children Review Abdominal Wall Defects—Current Treatments Isabella N. Bielicki 1, Stig Somme 2, Giovanni Frongia 3, Stefan G. Holland-Cunz 1 and Raphael N. Vuille-dit-Bille 1,* 1 Department of Pediatric Surgery, University Children’s Hospital of Basel (UKBB), 4056 Basel, Switzerland; [email protected] (I.N.B.); [email protected] (S.G.H.-C.) 2 Department of Pediatric Surgery, University Children’s Hospital of Colorado, Aurora, CO 80045, USA; [email protected] 3 Section of Pediatric Surgery, Department of General, Visceral and Transplantation Surgery, 69120 Heidelberg, Germany; [email protected] * Correspondence: [email protected]; Tel.: +41-61-704-27-98 Abstract: Gastroschisis and omphalocele reflect the two most common abdominal wall defects in newborns. First postnatal care consists of defect coverage, avoidance of fluid and heat loss, fluid administration and gastric decompression. Definitive treatment is achieved by defect reduction and abdominal wall closure. Different techniques and timings are used depending on type and size of defect, the abdominal domain and comorbidities of the child. The present review aims to provide an overview of current treatments. Keywords: abdominal wall defect; gastroschisis; omphalocele; treatment 1. Gastroschisis Citation: Bielicki, I.N.; Somme, S.; 1.1. Introduction Frongia, G.; Holland-Cunz, S.G.; Gastroschisis is one of the most common congenital abdominal wall defects in new- Vuille-dit-Bille, R.N. Abdominal Wall borns. Children born with gastroschisis have a full-thickness paraumbilical abdominal Defects—Current Treatments. wall defect, which is associated with evisceration of bowel and sometimes other organs Children 2021, 8, 170. https:// (Figure1a). The defect typically measures 2–3 centimeters and is thought to result from doi.org/10.3390/children8020170 involution of the right umbilical vein. Hence, the defect is mostly located to the right of the umbilical cord [1]. The prevalence of gastroschisis has decreased to baseline from 2009 Academic Editor: Piotr Kalici´nski through 2018 after increasing from 2–3 per 10,000 in 1995 to 4–5 per 10,000 in 2005 [2–7]. Risk factors associated with gastroschisis include maternal smoking, illicit drug use, alcohol Received: 23 January 2021 consumption, young maternal age, environmental factors, socio-economic status, opioid Accepted: 15 February 2021 Published: 23 February 2021 prescription rates and others [8–10]. It seems likely that changes in gastroschisis prevalence result from changes in risk factor prevalence. Gastroschisis is typically accompanied by intestinal non-rotation and may be associated with intestinal atresia and/or perforations. Publisher’s Note: MDPI stays neutral with regard to jurisdictional claims in In contrast to omphalocele, gastroschisis is rarely associated with other congenital or chro- published maps and institutional affil- mosomal anomalies [11]. As the midgut is freely floating in the amniotic fluid, the intestine iations. is typically thickened, edematous and foreshortened [12]. 1.2. Complex Gastroschisis Complex (as compared to simple) gastroschisis occurs in about 11–28% of cases and is defined by the presence of intestinal complications including atresia, perforation or Copyright: © 2021 by the authors. Licensee MDPI, Basel, Switzerland. necrosis. Intestinal atresia is the most common anomaly associated with gastroschisis This article is an open access article occurring in about 10–15% of patients [11–14]. distributed under the terms and Complex gastroschisis is associated with increased morbidity and mortality lower- conditions of the Creative Commons ing survival rate from >90% to 70–80% in developed countries [15]. Length of stay is Attribution (CC BY) license (https:// dramatically prolonged in complex gastroschisis [16]. creativecommons.org/licenses/by/ 4.0/). Children 2021, 8, 170. https://doi.org/10.3390/children8020170 https://www.mdpi.com/journal/children Children 2021,, 8,, 170x FOR PEER REVIEW 2 2of of 18 17 FigureFigure 1. Gastroschisis:Gastroschisis: ( a) Herniated intestine andand greatergreater omentum;omentum; ( b(b)) Silo Silo treatment; treatment; ( c()c) Abdomi- Ab- dominalnal wall afterwall after sutured sutured closure; closure; (d) Sutureless (d) Sutureless closure. closure. 1.2. ComplexThe abdominal Gastroschisis wall defect may shrink during the last trimester (referred to as closing gastroschisis), this can lead to compression of the herniated viscera resulting in intestinal obstructionComplex and/or (as compared mesenteric to simple) ischemia. gastroschisis In rare cases, occurs a complete in about closure 11%– of28% the of abdominal cases and iswall defined at birth by the can presence be observed. of intestinal This is complications referred to as including closed gastroschisis. atresia, perforation As a result,or ne- crosis.secondary Intestinal atresia, atresia the presence is the most of only common rudimentary anomaly herniated associated viscera with orgastroschisis the complete occur- loss ringof the in herniatedabout 10% bowel–15% (referredof patients to [11 as– vanishing14]. gastroschisis) may occur [17]. It is hence crucialComplex to detect gastroschisis signs of abdominal is associat walled with closure increased in utero. morbidity and mortality lowering survival rate from >90% to 70%–80% in developed countries [15]. Length of stay is dra- matically1.3. Diagnostic prolonged Approach in complex and Prenatal gastroschisis Management [16]. TheGastroschisis abdominal is wall typically defect diagnosed may shrink by ultrasoundduring the duringlast trimester the second (refer trimesterred to as anomaly closing gastroschisis),scan, at around this 20 weekscan lead gestational to compression age (WGA). of the In herniated contrast toviscera omphalocele, resulting the in herniatedintestinal obstructionbowel floats and/or freely mesenteric in the amniotic ischemia. cavity In rare and cases, is not a coveredcomplete by closure a membranous of the abdominal hernia wallsac [ 18at ].birth First can trimester be observed. diagnosis This of is gastroschisis referred to as is closed unreliable, gastroschisis. as early ultrasoundAs a result, diag- sec- ondarynosis can atresia, be confounded the presence by of the only physiologic rudimentary midgut herniated herniation viscera leading or the tocomplete false positive loss of theresults herniated [19]. As bowel physiologic (referred midgut to as herniation vanishing is gastroschisis) still present until may 12 occur WGA [17]. in up It tois 20%hence of crucialfetuses, to prenatal detect signs diagnosis of abdominal of gastroschisis wall closure earlier in inutero. pregnancy needs hence to be inter- preted cautiously. The prenatal diagnosis of gastroschisis should be differentiated from 1.3.other Diagnostic ventral abdominal Approach and wall P defects,renatal M asanagement management and treatment differ between entities. EspeciallyGastroschisis in the unusualis typically cases diagnosed of in utero by rupture ultrasound of the during omphalocele the second sac, distinction trimester anomalybetween gastroschisesscan, at around and 20 omphalocele weeks gestational may be age difficult. (WGA). Furthermore, In contrast to liver omphalocele, herniation—a the herniatedtypical sign bowel of omphalocele—has floats freely in the also amniotic been described cavity and in is cases not covered of gastroschisis by a membranous with large herniadefects sac [1]. [18]. In contrast, First trimester elevation diagnosis of Alpha of gastroschisis Fetoprotein (AFP)is unreliable, in maternal as early serum ultrasound is more diagnosislikely to be can observed be confounded in pregnancies by the physiologic with gastroschisis midgut asherniation compared leading to omphalocele to false posi- [1]. tiveAfter results in utero [19]. diagnosis As physiologic of an abdominal midgut herniation wall defect is still (gastroschisis present until or omphalocele),12 WGA in up the to 20%care of of fetuses, the pregnant prenatal mother diagnosis should of gastroschisis be transferred earlier to a in tertiary pregnancy care centerneeds hence for further to be interpretedcounselling cautiously. and treatment The by prenatal an interdisciplinary diagnosis of team gastroschisis encompassing should obstetricians, be differentiated neona- fromtologists other and ventral pediatric abdominal surgeons wall [18 ].defects, Serial ultrasoundsas management are performed and treatment to evaluate differ between different entities.intestinal Especially outcome in parameters the unusual (including cases of intestinalin utero rupture dilation of and the bowel omphalocele wall thickening), sac, dis- tinctionthe amount between of amniotic gastroschises fluid and and growth omphalocele of the fetus may [ 18be]. difficult. Dilation Furth of theermore, intra- (not liver extra-) her- niationabdominal—a typical bowel, sign as well of omphalocele as the presence—has of also polihydramnios been described are in typicallycases of gastroschisis observed in withcomplex large gastroschisis defects [1]. In (with contrast, intestinal elevation atresia). of Alpha Gastric Fetoprotein dilation ( mayAFP)be in associatedmaternal serum with isincreased more likely mortality to be [18 ].observed An increasing in pregnancies dilation of thewith small gastroschisis bowel could as indicate compared closing to Children 2021, 8, 170 3 of 17 gastroschisis, which necessitates premature delivery in order to avoid loss of the herniated viscera (i.e., vanishing gastroschisis)