Introduction Preterm infants are prone to life threatening gastrointestinal emergencies. These are commonly due to conditions such as necrotizing enterocolitis (NEC), spontaneous intestinal perforation (SIP), intestinal atresia and stenosis. However, other uncommon causes can present as gastrointestinal emergencies, often with atypical presentations potentially delaying the diagnosis and life-saving treatment. In this report, we describe a case of intestinal obstruction due to a congenital mesenteric hernia in a premature infant. Objectives: •To describe a rare cause of a gastrointestinal emergency in a preterm infant. •To review the etiology, clinical features, diagnosis and management of internal intestinal hernia due to a mesenteric defect in a preterm infant. Congenital Mesenteric Hernia Causing Intestinal Obstruction in a Preterm Neonate: A Rare Gastrointestinal Emergency Jenifer Cuestas, M.D 1 , PGY-4, Amy Hair, M.D 1 , Jonathan Davies, M.D 1 , Sohail R Shah, M.D 2 , Muralidhar Premkumar, M.D 1 1 Texas Children’s Hospital, Division of Neonatology, Baylor College of Medicine. 2 Texas Children's Hospital, Division of Pediatric Surgery. Literature Review An internal hernia is a visceral herniation through a normal or abnormal aperture within the peritoneal cavity, which can either be congenital or acquired (1) . Internal hernias involving the small and large bowel are classified according to their location as: paraduodenal, through the foramen of Winslow, intersigmoid, pericecal, transmesenteric and retroanastamotic (Fig. 3) (2) . Transmesenteric hernias are a rare cause of small intestinal obstructions, with an incidence of 0.2 -0.9%, with most of the cases being reported in adulthood. Intestinal obstruction in neonates due to a transmesenteric defect is rare (3) . The etiology of mesenteric defects is unclear; Hypotheses regarding their origin include genetic associations, defects occurring during embryonal development (3) , and ischemic insults in watershed areas of the mesentery most commonly near the ligament of Treitz, and the region near the ileocecal valve, known as Treves’ field (4) . A review in pediatric patients with surgically proven hernias over a 9 year period, showed that congenital internal hernias present in two distinct groups. Neonates are more likely to have congenital transmesenteric hernias (CTMH), while older children suffer from congenital paraduodenal hernias (1) . Neonates present acutely with vomiting and symptoms of intestinal obstruction, whereas older children have chronic intermittent abdominal pain. Moreover, the risk of volvulus and bowel ischemia due to a transmesenteric hernia is higher than with a paraduoneal hernia. These reviews also highlight the difficulty in prospective identification with help of diagnostic imaging. The lack of a hernia sac makes CTMH less likely to be identified with a GI contrast study (1) . However, paraduodenal hernias, common in older children were found in pre-operative imaging. The current reviews suggest that in the absence of imaging modality that is sensitive enough to diagnose CTMH, the definitive diagnosis is made surgically (5) . Clinical outcomes related to CTMH depend on early identification of an abdominal process that requires prompt surgical intervention. Case Presentation Conclusions A mesenteric defect can produce a Congenital Transmesenteric Hernia (CTMH), which may cause a gastrointestinal emergency in a neonate. In the neonatal period, a CTMH presents acutely with symptoms concerning for small bowel obstruction. Diagnostic imaging is often not helpful in detecting CTMH, and the diagnosis is often made during surgical exploration. Hence, high degree of suspicion and reliance on clinical symptoms is crucial in the treatment of gastrointestinal emergencies secondary to internal herniations. References 1. Tang, V., Danemian, A., Navarro, O.M., Miller, S.F., and Gerstle, J.T.,"Internal Hernias in Children: Spectrum of Clinical and Imaging Findings." Pediatric Radiology 41.12 (2011): 1559-1568. 2. Martin, L.C., Merkle, E.M., and Thompson, W.M. "Review of Internal Hernias: Radiographic and Clinical Findings." American Journal of Roentgenology 186.3 (2006): 703-717. 3. Page, M.P., Ricca, R.L., Resnick, A.S.,Puder, M., and Fishman, S.J., "Newborn and Toddler Intestinal Obstruction Owing to Congenital Mesenteric Defects." Journal of Pediatric Surgery 43.4 (2008): 755-758. 4. Galazka, P., Sadej, N., Reszczynska-Fomagala, M.,Dymek, K., Kroczeck, K., and Daniluk-Matras, I.,"Intrahepatic Intestinal Loop Through a Congenital Mesenteric Hernia." Journal of Pediatric Surgery Case Reports 25 (2017): 44-45. 5. Mandhan, P., Alshahwani, N., Al-Balushi, Z., and Arain, A.,"Congenital Mesenteric Hernia in Neonates: Still a Dilemma." African Journal of Paediatric Surgery 12.3 (2015): 203-207. Texas Pediatric Society Electronic Poster Contest A female, born at 23 3/7 weeks, with a birth weight of 585 g had an uneventful course tolerating full enteral feeds, stable on nCPAP support. At the age of 2 weeks, the infant acutely decompensated with metabolic acidosis, apnea, hypotension resulted in acute respiratory failure requiring intubation and vasopressor support. Abdominal exam which was unremarkable initially, showed moderate tenderness and distension twelve hours after initial presentation. Serial X rays showed non-specific bowel dilation. There were no evidence of pneumatosis or intraperitoneal air. The abdominal ultrasound showed small volume complex ascites with moderate bowel wall thickening and diminished peristaltic activity. Due to lack of clinical improvement, patient underwent a bedside laparotomy that showed several loops of ischemic bowel herniating through a distal ileal mesenteric defect (Figs 1 & 2). 22 cm of distal ileum was resected with creation of an ileostomy. Post-op course was complicated by a right sided grade IV intraventricular hemorrhage. Infant remained on partial parenteral nutrition until re- anastomosis performed 8 weeks later. The infant was discharged home on full enteral feeds at the age of 4 months, corrected to 44 weeks and 2 days. Fig. 2- Intraoperative image showing ischemic bowel Fig. 1- Intraoperative image showing the mesenteric defect Fig. 3- Illustration of various types of internal hernias. A= paraduodenal, B= formen of Winslow, C= intersigmoid, D= pericecal, E= transmesenteric, and F= retroanastomotic. Abstract An former 23 3/7 week preterm with a birth weight of 585 grams developed abdominal distension and tenderness with multi organ failure at the post menstrual age of of 25 3/7 weeks. Diagnostic imaging failed to show evidence of either NEC or SIP. Laparotomy revealed ischemic bowel as a result of internal herniation through a mesenteric defect. Ischemic bowel was resected and an ileostomy was created. This case highlights the challenges in the diagnosis of rare causes of gastrointestinal emergencies in a preterm neonate. It also stressed the need for close monitoring, and reliance on clinical examination to to guide intervention is such scenarios.
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Introduction Preterm infants are prone to life threateninggastrointestinal emergencies. These are commonly due toconditions such as necrotizing enterocolitis (NEC),spontaneous intestinal perforation (SIP), intestinal atresiaand stenosis. However, other uncommon causes canpresent as gastrointestinal emergencies, often withatypical presentations potentially delaying the diagnosisand life-saving treatment. In this report, we describe a caseof intestinal obstruction due to a congenital mesenterichernia in a premature infant.
Objectives:•To describe a rare cause of a gastrointestinal emergency ina preterm infant.•To review the etiology, clinical features, diagnosis andmanagement of internal intestinal hernia due to amesenteric defect in a preterm infant.
Congenital Mesenteric Hernia Causing Intestinal Obstruction in a Preterm Neonate: A Rare Gastrointestinal Emergency
Jenifer Cuestas, M.D1, PGY-4, Amy Hair, M.D1, Jonathan Davies, M.D1, Sohail R Shah, M.D2, Muralidhar Premkumar, M.D1
1Texas Children’s Hospital, Division of Neonatology, Baylor College of Medicine. 2Texas Children's Hospital, Division of Pediatric Surgery.
Literature ReviewAn internal hernia is a visceral herniation through anormal or abnormal aperture within the peritonealcavity, which can either be congenital or acquired (1).Internal hernias involving the small and large bowel areclassified according to their location as: paraduodenal,through the foramen of Winslow, intersigmoid, pericecal,transmesenteric and retroanastamotic (Fig. 3) (2).
Transmesenteric hernias are a rare cause of smallintestinal obstructions, with an incidence of 0.2 -0.9%,with most of the cases being reported in adulthood.Intestinal obstruction in neonates due to atransmesenteric defect is rare (3). The etiology ofmesenteric defects is unclear; Hypotheses regarding theirorigin include genetic associations, defects occurringduring embryonal development (3), and ischemic insultsin watershed areas of the mesentery most commonlynear the ligament of Treitz, and the region near theileocecal valve, known as Treves’ field (4).A review in pediatric patients with surgically provenhernias over a 9 year period, showed that congenitalinternal hernias present in two distinct groups. Neonatesare more likely to have congenital transmesenterichernias (CTMH), while older children suffer fromcongenital paraduodenal hernias (1). Neonates presentacutely with vomiting and symptoms of intestinalobstruction, whereas older children have chronicintermittent abdominal pain. Moreover, the risk ofvolvulus and bowel ischemia due to a transmesenterichernia is higher than with a paraduoneal hernia. Thesereviews also highlight the difficulty in prospectiveidentification with help of diagnostic imaging. The lack ofa hernia sac makes CTMH less likely to be identified witha GI contrast study (1) . However, paraduodenal hernias,common in older children were found in pre-operativeimaging. The current reviews suggest that in the absenceof imaging modality that is sensitive enough to diagnoseCTMH, the definitive diagnosis is made surgically(5).Clinical outcomes related to CTMH depend on earlyidentification of an abdominal process that requiresprompt surgical intervention.
Case Presentation
Conclusions
A mesenteric defect can produce a CongenitalTransmesenteric Hernia (CTMH), which may cause agastrointestinal emergency in a neonate. In the neonatalperiod, a CTMH presents acutely with symptomsconcerning for small bowel obstruction. Diagnosticimaging is often not helpful in detecting CTMH, and thediagnosis is often made during surgical exploration.Hence, high degree of suspicion and reliance on clinicalsymptoms is crucial in the treatment of gastrointestinalemergencies secondary to internal herniations.
References
1. Tang, V., Danemian, A., Navarro, O.M., Miller, S.F., and Gerstle,J.T.,"Internal Hernias in Children: Spectrum of Clinical andImaging Findings." Pediatric Radiology 41.12 (2011): 1559-1568.
2. Martin, L.C., Merkle, E.M., and Thompson, W.M. "Review ofInternal Hernias: Radiographic and Clinical Findings." AmericanJournal of Roentgenology 186.3 (2006): 703-717.
3. Page, M.P., Ricca, R.L., Resnick, A.S.,Puder, M., and Fishman, S.J.,"Newborn and Toddler Intestinal Obstruction Owing toCongenital Mesenteric Defects." Journal of Pediatric Surgery43.4 (2008): 755-758.
4. Galazka, P., Sadej, N., Reszczynska-Fomagala, M.,Dymek, K.,Kroczeck, K., and Daniluk-Matras, I.,"Intrahepatic Intestinal LoopThrough a Congenital Mesenteric Hernia." Journal of PediatricSurgery Case Reports 25 (2017): 44-45.
5. Mandhan, P., Alshahwani, N., Al-Balushi, Z., and Arain,A.,"Congenital Mesenteric Hernia in Neonates: Still a Dilemma."African Journal of Paediatric Surgery 12.3 (2015): 203-207.
Texas Pediatric Society Electronic Poster Contest
A female, born at 23 3/7 weeks, with a birth weight of 585g had an uneventful course tolerating full enteral feeds,stable on nCPAP support. At the age of 2 weeks, the infantacutely decompensated with metabolic acidosis, apnea,hypotension resulted in acute respiratory failure requiringintubation and vasopressor support. Abdominal examwhich was unremarkable initially, showed moderatetenderness and distension twelve hours after initialpresentation. Serial X rays showed non-specific boweldilation. There were no evidence of pneumatosis orintraperitoneal air. The abdominal ultrasound showedsmall volume complex ascites with moderate bowel wallthickening and diminished peristaltic activity. Due to lackof clinical improvement, patient underwent a bedsidelaparotomy that showed several loops of ischemic bowelherniating through a distal ileal mesenteric defect (Figs 1& 2). 22 cm of distal ileum was resected with creation ofan ileostomy. Post-op course was complicated by a rightsided grade IV intraventricular hemorrhage. Infantremained on partial parenteral nutrition until re-anastomosis performed 8 weeks later. The infant wasdischarged home on full enteral feeds at the age of 4months, corrected to 44 weeks and 2 days.
Fig. 2- Intraoperative image showing ischemic bowel
Fig. 1- Intraoperative image showing the mesenteric defect
Fig. 3- Illustration of various types of internal hernias. A= paraduodenal, B= formen of Winslow, C= intersigmoid, D= pericecal, E= transmesenteric, and F= retroanastomotic.
Abstract
An former 23 3/7 week preterm with a birth weight of 585grams developed abdominal distension and tendernesswith multi organ failure at the post menstrual age of of 253/7 weeks. Diagnostic imaging failed to show evidence ofeither NEC or SIP. Laparotomy revealed ischemic bowel asa result of internal herniation through a mesenteric defect.Ischemic bowel was resected and an ileostomy wascreated. This case highlights the challenges in the diagnosisof rare causes of gastrointestinal emergencies in a pretermneonate. It also stressed the need for close monitoring,and reliance on clinical examination to to guideintervention is such scenarios.
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