factors for early death among extremely lowbirth-weight infants. Am J Obstet Gynecol 2002;186(4):796-802. [13] EXPRESS Group. Incidence of and risk factors for neonatal mobidity after active perinatal care: extremely preterm infants study in Sweden (EXPRESS). Acta Peadiatr 2010;99(7):978-92. Epub 2010 Apr 26. [14] Markestad T, Kaaresen P, Ronnestad A, et al. Early death, mobidity, and need of treatment among extremely premature infants. Pediatrics 2005;115(5):1289-98. [15] Haywood JL, Morse SB, Goldenberg RL, et al. Estimation of neonatal outcome and perinatal therapy use. Pediatrics 2000;105(5):1046-50.
www.elsevier.com/locate/jpedsurg
Gastroschisis and extreme prematurity: a report of two survivors Sidney Johnson, Sarah Kimball ⁎ Hawai'i Pacific Health and Kapiolani Medical Center for Women and Children, Mililani, HI 96789, USA Received 15 November 2010; revised 29 January 2011; accepted 17 February 2011
Key words: Gastroschisis; Extreme; Prematurity; Premature; Preterm
Abstract Extreme prematurity is not commonly associated with gastroschisis, and there are no reports of babies surviving with both gastroschisis and extreme prematurity. We report 2 such cases. © 2011 Elsevier Inc. All rights reserved.
Over the past decade, there has been a notable increase in the incidence of gastroschisis (GS). Although, it is well known that babies with GS are often born prematurely, the gestational age of preterm GS babies is generally between 34 to 36 weeks [1]. Extreme prematurity is not commonly associated with GS, and there are no reports of babies surviving with both GS and extreme prematurity. We report 2 such cases. Given the increasing incidence of both extreme prematurity and GS, the outcomes of these cases are important because the association may be encountered more commonly in the future.
1. Case histories 1.1. Case 1 An infant boy with prenatally diagnosed GS was born to an 18-year-old mother. The infant was delivered by an emergency cesarean section at 24-weeks gestational age ⁎ Corresponding author. Tel.: +1 808 782 6232. E-mail address: [email protected] (S. Kimball). 0022-3468/$ – see front matter © 2011 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2011.02.057
because of premature rupture of membranes and a prolapsed umbilical cord. Birth weight was 760 g and his Apgar scores were 5 and 7 at 1 and 5 minutes, respectively. The GS defect was typical with herniated small bowel, colon, and left testicle. After intubation in the delivery room, the herniated GS contents were placed in a 3-cm silastic silo bag. During the next 2 days, the GS was reduced in the neonatal intensive care unit (NICU). Final closure and repair of the defect were completed without complications on the third day of life. The fascia was closed primarily and without undue tension; there were no signs of compartment syndrome postoperatively. The baby's early NICU course was thereafter remarkable for issues that are common in extreme prematurity. He was extubated on the sixth day of life and placed on continuous positive airway pressure (CPAP) and caffeine for respiratory support. Over the ensuing 2 months, he developed total parenteral nutrition (TPN)–associated hyperbilirubinemia, hyponatremia, and hypertriglyceridemia in addition to physiologic anemia, mild thrombocytopenia, and transient hypothyroidism. Because of the persistently bilious orogastric tube output, gastric decompression was needed for nearly a month. Thereafter, trophic feeds were slowly advanced using continuous nasal-jejunal tube feeds. Early feeding
Gastroschisis and extreme prematurity: two survivors difficulty was typical for GS with episodic emesis that resolved over time. An upper gastrointestinal at 40 days of life showed normal gastric emptying with slow transit through the gastrointestinal tract. Shortly thereafter, he was transferred from the NICU. He remained hospitalized to manage his emesis, reflux, and transition to full oral feeds. Hyperalimentation was discontinued at 95 days of life. He underwent right inguinal herniorrhaphy at 102 days and was discharged 5 days later with a weight of 3.717 kg. The left undescended testicle was addressed at a later date.
1.2. Case 2 An infant girl was born to an 18-year old gravida 1 para 0 at 24-weeks gestation with a weight of 747 g. She was delivered by an emergency cesarean section secondary to premature rupture of membranes (N7 days premature rupture of membranes), nonreassuring fetal heart tracings, and possible placental abruption. The GS defect was diagnosed at birth, and the baby was immediately intubated. After intubation, the GS contents were wrapped in sterile, moist gauze, and the baby was taken to the newborn intensive care unit where a spring-loaded silastic silo bag was placed. At the time of the initial silo placement a microperforation was noted in one of the loops of bowel. At 10 hours of life, the silo bag was removed because of worsening metabolic acidosis, hypotension, and concerns of abdominal compartment syndrome possibly secondary to premature compression of the silo contents. As it turned out, there was no compartment syndrome; rather, the baby's status was complicated by Escherichia coli sepsis owing to chorioamnionitis from the premature rupture of membranes. A Tegaderm occlusive dressing was placed over the abdomen, and the baby gradually improved with fluid resuscitation and appropriate antibiotics. The microperforation was managed nonsurgically because of its proximity to the GS defect. The enteral contents were allowed to drain from the defect with the assumption that the bowel would form a chronic fistula/stoma at the GS defect. As it turned out, the fistula closed completely within a few days. Because of her critical ventilatory status, the GS defect was never surgically closed, eventually closing completely with simple wound care. She required an OG tube for decompression for the first 44 days of life. This patient had a complicated hospital course with many difficulties typical of extreme prematurity including bronchopulmonary dysplasia, bacterial pneumonia, urinary sepsis, patent ductus arteriosis requiring ligation, prethreshold retinopathy of prematurity and bilateral grade 2 intraventricular hemorrhage. She also had GS-related problems including TPN-associated cholestasis and reflux. These resolved without surgical intervention. The baby was eventually discharged at 148 days of age at a weight of 3.546 kg. She was breastfeeding normally with supplemental nasogastric gavage feeds as needed. Her
1275 oxygen requirements related to her bronchopulmonary dysplasia and pneumonia were slowly weaned over the next few months. Evaluation at 8 months of age found a thriving infant with slight developmental delay, no signs of cerebral palsy, and no umbilical hernia at the GS site.
2. Discussion The general association between, and outcomes of, babies with GS and mild to moderate prematurity are well known [2,3]. In babies with GS, delivery before 37 weeks of gestation, elective or nonelective, is associated with higher rates of sepsis, longer lengths of stay, greater time to full enteral feeds, and overall higher health care costs compared with term neonates with GS [4,5]. Preterm infants with GS between 34 and 36 weeks of gestation also have a higher risk of recorded morbidities including sepsis, intestinal perforation, intestinal stricture, necrotizing enterocolitis, short gut syndrome, and cholestasis [4,6]. These infants spend a mean of 38 days on TPN and have a mean length of stay of 54 days [4]. Infants with GS born less than 37 weeks of gestation weighing less than 2500 g have an increased risk of neonatal death compared with term GS infants with larger birth weights [7]. Because of these data, it was unclear to us if the cumulative morbidities of GS and prematurity would be overwhelming in a baby with extreme prematurity. To our knowledge, there are no published reports of GS in extremely preterm infants (24-27 weeks of gestational age). In retrospect, we believe that most of the long-term morbidity we encountered in these patients is strongly associated with the extreme prematurity rather than the GS. The GS added some initial complicating factors that were managed with standard surgical measures. The slow progression of full enteral feeds was probably owing to a combination of both the inherent dysmotility associated with gastroschisis and the immaturity of the gut in extreme prematurity. It is important to view these cases within the greater context of extreme prematurity. The mortality of extremely preterm infants varies between 41.5% and 80.5%, and the risks of extreme prematurity are well described [8-11]. The cause of death for extremely low-birth-weight infants (501-1000 g) who die at less than 12 hours of age involves general immaturity of all organ systems, whereas infant deaths between 12 hours to 120 days and after 120 days are owing to respiratory distress syndrome and bronchopulmonary dysplasia, respectively [12]. In survivors, the major neonatal complications that occur both in infants 1000 g or lesser and infants born before 27 weeks of gestation include intraventricular hemorrhage, periventricular leucomalasia, retinopathy of prematurity, severe brain injury, growth failure, patent ductus arteriosus, necrotizing enterocolitis, and oxygen dependence [13]. The risk of severe illness increases as gestational age drops below 26\weeks [14]. At 24 weeks of gestation, the
1276 risk of severe complications including neurologic damage, oxygen requirements, or discharge with ileostomy is 51%; this decreases to 33% at 25 weeks. The hospital stay in severe preterm infants weighing 751 to 1000 g ranges between 66 and 108 days [9]. It has been suggested that a large degree of survival variation in extreme prematurity is owing to the variable practices that occur in maternity and neonatal units [8]. Most deaths among less than 1000 g birth weight infants occur in the first 3 days of life [9]. Infants who do not receive aggressive obstetric and neonatal intervention have an increased risk for early neonatal death [15]. The Vermont Oxford Network found that most extremely low-birth-weight infants who received delivery room resuscitation survive without major short-term morbidity. Nevertheless, both pediatricians and obstetricians underestimate the rates of survival and freedom from handicap in preterm infants from 24 to 35 weeks of gestation and are less likely to intervene with aggressive management [10,15]. We found that a similar bias existed toward both of our patients. In the first case, it was assumed the outcome would be universally fatal; and resuscitation efforts were questioned until it was suggested that little harm could come from trying. The bias against resuscitation in the second baby was softened somewhat by the experience with the first patient. In our limited experience, we found that combination of GS and severe prematurity did not seem to have long-term morbidity above what might be expected with severe prematurity alone.
3. Conclusion To our knowledge, this is the first report of survival in 2 patients with both GS and extreme prematurity. Because of the increasing frequency of GS and its association with prematurity, it is possible that this association with extreme prematurity will become more common in the future. In our experience, the GS defect can add some initial complicating factors that can be managed with standard surgical measures;
S. Johnson, S. Kimball the long-term morbidity of babies with both GS and extreme prematurity may be similar to that of gestational age– matched preterm infants.
References [1] Krause H, et al. Congenital abdominal wall defects—an analysis of prevalence and operative management by means of gastroschisis and omphalocele. Zentralbl Chir 2009;134(6):524-31. [2] Skarsgard E, et al. Canadian pediatric surgical network: a populationbased pediatric surgery network and database for analyzing surgical birth defects. The first 100 cases of gastroschisis. J Pediatr Surg 2008;43(1):30-4; discussion 34. [3] Boutros J, et al. Is timing everything? The influence of gestational age, birth weight, route, and intent of delivery on outcome in gastroschisis. J Pediatr Surg 2009;44(5):912-7. [4] Maramreddy H, et al. Delivery of gastroschisis patients before 37 weeks of gestation is associated with increased morbidities. J Pediatr Surg 2009;44:1360-6. [5] Molik KA, et al. Gastroschisis: a plea for risk categorization. J Pediatr Surg 2001;36(1):51-5. [6] Snyder CL. Outcome analysis for gastroschisis. J Pediatr Surg 1999;34 (8):1253-6. [7] Vilela PC, et al. Risk factors for adverse outcome of newborns with gastroschisis in a Brazilian hospital. J Pediatr Surg 2001;36:559-64. [8] Matta N, young D, Boulton R, et al. Survival of extremely preterm infants. Arch Dis Child Fetal Neonatal Ed 2010;95:F151-2. [9] Lemons JA, Bauer CR, Oh W, et al. Very-low-birth-weight (VLBW) outcomes of the NICHD Neonatal Research Network, January 1995 through December 1996. Pediatrics 2001;107:e1. [10] Haywood JL, Goldenberg RL, Bronstein J, et al. Comparison of perceived and actual rates of survival and freedom from handicap in premature infants. Am J Obstet Gynecol 1994;171:432-9. [11] Lausman A, et al. Gastroschisis: what is the average gestational age of spontaneous delivery? J Pediatr Surg 2007;42(11):1816-21. [12] Shankaran S, et al. Risk factors for early death among extremely lowbirth-weight infants. Am J Obstet Gynecol 2002;186(4):796-802. [13] EXPRESS Group. Incidence of and risk factors for neonatal mobidity after active perinatal care: extremely preterm infants study in Sweden (EXPRESS). Acta Peadiatr 2010;99(7):978-92. Epub 2010 Apr 26. [14] Markestad T, Kaaresen P, Ronnestad A, et al. Early death, mobidity, and need of treatment among extremely premature infants. Pediatrics 2005;115(5):1289-98. [15] Haywood JL, Morse SB, Goldenberg RL, et al. Estimation of neonatal outcome and perinatal therapy use. Pediatrics 2000;105(5):1046-50.