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Table of Contents   
ORIGINAL ARTICLE
Year : 2019  |  Volume : 24  |  Issue : 2  |  Page : 120-123
 

Management strategy of meconium ileus-outcome analysis


Department of Pediatric Surgery, King George's Medical University, Lucknow, Uttar Pradesh, India

Date of Web Publication1-Mar-2019

Correspondence Address:
Dr. Anand Pandey
Department of Pediatric Surgery, King George's Medical University, Lucknow - 226 003, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiaps.JIAPS_41_18

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   Abstract 


Background: Meconium ileus (MI) is defined as an intestinal obstruction caused by the impaction of inspissated meconium in the terminal ileum. In this study, we have evaluated the nonoperative management of patients of simple MI without fluoroscopic support –an important requisite of the Noblett's criteria. Besides this, surgical management in cases of failed conservative management and complicated MI was also assessed.
Materials and Methods: This was a retrospective observational study. Various clinical and radiological parameters were evaluated. Conservative management included the use of water-soluble contrast diatrizoate meglumine and diatrizoate sodium. In case of nonpassage of meconium in 24 h from first intervention, exploratory laparotomy with ileostomy was performed. All complicated MI underwent exploratory laparotomy with creation of stoma as and when needed.
Results: The duration of this study was 6½ years. Twenty-five neonates of MI were admitted. Of these, 22 had simple MI and remaining three had complicated MI. Eighteen neonates responded to the conservative management. In four neonates, who did not respond, exploratory laparotomy was performed. All three neonates having complicated MI underwent exploratory laparotomy. One patient expired in follow-up.
Conclusion: MI is an important neonatal emergency, which needed immediate attention of a pediatric surgeon. Proper evaluation of the patient, careful application of principals of conservative management, and timely surgical intervention may fetch satisfactory results.


Keywords: Conservative management, meconium ileus, Noblett's criteria, simple meconium ileus


How to cite this article:
Singh AK, Pandey A, Rawat J, Singh S, Wakhlu A, Kureel SN. Management strategy of meconium ileus-outcome analysis. J Indian Assoc Pediatr Surg 2019;24:120-3

How to cite this URL:
Singh AK, Pandey A, Rawat J, Singh S, Wakhlu A, Kureel SN. Management strategy of meconium ileus-outcome analysis. J Indian Assoc Pediatr Surg [serial online] 2019 [cited 2019 Nov 14];24:120-3. Available from: http://www.jiaps.com/text.asp?2019/24/2/120/253345





   Introduction Top


Meconium ileus (MI) is defined as an intestinal obstruction caused by the impaction of thick, inspissated, protein-rich, adhesive, and desiccated meconium filling the distal part of the terminal ileum.[1],[2] It has been divided into two types: simple MI and complex MI. In the latter type, the obstruction is complicated by the associated gastrointestinal (GI) pathology, such as atresia, necrosis, or perforation.

Differentiation between simple and complex MI is difficult, but in latter, the plain X-ray abdomen may reveal marked air-fluid levels or intra-abdominal calcifications.[3] In the past, MI was considered to be closely associated with cystic fibrosis (CF). However, some recent studies have shown that MI may occur even in the absence of CF.[4.5]

After the success of Noblett's criteria in the treatment of simple MI,[6] it has been a standard in its management. However, as a prerequisite of the Noblett's criteria, fluoroscopy is needed. There may be a problem in following the criteria if fluoroscopy facility is missing. This is a reality in many parts of the world, including our setup.

In this study, we have evaluated the nonoperative management of patients of simple MI without fluoroscopic support. Besides this, we have also evaluated the surgical management in cases of failed conservative management and complicated MI.


   Materials and Methods Top


This was a retrospective observational study of patients of MI who were admitted in the Department of Pediatric Surgery of our Medical University. Since it was a retrospective study with no new intervention, ethical waiver was requested for conducting the study. The medical records of all patients from January 2011 to July 2017 were evaluated. The parameters evaluated were age, birth weight, sex, and date of admission. Clinical presentation of the patients, finding of the abdominal X-ray, and lower GI dye study were assessed. The type of operation performed, duration of hospital stays, and mortality, if any, were also noted.

It was difficult to differentiate between CF and nonCF neonates as the genetic testing, and the sweat chloride test for CF is not available at our center.

The diagnosis of MI was made on clinical examination of the distended abdomen and lower GI dye study. The findings observed in radiologic study included the absence of typical air-fluid levels present in small bowel obstruction, microcolon and the presence of pellets in the bowel, and passage of some dye into the dilated bowel [Figure 1] and [Figure 2].
Figure 1: Clinical picture of a neonate showing distended abdomen

Click here to view
Figure 2: Lower gastrointestinal contrast study of the same neonate. Filling defects may be seen in the contrast. There is some passage of dye in the proximal dilated bowel, thereby ruling out atresia. No air-fluid levels are obvious

Click here to view


Initial resuscitation included the placement of infant feeding tube for gastric decompression. Intravenous fluid and antibiotics were administered. Urinary output was measured. Thereafter, X-ray abdomen was taken to look for any evidence of complicated MI such as pneumoperitoneum and intra-abdominal calcification.

Procedure for management of simple meconium ileus

After initial resuscitation, the patients were treated with diluted water-soluble contrast diatrizoate meglumine and diatrizoate sodium. A volume of 20 mL of contrast was mixed with an equal amount of normal saline. This mixture was administered per rectally with the help of 10 Fr infant feeding tube and 50 mL syringe. The mixture was pushed very gently, without any additional force till the time when resistance to the flow was felt. The procedure was abandoned at the point of resistance to the flow of mixture. After this, the anal orifice was packed with the small gauge piece to prevent efflux of contrast.

Heart rate, respiratory rate, and oxygen saturation were monitored in these patients. Those patients who passed meconium within 6 h were put on observation. In the remaining patients, another attempt of dye instillation was given. In case of nonpassage of meconium in 24 h from the first intervention, exploratory laparotomy with loop ileostomy was performed.

Complicated meconium ileus

All complicated MI underwent exploratory laparotomy with creation of stoma as and when needed.

In all patients, who underwent laparotomy, proximal and distal bowel cleaning was performed by instilling 1 ampule of N-acetyl cysteine diluted in 10 mL of saline three times a day.


   Results Top


The duration of this study was 6½ years. Twenty-five neonates of MI were admitted during this period. Of these, 22 had simple MI and the remaining three had complicated MI. The male-to-female ratio was 8:1.

In patients of simple MI, the mean age at presentation was 6.5 days (1–15 days). The mean weight was 2.6 kg (1.7–3.5 kg). Eighteen neonates responded to the conservative management and were successfully discharged. In four neonates, there was no response to the conservative management. These patients underwent exploratory laparotomy, which revealed thick tenacious meconium filling the terminal ileum. Loop ileostomy was made in these patients. Ileostomy started functioning on the 3rd postoperative day in all of them. The mean duration of stay was 7.5 days in patients who underwent conservative treatment (3–20 days). The duration of stay was longer in patients who underwent exploratory laparotomy (8–11 days). There was no mortality in this group; however, superficial wound dehiscence occurred in all of them. It was managed by secondary suturing after wound granulation.

All three neonates having complicated MI underwent exploratory laparotomy after resuscitation. Resection of part of the ileum with a double-barreled ileostomy was performed. The mean age at presentation was 4.2 days and mean weight was 2.8 kg. The duration of hospital stay was 19.6 days. One patient presented after 1 month of discharge with severe pneumonitis and expired on 2nd day of admission. The remaining two patients have successfully undergone ileostomy reversal.

The time gap between the first and second surgery was approximately 4 months in patients who underwent ileostomy formation.

The mean duration of follow-up was 7.08 months (range 5–9 months). None of the patients including those who underwent surgical intervention had any specific problem in the postoperative period or till the time they were in our follow-up. They did not need any additional supplementation nor developed malabsorption.


   Discussion Top


Conventionally, complicated MI is treated by laparotomy. Simple MI is initially treated by a conservative measure which includes Noblett's criteria.[6] Laparotomy is needed if conservative management fails. For application of Noblett's criteria, a set of prerequisite conditions are needed, one of which is the presence of fluoroscope. The scenario may be different in developing countries, where all facilities may not be available.

At the outset, it may be queried that normal saline rectal washouts would have achieved the same results as water-soluble contrast enema. Since it was not a comparative analysis between two treatment modalities, we cannot comment on it. However, it is to be noticed that diatrizoate meglumine and diatrizoate sodium are hyper-osmolar agents, which may be averaging approximately six times the osmolarity of normal serum.[7] This property will cause fluid to be drawn into the lumen, thereby making the thick menonium less viscid. This, we cannot achieve by using normal saline. Besides, we were not performing repeated rectal washouts. Rather, it was a contrast enema under controlled situations.

Patients with simple MI were initially treated with water-soluble contrast enema. The minimal invasive nature, the relatively high success rates, and the low complication rates make diluted water-soluble contrast diatrizoate meglumine and diatrizoate sodium enema the best choice in simple MI. In this study, it was safe and effective in resolving the obstruction in 80% of the patients with simple MI, which is comparable with other studies.[8],[9],[10],[11],[12],[13],[14] The use of diluted water-soluble contrast diatrizoate meglumine and diatrizoate sodium (saline, Tween 80, DNAse, N-acetyl cysteine, and gastrografin)[9] is however, not without risk. Perforations, hypovolemia, and electrolyte loss have been reported.[11] Since we were careful about these possibilities, none of our patients of simple MI suffered from any complications. In case of suspected pneumoperitoneum, confirmation may be needed with the help of an abdominal X-ray.

In case of failed contrast enema or complicated MI, laparotomy is needed. A common procedure is enterotomy and placement of a tube for further bowel irrigation, if needed.[15] We have, however, proceeded with loop ileostomy, which is also a described procedure in the literature.[16] We feel that the loop gives access to both proximal and distal stoma, which is not possible with the tube enterostomy. Besides this, since it is not possible to predict the efficacy of bowel cleansing intraoperatively, we never opted for enterotomy, cleaning with a tube, and its immediate removal. As we feel that loop ileostomy is quick to perform, we did not go for other types of ileostomy. However, we do agree that ileostomy has its own disadvantages for its care, extrasurgical procedure, high-output diarrhea, and an increased hospital stay, etc.[17],[18]

In complex MI, surgical intervention is mandatory. Resection with anastomosis is the best option.[19],[20] It has been advocated mostly in atresia, volvulus, or perforation. Given the clinical condition of the patients, other options may be needed. Our series suggests that conservative management may be successful even if fluoroscope facility is not available. This may be regarded as a modification to the Noblett's criteria, which arose out of necessity. However, careful clinical attention is a must in such a scenario. The high success rate in our series may also be attributed to the possibility of the absence of CF etiology of MI. It has been observed that MI due to non-CF is more amenable to an enema therapy.[21] Since none of our patients had any problem in the postoperative period or during follow-up, we did not go for further workup to evaluate any other possible etiology. However, we do agree that if there is any problem during follow-up, other possible etiologies such as small left colon syndrome in patients whose mothers may have diabetes mellitus or remote possibility of Hirschsprung's disease may need to be assessed. Still, it is to be noted that small left colon syndrome is a distinct clinical entity having a characteristic radiological picture. In contrast enema, there is reduced bowel caliber from the anus to the splenic flexure, and there is generally a zone of transition at the splenic flexure. This zone of transition is often longer than what we see with Hirschsprung's disease.[22] These features were not seen in our series.


   Conclusion Top


MI is an important neonatal emergency, which needs immediate attention of pediatric surgeon. Proper evaluation of the patient, careful application of principles of conservative management, and timely surgical intervention may fetch satisfactory results.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Carlyle BE, Borowitz DS, Glick PL. A review of pathophysiology and management of fetuses and neonates with meconium ileus for the pediatric surgeon. J Pediatr Surg 2012;47:772-81.  Back to cited text no. 1
    
2.
O'Neill JA Jr., Grosfeld JL, Boles ET Jr., Clatworthy HW Jr. Surgical treatment of meconium ileus. Am J Surg 1970;119:99-105.  Back to cited text no. 2
    
3.
Wyllie R. Intestinal atresia, stenosis and malrotation. In: Klieg Mann RM, Behrman RE, Jenson HB, Wyllie R, editors. Nelson Textbook of Pediatrics. 18th ed. Philadelphia PA: Saunders Elsevier; 2007. p. 1559-62.  Back to cited text no. 3
    
4.
Gorter RR, Karimi A, Sleeboom C, Kneepkens CM, Heij HA. Clinical and genetic characteristics of meconium ileus in newborns with and without cystic fibrosis. J Pediatr Gastroenterol Nutr 2010;50:569-72.  Back to cited text no. 4
    
5.
Fakhoury K, Durie PR, Levison H, Canny GJ. Meconium ileus in the absence of cystic fibrosis. Arch Dis Child 1992;67:1204-6.  Back to cited text no. 5
    
6.
Noblett HR. Treatment of uncomplicated meconium ileus by gastrografin enema: A preliminary report. J Pediatr Surg 1969;4:190-7.  Back to cited text no. 6
    
7.
Ott DJ, Gelfand DW. Gastrointestinal contrast agents. Indications, uses, and risks. JAMA 1983;249:2380-4.  Back to cited text no. 7
    
8.
Burke MS, Ragi JM, Karamanoukian HL, Kotter M, Brisseau GF, Borowitz DS, et al. New strategies in nonoperative management of meconium ileus. J Pediatr Surg 2002;37:760-4.  Back to cited text no. 8
    
9.
Boyd A, Carachi R, Azmy AF. Gastrograffin Enema in meconium ileus: The persistent approach. Pediatr Surg Int 1988;3:139-40.  Back to cited text no. 9
    
10.
Rescorla FJ, Grosfeld JL. Contemporary management of meconium ileus. World J Surg 1993;17:318-25.  Back to cited text no. 10
    
11.
Ein SH, Shandling B, Reilly BJ, Stephens CA. Bowel perforation with nonoperative treatment of meconium ileus. J Pediatr Surg 1987;22:146-7.  Back to cited text no. 11
    
12.
Copeland DR, St Peter SD, Sharp SW, Islam S, Cuenca A, Tolleson JS, et al. Diminishing role of contrast enema in simple meconium ileus. J Pediatr Surg 2009;44:2130-2.  Back to cited text no. 12
    
13.
Nguyen LT, Youssef S, Guttman FM, Laberge JM, Albert D, Doody D, et al. Meconium ileus: Is a stoma necessary? J Pediatr Surg 1986;21:766-8.  Back to cited text no. 13
    
14.
D'Agostino S, Musi L, Fabbro MA. Uncomplicated meconium ileus: Efficacy of enterotomy and bowel irrigation. Pediatr Surg Int 1995:10;329-31.  Back to cited text no. 14
    
15.
Venugopal S, Shandling B. Meconium ileus: Laparotomy without resection, anastomosis, or enterostomy. J Pediatr Surg 1979;14:715-8.  Back to cited text no. 15
    
16.
Bishop HC, Koop CE. Management of meconium ileus; resection, roux-en-Y anastomosis and ileostomy irrigation with pancreatic enzymes. Ann Surg 1957;145:410-4.  Back to cited text no. 16
    
17.
Del Pin CA, Czyrko C, Ziegler MM, Scanlin TF, Bishop HC. Management and survival of meconium ileus. A 30-year review. Ann Surg 1992;215:179-85.  Back to cited text no. 17
    
18.
Mak GZ, Harberg FJ, Hiatt P, Deaton A, Calhoon R, Brandt ML, et al. T-tube ileostomy for meconium ileus: Four decades of experience. J Pediatr Surg 2000;35:349-52.  Back to cited text no. 18
    
19.
Steiner Z, Mogilner J, Siplovich L, Eldar S. T-tubes in the management of meconium ileus Pediatr Surg Int 1997;12:140-1.  Back to cited text no. 19
    
20.
Jawaheer J, Khalil B, Plummer T, Bianchi A, Morecroft J, Rakoczy G, et al. Primary resection and anastomosis for complicated meconium ileus: A safe procedure? Pediatr Surg Int 2007;23:1091-3.  Back to cited text no. 20
    
21.
Ziegler MM. Meconium ileus. In: Coran AG, Adzick NS, Krummel TM, Laberge J, Shamberger RC, Caldmone AA, editors. Pediatric Surgery. 7th ed. Philadelphia PA: Elsevier Saunders; 2012. p. 1073-84.  Back to cited text no. 21
    
22.
Stewart DR, Nixon GW, Johnson DG, Condon VR. Neonatal small left colon syndrome. Ann Surg 1977;186:741-5.  Back to cited text no. 22
    


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