Fetal and Pediatric Pathology, 34:9–13, 2015 C Informa Healthcare USA, Inc. Copyright ISSN: 1551-3815 print / 1551-3823 online DOI: 10.3109/15513815.2014.947541
ORIGINAL ARTICLE
Prenatal Diagnosis of the Acute Meconium Peritonitis Secondary to Ileum Volvulus Perforation: A Case Report ˘ Keskin,1 Kazım Emre Karasahin,1 Mustafa Ozturk,1 Cuneyt ¨ Ugur Atabek,2 ˘ 2 and Ali Ergun ¨ 1 Suzi Demirbag, 1
Department of Obstetrics and Gynaecology, Gulhane Military Medical Academy and Medical School, Ankara, Turkey; 2 Department of Pediatric Surgery, Gulhane Military Medical Academy and Medical School, Ankara, Turkey
This is an unusual case in comparison to other sonographically described prenatal cases due to very early diagnosis and surgical intervention following prompt delivery. A 40-year-old pregnant, ultrasonography showed presence of cystic structure in the fetal abdomen that was consistent with intestinal dilatation. At 32 weeks’ of gestation, repeat ultrasound showed collapse of the bowel dilatation along with the presence of hyperechogenic fluid in the fetal abdominal cavity. Cesarean section was performed. The clinical utility of this report is the recognition that meconium peritonitis (MP) may be diagnosed in the acute phase with typical ultrasound features, and should be considered in the differential diagnoses of cases presented with reduced fetal movements. Although it appears that morbidity and mortality in MP cases depend upon gestational age, this case report may help to manage similar cases for defining the appropriate delivery time and treatment modality after prenatal identification of the problem. Keywords: meconium peritonitis, management, prenatal diagnosis, ultrasonography
INTRODUCTION There have been increasing numbers of fetuses with meconium peritonitis (MP) prenatally diagnosed by ultrasonography. MP is defined as a sterile chemical peritonitis induced by meconium extruding into the peritoneal cavity through a perforation in the intestine, proximal to the site of obstruction and causing peritoneal irritation. Little is known about its natural history. When the diagnosis is made, the main concern are when and how to deliver the baby. However, due to limited case series, recent literature does not provide clear guidelines concerning the antepartum or neonatal management of MP. We present an unusual case in which diagnosis was made shortly after bowel perforation at 32 weeks’ of gestation and definitive surgery was performed following prompt Cesarean delivery.
Received 23 May 2013; Revised 14 July 2014; accepted 19 July 2014. Address correspondence to: Mustafa OZTURK, MD, Specialist (Obstetrics and Gynaecology), GATA Kadın Hastalıkları ve Do˘gum Anabilim Dalı, 06018 Etlik/Ankara, Turkey. E-mail: [email protected]
U. Keskin et al.
CASE REPORT A 40-year-old woman, gravida 4 para 2, was referred to our tertiary center at 28 weeks’ of gestation because of prior recurrent pre-eclampsia. The present pregnancy had been uneventful including second trimester genetic amniocentesis. Ultrasound examination at admission showed presence of a cystic structure in the fetal abdomen that was consistent with intestinal dilatation (Figure 1). The fetal growth parameters, placental structure and amniotic fluid volume were all normal. Detailed ultrasound examination of fetal anatomy revealed no additional abnormalities. Maternal blood showed negative infection screen including TORCH and parvovirus. Follow-up sonographic examination at 30 and 32 weeks’ of gestation revealed the persistence of the bowel dilatation without a change in size. There was no ascites in the fetal abdomen. At 32 weeks’ of gestation, fetal biometric measurements were consistent with 29 + 6/7 weeks. One day later, the patient presented again with reduced fetal movements for 24 hours. Repeat ultrasound scan showed collapse of the bowel dilatation along with the presence of hyperechogenic fluid in the fetal abdominal cavity (Figure 2). Both parents were counseled regarding the possible diagnosis of fetal intestinal perforation and MP. The patient was admitted for intensive fetal surveillance and assessed also by a pediatric surgeon and neonatal care team. The patient expressed her desire to have a delivery as soon as possible. Fetal pulmonary maturation was induced by corticosteroid administration, then cesarean section was performed. A 1210 gram baby boy was delivered. Apgar scores were 8 and 9 at 1 and 5 min, respectively. Laparotomy and bowel resection were performed within the first day following delivery. The operative findings were volvulus at the level of terminal ileum, with a perforation in the distal segment of it, and intra-abdominal meconium (Figure 3). Ileal volvulus resection and end-to-end anastomosis were performed. Postsurgery follow through study showed that the anastomosis was not functioning. On day three of life the anastomosis was redone. The newborn remained hospitalized for 10 days receiving close neonatal and postoperative care. Pathological report revealed
Figure 1. Ultrasound examination at admission showed presence of a cystic structure in the fetal abdomen that was consistent with intestinal dilatation. Fetal and Pediatric Pathology
Prenatal Diagnosis-Meconium Peritonitis
Figure 2. Ultrasound scan showed collapse of the bowel dilatation along with the presence of hyperechogenic fluid in the fetal abdominal cavity.
distal ileum vascular congestion, intestinal wall bleeding and areas of acute inflammation. The final diagnosis was a MP due to perforated ileum volvulus. Apart from suboptimal weight gain, the infant is healthy at five months of age. DISCUSSION New modalities in fetal imaging have increased the rate and accuracy of prenatal diagnosis, with vast improvements in visualization and understanding of fetal abdomen.
Figure 3. The operative findings were volvulus at the level of terminal ileum, with a perforation in the distal segment of it, and intra-abdominal meconium. C Informa Healthcare USA, Inc. Copyright
U. Keskin et al.
However, the differential diagnosis of a sonographically visualized intra-abdominal cyst in a fetus is extensive, and includes intestinal duplication cyst, mesenteric cysts, choledochal cyst, meconium pseudocyst, congenital cyst of the pancreas, renal cyst, obstructive uropathy, urachal cyst, ovarian cyst, ureterocele, and tumorous lesions such as cystic sacroccocygeal teratomas. Possible causes of bowel perforation include mesenteric vascular insufficiency and bowel obstruction such as meconium ileus, intestinal atresia or stenosis, volvulus, intussusception, duplication, and extrinsic band [1]. Intestinal stenosis or atresia and meconium ileus account for 65% of the cases [2]. Dilated bowel loop and/or polyhydramnios are usually associated with bowel obstruction [1]. In the present case, lack of progressive increase in amniotic fluid volume or fetal ascites on follow-up ultrasound scans as well as sudden appearance of intraabdominal hyperechogenic fluid accumulation and disappearance of persistent cystic mass strongly suggested that there was an acute and ongoing intestinal perforation. Although the course of sonographic findings made the atresia or stenosis unlikely to be the possible cause of intestinal obstruction, we were not able to be sure of volvulus being the primary etiology at any point prenatally. We did not need to determine the bilirubin level in the fetal ascites by way of paracentesis, however, during the acute phase of MP, this invasive procedure can be used to establish the diagnosis [3]. Moreover, normalization of bilirubin levels may be used as a sign of spontaneous closure of bowel perforation in those already known to have MP. It is not clear whether earlier diagnosis and treatment of MP improves the prognosis of fetuses remote from term. In earlier reports, there was nearly 80% rate which has been described in spontaneous intrauterine remission. It is proposed that meconium and digestive enzymes are released at the site of the perforation and cause sterile inflammation of the peritoneum that leads to the accumulation of ascites. The subsequent adhesion between bowel loop and omentum can circumscribe the ascitic fluid, resulting in the formation of an abdominal pseudocyst. Therefore, closure of the perforation and clearance of the underlying bowel obstruction can lead to spontaneous remission, in which no postnatal surgery is required [4]. On the other hand, it is argued that meconium contains digestive enzymes such as trypsin that induce aseptic peritonitis. Extensive inflammation could inhibit spontaneous sealing of the perforation and inflammation-induced bowel edema could aggravate the underlying bowel obstruction. Prenatal sonography cannot define the persistence of intestinal perforation [5], and no marker has been reported to reliably predict the clinical course of meconium perinonitis in “wait and see” circumstances. Recent larger studies suggest that 76–100% of prenatally diagnosed cases do not resolve spontaneously [6], and persistent ascites, pseudocyst, or dilated bowel loop are most sensitive (92%) ultrasound findings to predict postnatal surgery [7]. In the present case, prior stillbirth of the woman at the third trimester, impending onset of fetal growth restriction, strong parental desire to have delivery as soon as possible, and preferences of pediatric surgeons in favor of surgical intervention outweighed the expected morbidities related to prematurity and surgery. In conclusion, this is an unusual case in comparison to other sonographically described prenatal cases due to very early diagnosis and surgical intervention following prompt delivery. The clinical utility of this report is the recognition that MP may be diagnosed in the acute phase with typical ultrasound features, and should be considered in the differential diagnoses of cases presented with reduced fetal movements. Although it appears that morbidity and mortality in MP cases depend upon gestational age, the complexity and the nature of the underlying lesion, process interval, presence of the cystic fibrosis or congenital anomalies, and complications of treatment or expectant management, this case report may help to manage similar
Fetal and Pediatric Pathology
Prenatal Diagnosis-Meconium Peritonitis
cases for defining the appropriate delivery time and treatment modality after prenatal identification of the problem. ACKNOWLEDGEMENTS We would like to thank Dr Tarik Purtuloglu, an assistant professor from Department of Anesthesia in this case report. Declaration of Interest The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper. REFERENCES [1] Forouhar F. Meconium peritonitis. Pathology, evolution, and diagnosis. Am J Clin Pathol. 1982;78:208–213. [2] Valladares E, Rodr´ıguez D, Vela A, et al. Meconium pseudocyst secondary to ileum volvulus perforation without peritoneal calcification: a case report. J Med Case Rep. 2010;4:292–296. [3] Chen FY, Chen M, Shih JC, et al. Meconium peritonitis presenting as isolated massive fetal ascites. Prenat Diagn. 2004;24:930–931. [4] Chalubinski K, Deutinger J, Bernaschek G. Meconium peritonitis: extrusion of meconium and different sonographical appearances in relation to the stage of the disease. Prenat Diagn 1992;12:631–636. [5] Yanfang Z, Chun H, Limin W, et al. Fetal meconium peritonitis complicated with bacterial infection. J Clin Ultrasound. 2008;36(5):312–314. [6] Izumi Y, Sato Y, Kakui K, et al. Prenatal treatment of meconium peritonitis with urinary trypsin inhibitor. Ultrasound Obstet Gynecol. 2011;37(3):366–368. [7] Shyu MK, Shih JC, Lee CN, et al. Correlation of prenatal ultrasound and postnatal outcome in meconium peritonitis. Fetal Diagn Ther. 2003;18(4):255–261.
C Informa Healthcare USA, Inc. Copyright
Copyright of Fetal & Pediatric Pathology is the property of Taylor & Francis Ltd and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
ORIGINAL ARTICLE
Prenatal Diagnosis of the Acute Meconium Peritonitis Secondary to Ileum Volvulus Perforation: A Case Report ˘ Keskin,1 Kazım Emre Karasahin,1 Mustafa Ozturk,1 Cuneyt ¨ Ugur Atabek,2 ˘ 2 and Ali Ergun ¨ 1 Suzi Demirbag, 1
Department of Obstetrics and Gynaecology, Gulhane Military Medical Academy and Medical School, Ankara, Turkey; 2 Department of Pediatric Surgery, Gulhane Military Medical Academy and Medical School, Ankara, Turkey
This is an unusual case in comparison to other sonographically described prenatal cases due to very early diagnosis and surgical intervention following prompt delivery. A 40-year-old pregnant, ultrasonography showed presence of cystic structure in the fetal abdomen that was consistent with intestinal dilatation. At 32 weeks’ of gestation, repeat ultrasound showed collapse of the bowel dilatation along with the presence of hyperechogenic fluid in the fetal abdominal cavity. Cesarean section was performed. The clinical utility of this report is the recognition that meconium peritonitis (MP) may be diagnosed in the acute phase with typical ultrasound features, and should be considered in the differential diagnoses of cases presented with reduced fetal movements. Although it appears that morbidity and mortality in MP cases depend upon gestational age, this case report may help to manage similar cases for defining the appropriate delivery time and treatment modality after prenatal identification of the problem. Keywords: meconium peritonitis, management, prenatal diagnosis, ultrasonography
INTRODUCTION There have been increasing numbers of fetuses with meconium peritonitis (MP) prenatally diagnosed by ultrasonography. MP is defined as a sterile chemical peritonitis induced by meconium extruding into the peritoneal cavity through a perforation in the intestine, proximal to the site of obstruction and causing peritoneal irritation. Little is known about its natural history. When the diagnosis is made, the main concern are when and how to deliver the baby. However, due to limited case series, recent literature does not provide clear guidelines concerning the antepartum or neonatal management of MP. We present an unusual case in which diagnosis was made shortly after bowel perforation at 32 weeks’ of gestation and definitive surgery was performed following prompt Cesarean delivery.
Received 23 May 2013; Revised 14 July 2014; accepted 19 July 2014. Address correspondence to: Mustafa OZTURK, MD, Specialist (Obstetrics and Gynaecology), GATA Kadın Hastalıkları ve Do˘gum Anabilim Dalı, 06018 Etlik/Ankara, Turkey. E-mail: [email protected]
U. Keskin et al.
CASE REPORT A 40-year-old woman, gravida 4 para 2, was referred to our tertiary center at 28 weeks’ of gestation because of prior recurrent pre-eclampsia. The present pregnancy had been uneventful including second trimester genetic amniocentesis. Ultrasound examination at admission showed presence of a cystic structure in the fetal abdomen that was consistent with intestinal dilatation (Figure 1). The fetal growth parameters, placental structure and amniotic fluid volume were all normal. Detailed ultrasound examination of fetal anatomy revealed no additional abnormalities. Maternal blood showed negative infection screen including TORCH and parvovirus. Follow-up sonographic examination at 30 and 32 weeks’ of gestation revealed the persistence of the bowel dilatation without a change in size. There was no ascites in the fetal abdomen. At 32 weeks’ of gestation, fetal biometric measurements were consistent with 29 + 6/7 weeks. One day later, the patient presented again with reduced fetal movements for 24 hours. Repeat ultrasound scan showed collapse of the bowel dilatation along with the presence of hyperechogenic fluid in the fetal abdominal cavity (Figure 2). Both parents were counseled regarding the possible diagnosis of fetal intestinal perforation and MP. The patient was admitted for intensive fetal surveillance and assessed also by a pediatric surgeon and neonatal care team. The patient expressed her desire to have a delivery as soon as possible. Fetal pulmonary maturation was induced by corticosteroid administration, then cesarean section was performed. A 1210 gram baby boy was delivered. Apgar scores were 8 and 9 at 1 and 5 min, respectively. Laparotomy and bowel resection were performed within the first day following delivery. The operative findings were volvulus at the level of terminal ileum, with a perforation in the distal segment of it, and intra-abdominal meconium (Figure 3). Ileal volvulus resection and end-to-end anastomosis were performed. Postsurgery follow through study showed that the anastomosis was not functioning. On day three of life the anastomosis was redone. The newborn remained hospitalized for 10 days receiving close neonatal and postoperative care. Pathological report revealed
Figure 1. Ultrasound examination at admission showed presence of a cystic structure in the fetal abdomen that was consistent with intestinal dilatation. Fetal and Pediatric Pathology
Prenatal Diagnosis-Meconium Peritonitis
Figure 2. Ultrasound scan showed collapse of the bowel dilatation along with the presence of hyperechogenic fluid in the fetal abdominal cavity.
distal ileum vascular congestion, intestinal wall bleeding and areas of acute inflammation. The final diagnosis was a MP due to perforated ileum volvulus. Apart from suboptimal weight gain, the infant is healthy at five months of age. DISCUSSION New modalities in fetal imaging have increased the rate and accuracy of prenatal diagnosis, with vast improvements in visualization and understanding of fetal abdomen.
Figure 3. The operative findings were volvulus at the level of terminal ileum, with a perforation in the distal segment of it, and intra-abdominal meconium. C Informa Healthcare USA, Inc. Copyright
U. Keskin et al.
However, the differential diagnosis of a sonographically visualized intra-abdominal cyst in a fetus is extensive, and includes intestinal duplication cyst, mesenteric cysts, choledochal cyst, meconium pseudocyst, congenital cyst of the pancreas, renal cyst, obstructive uropathy, urachal cyst, ovarian cyst, ureterocele, and tumorous lesions such as cystic sacroccocygeal teratomas. Possible causes of bowel perforation include mesenteric vascular insufficiency and bowel obstruction such as meconium ileus, intestinal atresia or stenosis, volvulus, intussusception, duplication, and extrinsic band [1]. Intestinal stenosis or atresia and meconium ileus account for 65% of the cases [2]. Dilated bowel loop and/or polyhydramnios are usually associated with bowel obstruction [1]. In the present case, lack of progressive increase in amniotic fluid volume or fetal ascites on follow-up ultrasound scans as well as sudden appearance of intraabdominal hyperechogenic fluid accumulation and disappearance of persistent cystic mass strongly suggested that there was an acute and ongoing intestinal perforation. Although the course of sonographic findings made the atresia or stenosis unlikely to be the possible cause of intestinal obstruction, we were not able to be sure of volvulus being the primary etiology at any point prenatally. We did not need to determine the bilirubin level in the fetal ascites by way of paracentesis, however, during the acute phase of MP, this invasive procedure can be used to establish the diagnosis [3]. Moreover, normalization of bilirubin levels may be used as a sign of spontaneous closure of bowel perforation in those already known to have MP. It is not clear whether earlier diagnosis and treatment of MP improves the prognosis of fetuses remote from term. In earlier reports, there was nearly 80% rate which has been described in spontaneous intrauterine remission. It is proposed that meconium and digestive enzymes are released at the site of the perforation and cause sterile inflammation of the peritoneum that leads to the accumulation of ascites. The subsequent adhesion between bowel loop and omentum can circumscribe the ascitic fluid, resulting in the formation of an abdominal pseudocyst. Therefore, closure of the perforation and clearance of the underlying bowel obstruction can lead to spontaneous remission, in which no postnatal surgery is required [4]. On the other hand, it is argued that meconium contains digestive enzymes such as trypsin that induce aseptic peritonitis. Extensive inflammation could inhibit spontaneous sealing of the perforation and inflammation-induced bowel edema could aggravate the underlying bowel obstruction. Prenatal sonography cannot define the persistence of intestinal perforation [5], and no marker has been reported to reliably predict the clinical course of meconium perinonitis in “wait and see” circumstances. Recent larger studies suggest that 76–100% of prenatally diagnosed cases do not resolve spontaneously [6], and persistent ascites, pseudocyst, or dilated bowel loop are most sensitive (92%) ultrasound findings to predict postnatal surgery [7]. In the present case, prior stillbirth of the woman at the third trimester, impending onset of fetal growth restriction, strong parental desire to have delivery as soon as possible, and preferences of pediatric surgeons in favor of surgical intervention outweighed the expected morbidities related to prematurity and surgery. In conclusion, this is an unusual case in comparison to other sonographically described prenatal cases due to very early diagnosis and surgical intervention following prompt delivery. The clinical utility of this report is the recognition that MP may be diagnosed in the acute phase with typical ultrasound features, and should be considered in the differential diagnoses of cases presented with reduced fetal movements. Although it appears that morbidity and mortality in MP cases depend upon gestational age, the complexity and the nature of the underlying lesion, process interval, presence of the cystic fibrosis or congenital anomalies, and complications of treatment or expectant management, this case report may help to manage similar
Fetal and Pediatric Pathology
Prenatal Diagnosis-Meconium Peritonitis
cases for defining the appropriate delivery time and treatment modality after prenatal identification of the problem. ACKNOWLEDGEMENTS We would like to thank Dr Tarik Purtuloglu, an assistant professor from Department of Anesthesia in this case report. Declaration of Interest The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper. REFERENCES [1] Forouhar F. Meconium peritonitis. Pathology, evolution, and diagnosis. Am J Clin Pathol. 1982;78:208–213. [2] Valladares E, Rodr´ıguez D, Vela A, et al. Meconium pseudocyst secondary to ileum volvulus perforation without peritoneal calcification: a case report. J Med Case Rep. 2010;4:292–296. [3] Chen FY, Chen M, Shih JC, et al. Meconium peritonitis presenting as isolated massive fetal ascites. Prenat Diagn. 2004;24:930–931. [4] Chalubinski K, Deutinger J, Bernaschek G. Meconium peritonitis: extrusion of meconium and different sonographical appearances in relation to the stage of the disease. Prenat Diagn 1992;12:631–636. [5] Yanfang Z, Chun H, Limin W, et al. Fetal meconium peritonitis complicated with bacterial infection. J Clin Ultrasound. 2008;36(5):312–314. [6] Izumi Y, Sato Y, Kakui K, et al. Prenatal treatment of meconium peritonitis with urinary trypsin inhibitor. Ultrasound Obstet Gynecol. 2011;37(3):366–368. [7] Shyu MK, Shih JC, Lee CN, et al. Correlation of prenatal ultrasound and postnatal outcome in meconium peritonitis. Fetal Diagn Ther. 2003;18(4):255–261.
C Informa Healthcare USA, Inc. Copyright
Copyright of Fetal & Pediatric Pathology is the property of Taylor & Francis Ltd and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
