Clinical Perinatal/Neonatal Case Presentation ⅢⅢⅢⅢⅢⅢⅢⅢⅢⅢⅢⅢⅢⅢ

Eric Reynolds, MD ileostomy and jejunostomy. An intestinal volvulus was also reduced. Brenda Douglass, MD After surgery, the baby’s severe respiratory difficulty quickly improved, John Bleacher, MD but she continued to have a small supplemental oxygen requirement for several days. Her postoperative course was complicated by feeding Meconium peritonitis can have a wide range of presentations. This report discusses two cases that have recently appeared in our neonatal intensive care unit. The first report discusses the case of a meconium pseudocyst in a preterm infant. The second case reports on a newborn baby with a healed bowel perforation during the prenatal period. Finally, a brief discussion of meconium peritonitis is also included. Journal of Perinatology 2000; 3:193–195.

Case One: Meconium Pseudocyst in a Preterm Infant K.M. is a newborn female delivered at 33 weeks’ gestation to a 37-year-old mother. The was complicated only by pre- term labor. Birth weight was 2210 gm, and Apgar scores were 3, 4, and 7 at 1, 5, and 10 minutes, respectively. The child was noted at birth to have marked abdominal distention to such a degree as to cause decreased respiratory function. The baby was intubated and given bolus intravenous fluids before transfer to the neonatal intensive care unit (NICU). On arrival at the NICU, the baby was in moderate distress. Lung sounds were clear to auscultation but there was poor air movement. The heart was without murmur, and it was difficult to evaluate the child’s femoral pulses due to the severe abdominal distention. The abdomen was described as “shiny,” with no bowel sounds heard and no visible bowel loops over the abdomen. There was a palpable mid-abdominal mass. An abdominal x-ray showed gas in the small bowel but not in the mid-to-low abdomen. An abdominal mass with a calcified rim had displaced the normal gas pattern upward in the abdomen (Figure 1). An abdominal sono- gram revealed a large fluid-filled mass in the lower abdomen with echogenic material within the mass (Figure 2). The rest of the abdominal organs were normal. At exploratory laparotomy, the child was found to have a large meconium pseudocyst in the lower abdomen. A copious amount of meconium was removed, and the pseudocyst was resected. A por- tion of necrotic small bowel was removed with construction of an

Departments of (E. R.), Neonatology (B. D.), and Pediatric Surgery (J. B.), Tod Children’s Hospital, Youngstown, OH.

Address correspondence and reprint requests to Eric Reynolds, MD, Department of Pediat- Figure 1. A,B: Abdominal x-rays of the baby from case one with meconium rics, Tod Children’s Hospital, 500 Gypsy Lane, Youngstown, OH 44501. pseudocyst. Note the calcified rim visible in both the flat plate and lateral views.

Journal of Perinatology 2000; 3:193–195 © 2000 Nature America Inc. All rights reserved. 0743–8346/00 $15 www.nature.com/jp 193 Reynolds, Douglass, and Bleacher Meconium Peritonitis

Figure 2. Abdominal sonogram of the baby from case one showing the calcified rim with echogenic material within the cyst.

difficulties including a requirement for intravenous hyperalimenta- tion due to the shortened segment of the small bowel proximal to the ileostomy. She also had meconium plugs that were dislodged follow- ing a radiocontrast enema. Chromosome studies were normal and a mutation analysis for was negative. The child gained weight on a combination of total parenteral nutrition and enteral feeding. Bowel reanastomosis was performed 7 weeks after the initial operation. The baby continued to do well and was discharged from the NICU on day of life 63. At the child’s 4-month well-baby visit, she Figure 3. Abdominal x-ray of the baby in case two with calcium deposition has continued to thrive and is developmentally appropriate. throughout the abdomen. Case Two: Healed Intestinal Perforation Before Birth K.T. is a newborn female infant delivered at 36 weeks’ gestation by obtained. These studies indicated that the baby had a small left colon; cesarean section secondary to fetal distress. The pregnancy was com- otherwise, the results were unremarkable. There was no malrotation plicated by poorly controlled gestational diabetes that required insulin or perforation present. The child advanced to full feedings without therapy and preterm labor. Prenatal ultrasound showed polyhydram- difficulty and was discharged to home on hospital day 15. nios and intra-abdominal calcifications. Membranes ruptured at delivery, and Apgar scores were 6 and 9 at 1 and 5 minutes, respec- tively. At delivery, the baby had hypoglycemia and was transferred to DISCUSSION the NICU for intravenous therapy. The cases presented here discuss two of the classic presentations of Physical examination on arrival at the NICU was significant for meconium peritonitis. Meconium peritonitis occurs in 1 out of 30,000 the baby being large for gestational age and having a heart murmur. live births and may present in several different ways.1 These include The baby was tachypnic, but no other signs of respiratory distress were free meconium in the peritoneal cavity (known as meconium as- present. cites), meconium pseudocyst, and intra-abdominal calcium deposi- The child’s hospital course was complicated by profound and tion.2 Meconium peritonitis arises following prenatal gastrointestinal persistent hypoglycemia that required intravenous therapy for 11 days perforation. The perforation during intrauterine life is usually second- despite what was otherwise normal oral intake. Cardiac workup re- ary to congenital intestinal obstruction, such as with meconium , vealed nonobstructive hypertrophic cardiomyopathy and a ventricu- atresia, stenosis, internal hernia, neonatal Hirschprung’s disease, or lar-septal defect. She developed congestive heart failure that required volvulus. Prenatal anoxic events can cause bowel ischemia with sub- medical treatment. sequent perforation giving rise to meconium peritonitis. Rarely, no Because of the intra-abdominal calcification on the prenatal definite cause is found.3–5 Because meconium ileus due to cystic ultrasound and abdominal x-ray (Figure 3), a thorough gastrointesti- fibrosis accounts for 25% to 40% of meconium peritonitis in the West- nal workup was done before starting feedings. A barium enema and ern hemisphere, cystic fibrosis should be ruled out in any newborn an upper gastrointestinal series with small bowel follow-through were with meconium peritonitis.1

194 Journal of Perinatology 2000; 3:193–195 Meconium Peritonitis Reynolds, Douglass, and Bleacher

After bowel perforation occurs, leakage of meconium into the nique and postoperative care, recent literature indicates that survival peritoneal cavity gives rise to meconium ascites. The extruded meco- is now near 100%.10 nium causes an intense inflammatory reaction that can lead to cal- cium deposition on the abdominal walls and other intra-abdominal References 1. Yang WT, Ho SY, Metreweli C. Case report: antenatal sonographic diagnosis of structures. If the perforation occurs early enough before delivery and meconium peritonitis and subsequent evolving meconium pseudocyst formation the leakage occurs over a considerable period, a fibrous wall may without peritoneal calcification. Clin Radiol 1997;52:477–9. 6 form around the meconium concentration, forming a pseudocyst. 2. Miller RC, Cockrell J. Prenatal intestinal perforation associated with meconium The cyst walls may contain calcium and thus be visible on an abdom- pseudocyst and ileal angiomatous malformation. J Pediatr Surg 1972;7:719–20. inal x-ray. Sonographic features of meconium pseudocysts include 3. Effmann EL, Griscom AH, Colodny AH, Vawter GF. Neonatal gastrointestinal thick, well-circumscribed, echogenic cyst walls with areas of focal masses arising late in gestation. AJR Am J Roentgenol 1980;135:681–6. 7 calcification and echogenic viscous contents. As recently as June 4. Deshmukh S, Deshpande MS, Rawat MS, Patil V, Kasliwal GJ. Meconium perito- 1997, only 10 cases of prenatal diagnosis of meconium peritonitis nitis with pseudocyst formation. Indian Pediatr 1982;19:542–3. could be found in the literature.1 5. Vanderhoof JA, Zach TL, Adrian TE. . In: Avery GB, Maternal polyhydramnios can be an early sign of intestinal mal- Fletcher MA, MacDonald MG, editors. Neonatology: Pathophysiology and Man- function, including meconium peritonitis.8 Other presenting signs agement of the Newborn. 4th ed. Philadelphia: JB Lippincott Company; 1994. p. include abdominal distention or other symptoms of abdominal ob- 614. struction or mass. 6. Lauer JD, Cradock TV. Meconium pseudocyst: prenatal sonographic and antena- The differential diagnosis of calcified intra-abdominal masses tal radiologic correlation. J Ultrasound Med 1982;1:333–5. should include intestinal duplication, hydrometrocolpos with imper- 7. Nyberg DA. Intra-abdominal abnormalities. In: Nyberg DA, Mahony BS, Preto- rius DH, editors. Diagnostic Ultrasound of Fetal Anomalies. St. Louis: Mosby forate anus, mesenteric cyst, neuroblastoma, Wilm’s tumor, teratoma, Year-Book; 1990. p. 342–94. hepatoblastoma, adrenal hemorrhage, adrenal tumor, adrenal cyst, 8. Olnick HM, Hatcher MB. Meconium peritonitis. JAMA 1953;152:582–4. and giant Meckel’s diverticulum.3,9 Calcification can occur within the 9. Kliegman RA. The fetus and the neonatal infant. In: Behrman RE, Kliegman bowel if a fistula is present between the bowel and urinary tract as RM, Arvin AM, editors. Nelson Textbook of Pediatrics. 15th ed. Philadelphia: WB urine mixes with meconium. Saunders Company; 1996. p. 436. Most babies with meconium peritonitis do well. At one time, the 10. Nakayama DK. Meconium ileus, meconium peritonitis, and meconium plug. In: presence of a meconium pseudocyst carried a uniformly poor progno- Nakayoma DK, Bose CL, Chescheir NC, Valley RD, editors. Critical Care of the sis. In 1982, survival was ϳ70%.6 Due to advances in surgical tech- Surgical Newborn. Armonk, NY: Futura Publishing Company; 1997. p. 362.

Journal of Perinatology 2000; 3:193–195 195