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provided by Elsevier - Publisher Connector Pediatr Neonatol 2009;50(2):59−64

ORIGINAL ARTICLE

Clinical Manifestations in Infants with Symptomatic

Ming-Horng Tsai1,3,4, Shih-Ming Chu1,4, Reyin Lien1,4, Hsuan-Rong Huang1,4, Chih-Cheng Luo2,4*

1Division of Pediatric Neonatology, Department of , Chang Gung Memorial Hospital, Taoyuan, Taiwan 2Division of Pediatric Surgery, Department of Pediatrics, Chang Gung Memorial Hospital, Taoyuan, Taiwan 3Division of Pediatric Hematology/Oncology, Department of Pediatrics, Chang Gung Memorial Hospital, Taoyuan, Taiwan 4College of Medicine, Chang Gung University, Taoyuan, Taiwan

Received: Jun 25, 2008 Background: Meconium peritonitis (MP) develops when bowel perforation occurs Revised: Aug 12, 2008 in utero or soon after birth, resulting in leakage of meconium into the peritoneal Accepted: Sep 15, 2008 cavity. The clinical features are often variable and prenatal ultrasonography plays an important role in prenatal diagnosis. KEY WORDS: Methods: We conducted a retrospective review of neonates diagnosed with MP in intra-abdominal our hospital from January 1998 to December 2007. Prenatal examinations, postnatal calcification; presentations, investigations, management, patient outcomes, and possible causes were analyzed. meconium peritonitis; Results: Ten patients (five boys, five girls) diagnosed with MP were studied. The most meconium pseudocyst; common prenatal ultrasonographic finding was fetal ascites (7/10, 70%), followed neonates by polyhydramnios (3/10, 30%), bowel dilatation (3/10, 30%), intra-abdominal cal- cification (1/10, 10%), and (1/10, 10%). Two of our patients were completely normal on prenatal ultrasonography. Only one MP diagnosis (1/10, 10%) was definitely confirmed by prenatal ultrasonography due to the presence of ascites, bowel perforation and intra-abdominal calcification. Nine patients (90%) required surgical intervention because of bowel perforation (5), bowel atresia (2), obstruction due to (1), and intussusception (1). Peritonitis was of cystic type in one case (10%), fibroadhesive in five cases (50%), and generalized in four cases (40%). All 10 patients survived without long-term gastrointestinal complications. Conclusion: Prenatal ultrasonography can be diagnostic for MP, which should be con sidered in the differential diagnosis of patients presenting with ascites or ab dominal distension at birth. Close observation of postnatal clinical manifesta- tions and timely surgical intervention resulted in a high survival rate and favorable outcome in these patients at our hospital.

*Corresponding author. Division of Pediatric Surgery, Department of Pediatrics, Chang Gung Memorial Hospital, 5 Fu-Shing Street, Kwei-Shan, Taoyuan 333, Taiwan. E-mail: [email protected]

©2009 Taiwan Pediatric Association 60 M.H. Tsai et al

1. Introduction or ascites), or both. The confirmation of MP was difficult in some patients who were only found to Meconium peritonitis (MP) is a sterile chemical have plaques of calcification in the peritoneal cavity peri tonitis resulting from small bowel perforation discovered by radiological investigation after birth, in utero or soon after birth. It is reported to have and these patients were excluded from our study. an incidence of one in 30,000−35,000 live births.1−3 Prenatal ultrasonography is performed routinely Any cause of small bowel ischemic lesion or associ- in our institution in all after around 20 ated mechanical obstruction may result in MP, weeks of gestation. All fetuses with abnormalities including , idiopathic bowel per- detected by prenatal ultrasonography suggestive of foration, volvulus, intussusception, congenital bands, MP are reviewed, and neonatologists are consulted. and meconium plug syndrome in .4−6 Postnatal evaluation and discussion with a pediatric After leakage of meconium into the peritoneal cav- surgeon is arranged in our neonatal care unit, which ity, a secondary inflammatory response results in the is a tertiary referral center that also receives re- production of ascites, fibrosis, calcification and some- ferred cases from other local hospitals. times cyst formation. The clinical presentations after Data were retrospectively collected from pa- birth vary from completely sealed-off peritonitis with- tients’ charts and included; gestational age, findings out any symptoms, to severe peritonitis requiring of antenatal ultrasonography, birth weight, clinical emergency surgical intervention. Improvements in features, imaging findings, management strategies, prenatal diagnostic techniques and proper early man- operative findings, and outcome. Statistical analysis agement have reduced the mortality rate of MP pa- was performed using the SPSS statistical package tients from 50% to below 10% in recent decades.6−9 (SPSS 11.0 for Windows, Chicago, IL, USA). Patient In this study, we reviewed our experience of the data not satisfying the criteria for a normal distribu- treatment of neonates with MP, and analyzed the tion were analyzed using nonparametric techniques. pre natal ultrasonographic findings, postnatal fea- A p value < 0.05 was considered as significant. tures, management strategies, and possible causes.

3. Results 2. Materials and Methods Ten patients with MP were identified during the pe- The medical records of neonates with symptomatic riod from January 1988 to December 2007 (Table 1). MP hospitalized in Chang Gung Children’s Hospital There were five boys and five girls. The median from January 1988 to December 2007 were retro- gestational age was 36 weeks (range: 27−40 weeks), spectively reviewed. The diagnosis of MP was con- and the median birth weight was 2910 g (range: firmed either by postnatal laparotomy (showing 815−3120 g). No patient had any hereditary genetic meconium-stained ascites, meconium staining inside disorder or family history of cystic fibrosis. the peritoneal cavity, and/or pseudocyst) or by pre- Prenatal ultrasonographic abnormalities were de- natal ultrasonography (showing bowel perforation, tected in eight fetuses (four boys and four girls) at ges- intra-abdominal calcification, dilated bowel loops, tational ages ranging from 20−35 weeks (mean: 28.8

Table 1 Prenatal ultrasonographic findings in patients with symptomatic meconium peritonitis

Case Gestational age & Gestational age of Sex Prenatal ultrasonographic finding no. weight at birth prenatal findings (wk)

1 F 34 wk, 2700 g Hydrops fetalis 32 2 M 38 wk, 3105 g Fetal ascites 28 3 M 31 wk, 2065 g Normal Nil 4 M 36 wk, 2800 g Fetal ascites, polyhydramnios 35 5 M 40 wk, 3100 g Fetal ascites, bowel dilatation 25 6 F 37 wk, 2920 g Fetal ascites with calcification, 20 bowel perforation 7 F 35 wk, 3048 g Fetal ascites, bowel dilatation, 32 polyhydramnios, bowel obstruction 8 M 36 wk, 2898 g Bowel dilatation, megacolon 28 9 F 36 wk, 3120 g Fetal ascites, polyhydramnios 31 10* F 27 wk, 815 g Normal Nil

*Patient 10 was the “B” of a triple ; the other two siblings were normal. Meconium peritonitis in neonates 61 weeks). The most common prenatal ultrasonographic loops with a blind pouch 25 cm proximal to the ileoce- finding was fetal ascites (7/10, 70%), followed by cal valve, along with an ileo-ileal intussusception, polyhydramnios (3/10, 30%), bowel dilatation (3/10, were found in the distal blind end. Prenatal intussus- 30%), and hydrops fetalis (1/10, 10%). Two patients ception was diagnosed based on operative findings. were completely normal by prenatal ultrasound. Pre- Patient 3 was unique in his late-onset presentation natal ultrasonography showing ascites plus bowel of MP. The prenatal ultrasonography findings were perforation and/or dilatation or intra-abdominal nor mal, and his neonatal period was unremarkable, calcification was considered to be diagnostic for MP. except for mild respiratory distress due to his prema- Only one patient was definitely confirmed with MP turity. He was discharged home on his 19th day of life by prenatal ultrasonography. Hydrops fetalis was with full-diet feeding, but was soon readmitted be- detected in patient 1 by prenatal ultrasonography, cause of bilious , abdominal distension, and but his postnatal chest X-ray showed resolution of positive peritoneal signs. Emergency laparotomy was pleural effusion. performed because of suspected malrotation and Table 2 lists the clinical features and management midgut volvulus based on abdominal ultrasound. A strategies of the MP patients. Table 3 lists all clini- meconium pseudocyst and meconium-stained ascites cal manifestations of patients with meconium peri- with tight adhesion were found. Neither bowel per- tonitis. Patient 6 was the only patient with complete foration nor malrotation was noted. Postoperative resolution of MP. She presented postnatally with a ileus resulted in a prolonged hospital stay (54 days). palpable abdominal mass. All the other infants dem- Incarcerated inguinal hernia and bilateral scro- onstrated postnatal findings including varying degrees tal swelling were two of the less common manifes- of acute abdomen, and five patients developed res- tations of MP in our series. Bilateral scrotal swelling piratory distress requiring mechanical ventilation. or hydrocele was noted at birth, but progressed to Respiratory distress was caused by compression of bilateral inguinal hernia with left-side incarceration chest volume due to abdominal distension and/or on- requiring surgical repair in one patient at the age going peritonitis, except in patient 10, where respi- of 4 months. Meconium staining without calculus ratory distress syndrome was diagnosed and treated was found in the right sac during repair, and a per- with 200 mg/8 mL Surventa (Hospira Inc, North forated left sac with very edematous, fragile tissue Chicago, IL, USA). Plain abdominal X-ray on the first was also noted. The total-body X-rays of these two day showed only absent or abnormal bowel air, with patients failed to show any calcification in the calcifications seen in only four patients (4/10, 40%). scrotum or abdomen. Except for patients 3 and 6, bowel dilatation or ileus Preliminary enterolysis of fibro-adhesive meco- detected by plain film on the second day was the nium, resection of bowel atresia with restoration first clue of bowel obstruction. Abdominal sonogra- of bowel continuity, or repair of perforated ileum phy showed ascites containing echoic debris with/ were carried out in nine infants. Except for patient without bowel dilatation, which was compatible with 10 who was born prematurely at 27 weeks, patient MP in eight patients (8/10, 80%). Persistent perfora- 8 with midgut volvulus had the longest hospital tion of the bowel, presenting as pneumoperitoneum stay (140 days). The hospital stay of patients un- on the second day of life, could not be identified dergoing surgery ranged widely, from 12−140 days by prenatal or postnatal ultrasonography. (median: 31 days), depending on the development Most of the nine infants who required surgical in- of post-operative ileus, nosocomial infection, and tervention were operated on during their first–sixth other gastrointestinal complications. There were days of life, except for patient 3, who was operated no significant differences in gestational age, sex, on during his 19th day of life. Indications for surgical duration of ventilator use, and incidence of extra- intervention included postnatal bowel perforation gastrointestinal complications between patients (n = 5) and bowel obstruction (n = 4) suspected follow- with and without persistent postnatal bowel perfo- ing postnatal ultrasonography. In three cases, meco- rations. All the patients survived and had no symp- nium pseudocysts of varying sizes were found. These toms of cystic fibrosis during a follow-up period pseudocysts were fed by bowel atresia (n = 1), ileal ranging from 14 months to nearly 7 years. perforation (n = 1) or sealed-off bowel perforation without obvious cause (n = 1). Marked meconium- stained ascites and diffuse adhesions were encoun- 4. Discussion tered in all nine patients. The terminal ileum was the most common site of perforation (n = 3), while MP is defined as an aseptic chemical inflammation two patients had bowel perforations in the colon resulting from fetal bowel perforation and subse- and jejunum, respectively. Our patients with ileal quent meconium leakage in utero, or shortly after atresia tended to have sealed-off MP presenting at birth. Its association with cystic fibrosis is well birth. In patient 5, markedly dilated small bowel known in western countries10,11 and other causative 62 M.H. Tsai et al ption ileus Meconium Unknown Unknown Ileal atresia Unknown Unknown Unknown Ileal atresia Microcolon Full recovery Full recovery Full recovery Full recovery Intussusce Full recovery Full recovery Full recovery Full recovery Full recovery Outcome causes Possible Full recovery ity and respiratory distress syndrome. ileum and colon Jejunal perforation, diffuse ileal atresia and calcification An ileo-ileal Marked meconium staining and adhesion intussusception Nil Ileal perforation with tight adhesion Meconium pseudocyst Meconium-stained ascites Ileal perforation Meconium Operative findings end-to-end

Enterolysis, resection and Colon perforation, disuse volvulus, one segment

and atresia segment Enterolysis, partial and anastomosis Nil of ileal perforation resection of ileum the adhesion MP, resection of atresia and MP, end-to-back anastomosis meconium-coated bowel Enterolysis, freeing and severe adhesion Enterolysis for fibroadhesive Ileal atresia much Operative methods pneumoperitoneum end-to-end anastomosis atrophy of terminal pneumoperitoneum of small bowel resected calcification and adhesion Calcification

Imaging findings hernia respiratory distress minimal ascites, ascites, respiratory pneumoperitoneum end-to-end anastomosis meconium-stained ascites distress ascites, respiratory ascites, calcification, ascites, respiratory calcification ascites, respiratory distress, incarcerated bilious vomiting bilious vomiting, bloody stool, with peritoneal sign ascites, bilateral scrotal swelling ascites, respiratory pneumoperitoneum ileum resection, enterolysis pseudocyst Postnatal outcome and management of patients with meconium peritonitis Postnatal Postnatal clinical Postnatal

distress 10 *Abdominal distension, Bowel ileus 9 Abdominal distension, Bowel dilatation, ascites, Enterolysis, resection and Ileal perforation,

distress 8 Abdominal distension, Bowel dilatation, Enterolysis, reduction of Small bowel volvulus,

inguinal 5 Abdominal distension, Bowel dilatation 7 Abdominal distension, Bowel dilatation, Excision of dry pseudocyst Meconium pseudocyst, 6 mass Abdominal

4 Abdominal distension, Pneumoperitoneum Enterolysis, repair

3 Abdominal distension, Bowel dilatation distress 2 Abdominal distension, Bowel dilatation manifestation 1 Abdominal distension, Ascites, calcification, terminal Ileostomy, Table 2 Table Case no. *The abdominal distension and minimal ascites developed on the second day of life, respiratory distress was due to prematur Meconium peritonitis in neonates 63

and required surgical intervention in the immediate Table 3 Clinical manifestations of patients with meconium peritonitis postnatal period. This is compatible with previous reports that described intrauterine midgut volvu- Clinical manifestation Total (n = 10) lus as being commonly associated with complicated postnatal courses and high mortality.5,17,18 Midgut Abdominal distension 10 volvulus in our patient was thought to be associ- Ascites 8 ated with fibro-adhesive MP, but this was not the Respiratory distress 6 Intra-abdominal calcification 4 causative etiology, because prenatal bowel dilata- Pneumoperitoneum with tion and megacolon were detected 2 months be- peritoneal sign* 5 fore birth. Although volvulus is a well-known cause Polyhydramnios 3 of MP, MP caused by midgut volvulus has rarely been Bilious vomiting 2 reported in the literature, presumably because most Hydrocele 2 of these fetuses die in utero.16 Inguinal hernia 1 A higher proportion of patients in our series (90%) Abdominal mass 1 underwent surgery compared with other reports, *Pneumoperitoneum with peritoneal sign requiring surgical because we emphasized the ‘symptomatic’ patients. th intervention presenting on the second day of life. Patient 3 had onset of symptoms on his 19 day of life and presented with signs of acute abdomen. We considered him to be a case of sealed-off MP, etiologies include bowel atresia, volvulus, intus- without detection by prenatal ultrasonography. The susception, congenital band, and meconium plug tight adhesion inside his abdomen and meconium syndrome. Fetal bowel disease with transient hy- pseudocyst resulted in adhesion ileus and his full-diet poxia or vascular insufficiency, or infectious bowel feeding prompted his acute clinical presentation. damage, can account for ‘idiopathic’ or ‘simple’ Meconium has also been reported in the thoracic MP in patients without known etiologies or identi- cavity, in the pelvic soft tissues, and in the scrotum.19 fiable bowel abnormalities.12,13 Although patients Meconium migrating from the peritoneal cavity into with underlying abnormali- the fetal scrotum via a patent processus vaginalis ties are reported to be at increased risk of postna- is possible, and progressive herniation with bowel tal bowel obstruction and perforation,14 we failed loops has recently been reported.20,21 Although meco- to find any associated bowel abnormalities in most nium can lead to local calcification inside the scro- of our patients. Additionally, the identification of tum, granulomas and calcium deposits can regress postnatal bowel perforation depends on close clin- in patients with healed MP,22 which could possibly ical observation or plain abdominal X-rays performed account for the negative radiological findings in at close intervals, regardless of whether underly- our patients. ing bowel abnormalities exist or not. Two of our patients had completely normal pre- Some recent studies have reported a low diag- natal examinations. Prenatal ultrasonography might nostic yield of prenatal ultrasonography,7,8 whereas detect a sealed-off bowel but miss the period when others have emphasized the importance of de- fetal ascites or bowel dilatation are present. Prenatal tailed fetal ultrasound scans for providing prenatal fetal ascites can subsequently disappear. Patient diagnosis and proper perinatal management of 10, a preterm infant, may have suffered from bowel MP.9,15 Only one patient in our series was diagnosed perforation soon after birth, which could explain by prenatal ultrasonography, while two patients were her normal prenatal ultrasonographic examination. misdiagnosed with bowel obstruction and volvulus, Several case reports have described intrauter- respectively. In our experience, bowel dilatation and ine intussusception as a rare etiology of MP.23−25 ascites with echoic contents were the most com- Intrauterine intussusception, with or without lead- mon postnatal ultrasonographic findings, which were ing point, may result in bowel ischemia or ileal compatible with MP, but not diagnostic. The low atresia. This intrauterine bowel perforation may be percentage of intra-abdominal calcification found complicated by MP if bowel perforation develops, in our patients compared with those reported in and can be detected by serial prenatal ultrasonog- other recent publications accounted for this under- raphy.23−25 Patient 5 was found to have markedly recognized disease entity in our hospital.7,8,14,16 dilated small bowel loops with a blind pouch 25 cm Our observations revealed that all affected pa- proximal to the ileocecal valve, and an intussus- tients who required surgical intervention developed ception was found in the distal blind end during symptoms of acute abdomen during the second-to- postnatal laparotomy. He was diagnosed with intra- fifth postnatal day. The only exception was patient uterine intussusception based on postnatal operative 8, with fetal midgut volvulus, who presented with findings, and he underwent surgical exploration with respiratory distress and signs of pneumoperitoneum, resection of the ileum and end-to-end anastomosis. 64 M.H. Tsai et al

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