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Journal of Indian Association of Pediatric Surgeons
     Journal of Indian Association of Pediatric Surgeons
Official journal of the Indian Association of Pediatric Surgeons         
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CASE REPORT
Year : 2023  |  Volume : 28  |  Issue : 6  |  Page : 529-531
 

Congenital segmental dilatation of ascending colon with distal microcolon: A diagnostic dilemma


Department of Pediatric Surgery, IMS-Banaras Hindu University, Varanasi, Uttar Pradesh, India

Date of Submission11-Jun-2023
Date of Decision26-Jul-2023
Date of Acceptance07-Aug-2023
Date of Web Publication02-Nov-2023

Correspondence Address:
Seth Kachhap
Department of Pediatric Surgery, Banaras Hindu University, Varanasi - 221 005, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiaps.jiaps_132_23

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   Abstract 


A 5-day-old male presented with bilious vomiting, a grossly distended abdomen, and passage of a small amount of stool. The anal opening was at a normal position. X-ray abdomen showed a large bowel loop with a single air-fluid level occupying more than half of the abdominal width. On laparotomy, the ascending colon was dilated to form a pouch-like structure, and the ileum and appendix were opening into it. Colon distal to pouch was present as microcolon. Histopathology of the dilated segment was suggestive of congenital segmental dilatation (CSD). In CSD, the distal bowel is of normal caliber. This is a rare case of CSD of ascending colon with distal microcolon.


Keywords: Ascending colon, colonic atresia, congenital segmental dilatation, microcolon


How to cite this article:
Nandan R, Pandey V, Suresh G, Singh RK, Kachhap S. Congenital segmental dilatation of ascending colon with distal microcolon: A diagnostic dilemma. J Indian Assoc Pediatr Surg 2023;28:529-31

How to cite this URL:
Nandan R, Pandey V, Suresh G, Singh RK, Kachhap S. Congenital segmental dilatation of ascending colon with distal microcolon: A diagnostic dilemma. J Indian Assoc Pediatr Surg [serial online] 2023 [cited 2023 Nov 28];28:529-31. Available from: https://www.jiaps.com/text.asp?2023/28/6/529/389316





   Introduction Top


Intrauterine vascular accidents can lead to a spectrum of intestinal anomalies. At one end of the spectrum, there is dilatation as in congenital segmental dilatation (CSD), while at the other end is atresia and stenosis.[1] However, what type of vascular insult leads to CSD and what type leads to colonic atresia and stenosis are still not clear. Many theories have been proposed. Vascular insults of different impacts or those occurring at different times of embryonal development can result in two different pathologies. Vascular insults during early embryonal development lead to defective mesenchymal formation. This can present as colonic dilatation with absent appendices epiploicae, haustrations, and taenia coli as in CSD.[1] While vascular insult late during embryonal development after mesenchymal formation can result in colonic atresia or stenosis.[1] No passage of meconium distal to the atretic or stenotic site during the intrauterine period leads to microcolon. Hereby, we report a case of CSD of ascending colon with distal microcolon and discussed the possible diagnosis.


   Case Report Top


A full-term, 2300 g male child was delivered by vaginal delivery at hospital following an uncomplicated pregnancy. He presented to us on day 5 of life with bilious vomiting, a grossly distended abdomen, and passage of a small amount of stool. The stool was of thin caliber [Figure 1]a. The anal opening was at a normal position. A 5 French feeding tube could not be negotiated beyond 3 cm of the anal verge. On general physical examination, the anterior fontanelle was depressed. Capillary filling time was prolonged. Routine blood investigations revealed leukopenia (3100/mm3) and thrombocytopenia (58,000/mm3). The renal function test was normal. The erect X-ray abdomen showed multiple air-fluid levels and a large bowel loop with a single air-fluid level occupying more than half of the total abdominal width located in the right upper abdomen and displacing the small bowel to the left [Figure 1]b. We kept the differential of pouch colon with rectal atresia. After fluid resuscitation and intravenous antibiotics administration, an exploratory laparotomy was done. Dilated small bowel loops were seen. A pouch colon-like dilated bowel loop was seen in the right upper quadrant which could not be delivered out of the incision site. Hence, we decompressed it and delivered it. The ileum and appendix opened into the pouch [Figure 2]a. Taenia coli, appendices epiploicae, and haustrations were absent. Its mesentery was short and poorly developed. The pouch had a separate blood supply which was divided into a leash of vessels around it. The transverse colon, descending colon, and sigmoid colon were present as microcolon [Figure 2]a. Duodenojejunal flexure was present to the left of the vertebral column. The pouch was excised [Figure 2]b. A pinpoint opening in the pouch leads to the microcolon. An ileostomy was done. The distal bowel was brought out as a mucous fistula. Biopsy was sent from both ileostomy and distal bowel which showed ganglion cells. Postoperatively, the child succumbed to sepsis on postoperative day 2. Histopathology was suggestive of CSD. Colonic mucosa showed normal-appearing crypts along with marked submucosal widening by fibrosis and edema. Muscularis propria showed marked muscular hypertrophy of both circular and longitudinal layers with variable-sized number of ganglion cells. Serosa showed several thick-walled congested blood vessels. Appendix shows normal histology.
Figure 1: (a) X-ray abdomen. (b) Grossly distended abdomen, normal anal position, thread-like stool (arrow)

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Figure 2: (a) Ileum and appendix (arrow) opening into congenital segmental dilatation (CSD) of ascending colon which is in continuation with microcolon (dotted line). (b) Cut-section of CSD with appendix (black arrow) and small part of microcolon (white arrow)

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   Discussion Top


CSD is a rare developmental anomaly. In a review of CSD, Sergi et al. reported <150 cases.[2] Brawner and Shafer described the common features of CSD to be lack of radiologically motility of dilated segment, normal bowel proximal and distal to CSD, the absence of tenia coli, normal ganglion cells in CSD, proximal and distal bowel, and hypertrophy of both circular and longitudinal muscle layers.[3]

Making a diagnosis in this patient was challenging. Bilious vomiting, abdomen distention, the passage of a small amount of stool, and anal opening at a normal position gave an initial impression of Hirschsprung disease. X-ray abdomen was done which was suggestive of a pouch colon. A feeding tube could not be negotiated beyond 3 cm of the anal verge. Hence, we kept a differential diagnosis of pouch colon with rectal atresia. Since the child had presented late and was already in sepsis, we did not opt for other investigations such as barium enema and rectal biopsy. On exploratory laparotomy, the findings were least expected, i.e., ascending colon dilated into a pouch-like structure, followed by microcolon. The dilated ascending colon had no appendices epiploicae, haustrations, and taenia coli. Since the pouch-like colon was not in continuity with the genitourinary tract, the diagnosis of pouch colon was ruled out. A diagnosis of CSD was made. CSD is characterized by an abrupt transition between normal and dilated segments as in our patient.[4] The bowel proximal and distal to the dilated segment are normal. However, in our patient, the bowel distal to pouch was microcolon. The cause of microcolon in our patient was still not understood. Microcolon results from failure of passage of meconium through the colon during the intrauterine period. One of the causes of microcolon is Hirschsprung disease. Biopsy from the microcolon showed ganglion cells which rules out the possibility of Hirschsprung disease. We also considered the possibility of colonic atresia or stenosis, which can explain both distal microcolon and proximal dilatation of the colon. There was only a pinpoint passage leading from the pouch-like structure to the microcolon and the mesentery was intact. If colonic atresia or stenosis was responsible for the dilatation of the proximal colon, there would be no loss of appendices epiploicae, haustrations, and taenia coli in the dilated colon. Moreover, a separate blood supply to the dilated colon was seen in our patient which is seen in CSD. We, therefore, concluded that CSD may have been associated with colonic atresia or stenosis.

Among the various causes of neonatal intestinal obstruction, both CSD and colonic atresias are uncommon causes. A single case of colonic atresia with CSD of the sigmoid colon has been reported.[5] Our patient had colonic atresia with CSD of ascending colon which has never been reported before. A single case of colonic atresia with pouch colon has also been reported.[1] CSD is usually seen in children and is rare in neonates.[6] Uga et al. reported the first case of antenatally diagnosed case CSD which remained asymptomatic till 10 months after which he required surgery.[4] Neonates usually present with features of intestinal obstruction while older children present with history of constipation, failure to thrive, and anemia.

Besides intrauterine vascular accidents, strangulation of the intestine in vitelline vessels, omphalomesenteric duct or umbilical ring, neurogenic causes, and defective muscular development have also been proposed as etiological factors.[6] CSD is characterized by an abrupt transition between normal and dilated segments as in our patient.[4] The bowel proximal and distal to the dilated segment are normal. However, in our patient, the bowel distal to pouch was microcolon due to associated colonic atresia. CSD can affect any part of the intestine. In small intestines, the ileum, and in large intestines, the rectosigmoid colon is the most commonly affected site. In our patient, the ascending colon was affected. CSD has dilated tortuous serosal vessels supplied by a large marginal, colonic artery as seen in our patient also.[7]

The treatment of CSD is resection of the affected intestinal segment and anastomosis of the normal segments. The distal bowel in our patient was microcolon. Considering the poor general condition of the child and the disparity between proximal and distal bowel, ileostomy and mucous fistula of the distal colon was made. If the duodenum is involved, resection is avoided and tapering has been done to avoid damage to the ampulla of Vater.

Both CSD and colonic atresia are rare entities with different sets of clinical and radiological findings. A combination of both rare entities created a diagnostic dilemma. Early diagnosis and management of this rare association are important for good outcomes.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Mathur P, Gubbi S, Farooq A, Mittal P, Sharma G. Congenital pouch colon with colonic atresia- an unusual embryological association: A case report. J Neonatal Surg 2021;10:33.  Back to cited text no. 1
    
2.
Sergi C, Hager T, Hager J. Congenital segmental intestinal dilatation: A 25-year review with long-term follow-up at the medical university of Innsbruck, Austria. AJP Rep 2019;9:e218-25.  Back to cited text no. 2
    
3.
Brawner J, Shafer AD. Segmental dilatation of the colon. J Pediatr Surg 1973;8:957-8.  Back to cited text no. 3
    
4.
Uga N, Suzuki T, Sakurai K, Tsukamoto T, Tsuchiya T, Kondo Y, et al. Segmental dilatation of colon detected on prenatal ultrasound. J Pediatr Surg Case Rep 2019;45:101208.  Back to cited text no. 4
    
5.
Kothari P, Gowrishankar, Rastogi A, Dipali R, Kulkarni B, Gowrishankar. Congenital segmental dilatation of colon with colonic atresia. Indian J Gastroenterol 2005;24:123-4.  Back to cited text no. 5
[PUBMED]  [Full text]  
6.
Kaiser M, Castellani C, Singer G, Marterer R, Ratschek M, Till H. Huge Congenital segmental dilatation of the sigmoid colon in a neonate: A “rarity to meet” and a “challenge to treat”. Case Rep Pediatr 2016;2016:9685307.  Back to cited text no. 6
    
7.
Puri A, Choudhury SR, Yadav PS, Grover JK, Pant N, Chadha R. Congenital pouch colon and segmental dilatation of the colon: A report of two unusual cases. J Indian Assoc Pediatr Surg 2011;16:61-3.  Back to cited text no. 7
[PUBMED]  [Full text]  


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