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1994-01-08-23 Meconium peritonitis, spontaneous resolution © de Catte www.thefetus.net/


Meconium peritonitis, spontaneous resolution

Luc De Catte, MD, An Loccufier, MD, Tony Waterschoot, MS, Carine Mares, MS, Claire Bourgain MD, Antoon De Backer, MD.

Address correspondence to: Luc De Catte, MD, Academic Hospital, Dept. of Obstetrics and Gynecology, Free University of Brussels, Laarbeeklaan 101, 1090 Brussels, Belgium. Ph: 32-2-477-6531; Fax: 32-2-477-6505

Introduction

Meconium peritonitis is a sterile inflammatory reaction in the fetal abdomen resulting from in utero bowel perforation that nearly always involves the small bowel. Its prevalence is 0.29 in 10,000 live-birthsl,2,3. In the neonatal period, meconium peritonitis presents as an intestinal obstruction and requires surgical exploration. This form was first described by Morgagni in 17613. On rare occasions, this chemical peritonitis heals spontaneously without clinical manifestation. Attention to this type of pathology was drawn by the finding of intraperitoneal, inguinal or scrotal masses or calcifications4. However, differential diagnosis with other intraperitoneal tumors or testicular neoplasm caused some unnecessary surgeries. Since the introduction of ultrasound, 40 cases of meconium peritonitis with associated findings, investigation and outcome have been reported. Recently, the natural history of meconium peritonitis and, even more exceptional, the extrusion of the meconium plug has been depicted2.

The prenatal diagnosis process should include: testing for cystic fibrosis, chromosomal abnormalities and congenital anatomical or structural anomalies as well as the chemical and histologic analysis of the peritoneal fluid obtained by fetal paracentes56. The resulting diagnosis is of paramount importance in formulating a management plan for the obstetrical and perinatal management of the patient.

We present a patient with spontaneous in utero regression of meconium peritonitis. Neonatal surgery was required to treat ileal atresia.

Case report

A G1P0 woman was referred to our unit for a detailed ultrasound scan after diagnosis of fetal ascites at 29 weeks of gestation. This 30-year-old woman became pregnant only after several attempts of intrauterine insemination. The first trimester of pregnancy evolved uneventfully. However, at 16 weeks of gestation, the patient was admitted for an acute appendicitis. Recovery was complete after appendectomy. A detailed sonogram at 21 weeks showed a normal developing fetus. At 29 weeks of gestation, abundant ascites with debris, a clustering of hyperechogenic intestines with no peristalsis and an echogenic spot was observed on the diaphragmatic aspect of the liver were observed (fig. 1-2).

Figure 1: Longitudinal view of the abdomen showing the ascites and clustering of the intestinal loops.

 

Figure 2: Magnified view of the abdominal wall and the ascites, demonstrating the meconium fragment in the ascites.

The differential diagnosis included a chromosomal malformation, congenital viral infection, meconium peritonitis secondary to bowel perforation due to cystic fibrosis or mechanical bowel obstruction.

Fetal blood and ascitic fluid were obtained for chromosomal, microbiological, DNA, liver enzymes and hematologic analysis. These analysis revealed a normal female karyotype. Fetal hematological values and fetal liver enzymes were normal. Fetal DNA analysis to exclude cystic fibrosis was negative for the most common deletions. Light microscopic analysis of fetal ascites, however, revealed intra- and extrahistiocystic dark-stained amorphous sediments and numerous inflammatory cells (fig. 3). The diagnosis of meconium peritonitis secondary to mechanical bowel obstruction and perforation was made.

 

Figure 3: Cytological analysis of the ascites shows macrophages and numerous polynuclear leukocytes and lymphocytes. Some of the macrophages contain intracytoplasmatic brownish sediment, consistent with meconium (Papanicolaou stain, 400x).

A remarkable recovery was sonographically observed over the next 4 weeks. Fetal ascites regressed spontaneously and bowel peristalsis reappeared. However, intestinal contents remained echogenic (fig. 4). At 38 weeks of gestation, labor was induced and a girl of 3000g was born.

  

Figure 4: After total resorption of ascites.

Physical examination revealed a healthy newborn. The abdomen was not distended, and a plain X-ray failed to show dilated bowel loops. However, small nonparenchymal calcified areas in the left hypochondrium were observed. A few hours after the first feeding, the baby started vomiting meconial fluid. Abdominal radiography revealed distension of the small bowel loops without intraperitoneal air. Surgical exploration of the neonatal abdomen demonstrated multiple small atretic ileal segments. A 25cm bowel fragment including the proximal dilated part was removed, followed by an end-to-end anastomosis (fig. 5).

Figure 5: The resected ileal bowel fragment consist of a large proximal dilatation and a atretic segment with perforation.

Postoperative evolution was uncomplicated, and the baby is thriving well. Serial sweat chloride tests performed in the neonatal period were negative. On histological examination, the submucosal layer of bowel wall showed various degrees of inflammatory reaction. Distal to the atretic area, a meconium plug was found. The ileal atretic segment was characterized by an intraluminal septum, causing a type 1 ileal atresia (fig 6). A small bowel perforation was surrounded by subserosal calcifications (fig. 7).

  

Figure 6: Atretic bowel segment, transverse section. Notice the two narrow lumina, separated by a mucosal septum, lined with a hypoplastic bowel wall. (HES, 40x).

Figure 7: Cross-section of the atretic bowel segment at the level of the perforation. Note the strong inflammatory reaction in the bowel wall and the perforation at the top of the image.

Discussion

Definition

Meconium peritonitis is a sterile chemical peritonitis which is most frequently the result of intrauterine small bowel perforations.

Prevalence

Meconium peritonitis is a rare condition occurring in 1/35,000 live births1. This figure is probably underestimated because of spontaneous regression of the inflammatory process and sealing of the perforated intestinal area, without neonatal clinical manifestations.

Etiology

Any condition causing bowel obstruction may be responsible for bowel distension and perforation, leading to a sterile chemical peritonitis. Meconium ileus, which related to cystic fibrosis in 90% of cases, accounts for less than 25%. More frequently, mechanical bowel obstruction is provoked by intestinal atresia, volvulus, intussusception or herniation. Exceptionally colonic aganglionosis, resulting in an aperistalsis and a microcolon, is responsible for a meconium ileus and perforation of the intestinal wall1,3,5.

Analysis of 40 cases of prenatally detected meconium peritonitis (table 1) revealed an etiologic factor in only 20 of them; intestinal obstruction due to volvulus or atresia; accounted for 75%(15/20); cystic fibrosis for 20% and congenital infection for 5% (1/20). The large majority of cases, however, lacked information about perinatal analysis for cystic fibrosis.

Pathology - pathogenesis

Meconium formation starts in the third month of gestation. Some components -lipases, bile acids and salts - are particularly irritating.

Dilatation of the bowel loops leads to local vascular impairment of the intestinal wall, necrosis and subsequent perforation. Some authors suggest that an intestinal hypoperfusion, as a result of fetal hypoxia, is the primary cause of bowel atresia and perforation. Bowel peristalsis forces meconium and digestive enzymes into the peritoneal cavity, resulting in an intense chemical inflammatory process. Within days, giant cells and histiocytes surround the extruded meconium to form foreign body granulomas and calcifications. Depending on the spread of the inflammatory response, three pathological types are distinguished. In the generalized type, characterized by diffuse peritoneal fibrotic thickening and calcium deposits, the meconium spread throughout the peritoneal cavity. The fibroadhesive variant, which is the most common, produces obstruction by adhesive bands sealing the perforated site. If the perforated site is not effectively sealed, a thick-walled cyst is formed by adhesion of the proximal bowel loops to the perforated site. So the perforated intestinal area communicates only with the newly formed pseudocyst, which is lined by a calcified wall.

Associated malformations

Cystic fibrosis is associated with meconium ileus and subsequent meconium peritonitis in about 15% of the cases3. Newer data that analyse the Cystic Fibrosis Transconductance Receptor gene suggest that the association is higher. Characteristic findings in the second and early third trimester include highly echogenic intra-abdominal masses. In the third trimester, often enlarged bowel loops are observed7. Parental carrier detection and prenatal diagnosis by DNA analysis is possible in about 70% of cases related to the mutation in DF508 allele on chromosome 78. Neonatal investigation should include repetitive sweat chloride tests.

Polyhydramnios is present in 10-64% of cases and has been attributed to difficulty in swallowing as the result of deficient bowel peristalsis2,9. On rare occasions, fetal hydrops may be present.

Diagnosis and differential diagnosis

Prenatal diagnosis is suspected when fetal intra-abdominal calcifications are observed, especially in association with fetal ascites and polyhydramnios5. Fetal bowel obstruction associated with fast developing fetal ascites or hydrops should alert the sonographer. On rare occasions, however, fetal ascites regresses, intestinal dilatation disappears and peristalsis reappears. Only hyperechoic area remains2,9. Sometimes, intra-abdominal meconium pseudocysts are the sole remnants of the meconium peritonitis. Fetal abdominal hyperechoic masses or pseudocysts have recently been associated with congenital infectionsll, chromosomal abnormalities and cystic fibrosis12,13. Differential diagnosis further includes hematometrocolpos, ovarian, urachal, mesenteric and retroperitoneal cysts, and rare intra-abdominal tumors8.

Prenatal investigation by fetal blood sampling should detect chromosomal abnormalities, rule out cystic fibrosis by DNA analysis and exclude congenital infection through fetal hematological, immunological and hepatic investigation. More precise information about fetal ascites and bowel perforation can be obtained by fetal paracentesis and histological analysis of the aspirated fluid. As observed in our case and as previously reported by Baxi6 an intense inflammatory reaction is present together with intra- and extracellular amorphous material. 

 Prognosis

Prenatal detected cases of meconium peritonitis most frequently have a fair prognosis, as proven by a rather low perinatal mortality rate (6/40). Exclusion of chromosomal or rare infectious etiologies results in a perinatal survival rate of more than 80% (table 1). In about 35% of cases no surgical exploration was needed. The intense chemical peritonitis may seal the intestinal perforation permanently. Fifty percent of the newborns had a laparotomy and intestinal exploration, with resection of atretic or perforated segments in most cases.

Long-term prognosis is strongly affected by the presence of cystic fibrosis resulting in pancreatic insufficiency and digestive disturbances, multiple respiratory infections and chronic lung disease. Therefore, prenatal investigation should include DNA analysis of the DF508 mutation on chromosome 7, or repetitive sweat-chloride tests in the neonatal period. However, the association of cystic fibrosis and meconium peritonitis has been made only in 15/40 reported cases: 4 neonates were found positive for cystic fibrosis.

Table 1: Review of the literature.

Gray background cells represent fatal outcome.

Age

Ascites

Calcifications

Dilated Bowel

Polyhydramnios

Etiology

Cystic fibrosis

Outcome

25 6

present

absent

present

present

Volvulus

?

delivery;surgery

18 14

present

present

absent

absent

Ileal perfuratiopresent

?

termination of pregnancy

 38 1

absent

present

absent

?

 Volvulus

?

 delivery;surgery
 32 1

absent

present

absent

absent

 Ileal atresia

absent

 delivery;surgery
 33 8

present

present

absent

present

 Cystic fibrosis

present

 delivery; no surgery
 26 2

present

present

absent

absent

 ?

?

 36w, c-section, no surgery
 ? 2

?

?

absent

?

 ?

?

 38w; fetal death
 32 2

present

present

absent

?

 ?

?

 38w; no surgery
 24 2

absent

present

absent

?

 ?

?

 38w; no surgery
 23 2

present

present

absent

?

 ?

?

 38w; c-section
 28 2

absent

present

absent

?

 ?

?

 39w; no surgery
 29 2

absent

present

absent

?

 ?

?

 40w; no surgery
 31 2

absent

present

absent

?

 ?

?

 38w; no surgery
 33 2

absent

present

absent

?

 ?

?

 40w; no surgery
 30 1

present

present

absent

present

 ?

absent

 delivered; no surgery
 32 1

absent

present

present

?

 ileal narrowing

absent

 delivered; surgery
 33 1

present

absent

absent

present

 cystic firbrosis

present

 delivered; surgery
 30 15

present

present

absent

present

 umbilical hernia with jejunal infarction

?

 delivered; surgery
 38 1

absent

present

absent

present

 ?

?

 no surgery; normal evolution
 31 1

absent

present

present

absent

 swall bowel obstruction

absent

surgery; normal evolution
 29 1

present

present

absent

present

 ?

?

 no surgery; normal evolution
 33 1

present

present

absent

present

 ileal atresia

?

 surgery; normal evolution
 37 1

present

absent

absent

present

 ileal volvulus

absent

 surgery; normal evolution
 25 1

absent

present

present

present

 ileal obstructions

?

 34w; neonatal death
 28 1

present

present

absent

present

 ?

absent

 surgery; normal evolution
 30 1

present

present

absent

absent

 ?

absent

 delivered; surgery
 38 1

present

present

absent

?

 jejunal atresia

?

 delivered; surgery
 33 1

absent

present

present

present

 ileal atresia

absent

 delivered; surgery
 28 4

absent

present

absent

present

 ?

?

 delivered; surgery
 34 1

absent

absent

present

?

 cystic fibrosis

present

 delivered; surgery
 32 16

present

present

present

present

 volvulus

?

 delivered; surgery
 32 16

present

present

present

absent

 volvulus cystic fibrosis

present

 delivered; surgery
 30 1

absent

present

absent

absent

 jejunal atresia

absent

 delivered; surgery
 24 17

present

present

absent

present

 congenital varicella

?

 fetal death; ileal and colonic perforation
 26 10

absent

present

absent

present

 meconium pseudocyst

?

 neonatal death
 23 1

absent

present

absent

absent

 ileal perforation

?

 neonatal death
 35 1

absent

present

absent

absent

 ?

absent

 delivered; surgery
 21 1

present

present

present

present

 multiple bowel atresia

?

 delivered; surgery
 28 9

present

present

present

present

 ?

absent

 delivered; no surgery
 37 1

present

present

present

?

 umbilical hernia

?

 delivered; surgery

 Management

Frequent sonographic observation permits the evaluation the amount of fetal ascites, the evolution of inta-abdominal calcification and the restoration of bowel peristalsis. Exclusion of chromosomal malformations, congenital infections and cystic fibrosis is an essential element in the further management. Early diagnosis may change the couple"s attitude towards termination of pregnancy5.

If meconium peritonitis resolves spontaneously, there is no need for induction of labor. Postnatal observation of bowel peristalsis and a plain radiography of the child"s abdomen should alert the pediatrician. Surgical exploration might be necessary.

In case of progressive deterioration of fetal condition with increasing amount of ascites, preterm delivery can be considered in a tertiary care center. Postnatal surgery will be required immediately. Cesarean section has not been proven to improve neonatal outcome5

References

1. Foster FA, Nyberg DA, Mahony BS, et al.; Meconium peritonitis: prenatal sonographic findings and their clinical significance. Radiology 165:661-5, 1987.

2. Chalubinski K, Deutinger J, Bernaschek: Meconium peritonitis: extrusion of meconium amd different sonographical appearances in relation to the stage of the disease. Prenat Diagn 12: 631-636, 1 992.

3. Forouchar F : Meconium peritonitis. Am J Clin Pathol 78: 208213, 1982.

4. Kenney PJ, Spirt BA, Ellis DA et al.: Scrotal masses caused by meconium peritonitis: prenatal sonographic diagnosis. Radiology 154: 362, 1985.

5. Romero R, Pilu G, Jeanty P et al.: Prenatal diagnosis of congenital anomalies. Norwalk: Appleton & Lange pp 243-245, 1986.

6. Baxi LV, Yeh MN, Blanc WA et al.: Antepartum diagnosis andmanagement of in utero intestinal volvulus with perforation. New Engl J Med 308: 1519-1521, 1983.

7. Caspi B, Elchalal U, Lancet M et al.: Prenatal diagnosis of cystic fibrosis: ultrasonographic appearance of meconium ileus in the fetus. Prenat Diagn 8: 379-382, 1988.

8. Cantazarite V, Wozniak P, Maida CS et al.: Meconium peritonitis.Fetus 3: 2770 4- 2770 7, 1993.

9. Williams J, Nathan RO, Worthen NJ: Sonographic demonstration of the progression of meconium peritonitis. Obstet Gynecol 64: 822-826, 1984.

10. McGahan JP, Hanson F: Meconium peritonitis with accompanying pseudocyst: prenatal sonographic diagnosis. Radiology 148: 125-126, 1983.

11. Forouzan 1: Fetal abdominal echogenic mass: early sign of intrauterine cytomegalovirus infection. Obstet Gynecol 80: 535-7, 1992.

12. Porter KB, Plattner MS: Fetal abdominal hyperschoic mass; diagnosis and management.Fetal Diagn Ther 7: 116-122, !992.

13. Dicke JM, Crane JP: Sonographically detected hyperechoic fetal bowel: significance and implications for pregnancy management. Obstet Gynecol 80: 778-82, 1992.

14. Blumenthal DH, Rushovich AM, Williams RK et al.: Prenatal sonographic findings of meconium peritonitis with pathologic correlation. JCU 10: 350-352, 1982.

15. Dunne M, Haney P, Sun CC: Sonographic features of bowel perforation and calcific meconium peritonitis in utero. Pediatr Radiol 13: 231-233, 1983.

16. Langer JC, Adzick NS, Filly RA et al.: Gastrointestinal tract obstruction in the fetus. Arch Surg 124: 1183-1187, 1989.

17. Lince DM, Pretorius DH,Manco-Johnson et al.: The clinical significance of increased echogenicity in the fetal abdomen. AJR 145: 683-686, 1985.

18. Skoll MA, MarquetteGP, Hamilton EF: Prenatal ultrasonic diagnosis of multiple bowel atresias. Am J Obstet Gynecol 156: 472-3, 1987.

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