Congenital segmental dilatation of the colon is a rare cause of intestinal obstruction in neonates. We report a case of congenital segmental dilatation of the colon and highlight the clinical, radiological, and histopathological features of this entity. Proper surgical treatment was initiated on the basis of preoperative radiological findings.Keywords: congenital segmental dilatation of the colon, segmental dilation of intestines, neonatal intestinal obstructio

Al-Garni, Abdulhadi F.

AL-Omari, Hatim

Al-Zaiem, Feras M.

Al-Zaiem, Maher M.

Asghar, Asim A.

English

AJOL - African Journals Online

Congenital segmental dilatation of the colonMaher M. Al-Zaiem, Abdulhadi F. Al-Garni, Asim A. Asghar, Feras M. Al-Zaiemand Hatim AL-OmariCongenital segmental dilatation of the colon is a rare causeof intestinal obstruction in neonates. We report a case ofcongenital segmental dilatation of the colon and highlightthe clinical, radiological, and histopathological features ofthis entity. Proper surgical treatment was initiated on thebasis of preoperative radiological findings. Ann PediatrSurg 11:46–48 c 2015 Annals of Pediatric Surgery.Annals of Pediatric Surgery 2015, 11:46–48Keywords: congenital segmental dilatation of the colon, segmental dilationof intestines, neonatal intestinal obstructionPediatric Surgery Department, Maternity and Children Hospital, Mecca,Kingdom of Saudi ArabiaCorrespondence to Maher M. Al-Zaiem, CU, ABPS, Pediatric Surgery Department,Maternity and Children Hospital, 21955 Mecca, Kingdom of Saudi ArabiaTel: + 966 598 759 063; fax: + 966 125 530 400;e-mail: maher_zaiem@hotmail.comReceived 29 March 2014 accepted 26 May 2014IntroductionSegmental dilatation of the intestine is a rare conditioncharacterized by localized dilatation of the intestine andan abrupt transition between the normal and the dilatedsegments. It was first reported by Swenson and Rathauserin 1959 [1].Segmental dilatation of the intestine can affect any partof the gastrointestinal tract from the duodenum to therectum. Congenital segmental dilatation of the colon(CSDC) is a rare entity; it has been described mostly inchildren [2]. It is even more rare in neonates [3].Mahadevaiah et al. [4] reviewed the literature in 2011 andfound only nine cases of CSDC reported in neonates.The characteristic features of CSDC include abrupttransition to the normal proximal and distal colon [5],hypomotility of the dilated segment, the absence of teniacoli, and abundant serosal vascularization fed by a large,marginal colonic artery [6].We report a case of CSDC, presented in the neonatalperiod with a clinical picture of intestinal obstructionsimilar to that of Hirschsprung’s disease, diagnosedpreoperatively by contrast enema, and treated surgicallyby resection of the dilated segment and the Soave pull-through procedure. We discuss the clinical features, theradiological picture, the histopathological findings, andthe surgical modalities for this pathology.Case reportA full-term male baby, delivered by spontaneous vaginaldelivery, with a birth weight of 3 kg presented on the firstday of life with complaints of decreased oral intake,lethargy followed by abdominal distension. There wasno history of vomiting and the baby passed meconiumspontaneously.On examination, he was afebrile but lethargic and mildlydehydrated. The abdomen was moderately distended andmildly tense, but there was no tenderness on palpation.An abdomen radiograph revealed a hugely dilated colonicshadow. A rectal tube was passed, and a moderate amountof well-formed stool was drained along with a large gushof air, and immediately the abdominal distensiondecreased significantly.The patient was admitted in neonatal ICU for furthermanagement. During the hospital course, the patient wasmanaged with frequent rectal washes along with suppor-tive medical management. Meanwhile, the patient alsokept on passing spontaneous stool. Contrast enema wasperformed, and it revealed a hugely dilated recto-sigmoidcolon with abrupt normal-caliber colon proximal to it andwithout any transition zone (Fig. 1). The proximal endof the dilated segment was at the lower part of thedescending colon, and the distal end of the dilatedsegment was at the level of the third sacral vertebra. Thisradiological finding was suggestive of segmental dilatationof the recto-sigmoid colon. Hence, surgery was planned,and we started by laparoscopy, which helped visualize thesegmental dilation of the colon along with its prominentvasculature (Fig. 2). Laparotomy was performed, and themacroscopic appearance of the dilated colonic segmentshowed abundant serosal vascularization with a largemarginal colonic artery, and the tenia coli was absent(Fig. 3).Resection of the dilated segment was performed, andthen Soave pull-through of the normal-caliber colon wasperformed and anastomosed to the anus (Fig. 4). Theresected segment measured 20 cm in length and 15 cm inwidth.The postoperative course was smooth; feeding wasresumed on the fourth postoperative day, and the babywas discharged on the eighth postoperative day in stablecondition.Histological examination demonstrated the presence ofganglion cells on both the submucosal layer and themuscularis propria of the dilated resected segment.Nerve fibers were hyperplasic and hypertrophic. In themuscularis propria layer, there was significant disarrange-ment, disorganization, and focal multiple atrophy ofmuscle fibers.The presence of significant dilatation of blood vessels wasalso noted in the subserosal layer. The ganglion cells werepresent in the distal rectum.46 Case report1687-4137 c 2015 Annals of Pediatric Surgery DOI: 10.1097/01.XPS.0000452059.63870.bcCopyright © Annals of Pediatric Surgery. Unauthorized reproduction of this article is prohibited.Fig. 1Contrast enema reveals a hugely dilated recto-sigmoid colon with abrupt normal-caliber colon proximal to it, without a transition zone.Fig. 2The laparoscopy view demonstrates the segmental dilation of the colon with prominent vasculature.Fig. 3The perioperative view shows a large marginal colonic artery, theprominent vasculature, and the normal colonic caliber proximal to thesegmental dilatation.Fig. 4Endorectal Soave pull-through.Segmental dilatation of the colon Al-Zaiem et al. 47Copyright © Annals of Pediatric Surgery. Unauthorized reproduction of this article is prohibited.DiscussionSwenson and Rathauser in 1959 [1] were the first todescribe three cases of segmental dilatation of the colon.Since then, there have been several case reports of CSDCin the literature mostly described in children beyond theneonatal period [2,7].Mahadevaiah et al. [4] reviewed the literature in 2011,and found nine cases of CSDC reported in neonates.Most of these neonatal cases were diagnosed peropera-tively [8], whereas in our case, the diagnosis was madepreoperatively due to the classical picture of the contrastenema, demonstrating an abrupt transition between thedilated segment and the normal colon proximally anddistally; this radiological picture differentiates CSDCfrom Hirschsprung’s disease.It has been presumed that segmental dilatation of thecolon is a congenital malformation [9,10]; the earlypresentation in the first day of life in our case supportsthis assumption.The etiology of CSDC is still unknown. Several theorieshave been proposed to explain its pathogenesis. Mathéet al. [11] proposed a primitive neuromuscular dysfunction ofthe bowel, but this presumed theory does not explain theoccurrence of the disease in a selective segment of the colon.In our case, two interesting findings were present in thehistopathological examination: the first one is the presenceof abundant, dilated, and tortuous blood vessels noted in themesentry and the subserosal and the seromuscular layersof the dilated segment, which may play a role in thepathogenesis of the disease; this feature was also noted byAl-Salem [12]. The second interesting histopathologicalfinding in our case was the presence of significantdisarrangement, disorganization of the muscle fibers in themuscularis propria layer, associated with focal multipleatrophy. The absence of muscle hypertrophy in our casecomplies with the hypothesis raised by Helikson et al. [9],who supposed that the muscle hypertrophy observed in thecases discovered in older infants is acquired.The surgical treatment of CSDC consists of the resectionof the dilated segment and re-establishment of theintestinal continuity by end-to-end anastomosis [8].Al-Salem [12] performed a staged Duhamel’s pull-through after thorough bowel preparation. In our case,the distal end of the dilated segment was just at the levelof the pelvic peritoneal reflexion. Hence, we performedthe Soave pull-through technique, which is proved to beeasy in the neonatal period, requiring minimal pelvicdissection with minimal danger of injuring the surround-ing pelvic structure and achieving a safe extra-abdominalrecto-anal anastomosis. An alternative technique is theresection of the dilated segment with an end-to-endanastomosis. This, however, requires a pelvic dissectionwith the intra-abdominal anastomosis.ConclusionCSDC, although a rare condition, should be consideredin the differential diagnosis of neonatal low-intestinalobstruction. Barium enema is diagnostic, and differenti-ates CSDC from Hirschsprung’s disease and other causesof intestinal obstruction in the neonatal period.AcknowledgementsConflicts of interestThere are no conflicts of interest.References1 Swenson O, Rathauser F. Segmental dilatation of the colon: a new entity.Am J Surg 1959; 97:734–738.2 Al-Salem AH, Grant C. Segmental dilatation of the colon. Reportof a case and review of the literature. Dis Colon Rectum 1990;33:515–518.3 Yadav K, Singh G, Budhiraja S. Congenital segmental dilatation of intestine.Indian J Pediatr 1996; 63:561–563.4 Mahadevaiah SA, Panjwani P, Kini U, Mohanty S, Das K. Segmentaldilatation of sigmoid colon in a neonate: atypical presentation and histology.J Pediatr Surg 2011; 46:e1–e4.5 Brawnier J, Schafer D. Segmental dilatation of the colon. J Pediatr Surg1973; 8:957–958.6 Takehara H, Komi N, Hino M. Congenital segmental dilatation of the colon:report of a case and review of the literature. Pediatr Surg Int 1988; 4:66–68.7 De Lorimer AA, Benzian SR, Gooding CA. Segmental dilation of the colon.Am J Roentgenol 1971; 112:100–104.8 Sarin YK, Singh VP. Congenital segmental dilatation of colon. Indian Pediatr1995; 32:116–118.9 Helikson MA, Schapiro MB, Garfinkel DJ, Shermeta DW. Congenitalsegmental dilatation of the colon. J Pediatr Surg 1982; 17:201–202.10 Chiba T, Kokubo T. Congenital segmental dilatation of the colon. NihonGeka Hokan 1976; 45:45–47.11 Mathé JC, Khairallah S, Phat Vuoung NP, Boccon-Gibod L, Rey A, Costil J.Dilatation segmentaire du grêle à révélation néonatale. Etude parimprégnation argentique des plexus myentériques [Segmental dilatation ofthe ileum in a neonate. Study of the myenteric plexus with a silver stainingpreparation]. Nouv Presse Med 1982; 11:265–266.12 Al-Salem AH. Congenital segmental dilatation of the rectosigmoid colon:a forgotten cause of constipation. J Pediatr Surg Spec 2008;2:20–22.48 Annals of Pediatric Surgery 2015, Vol 11 No 1Copyright © Annals of Pediatric Surgery. Unauthorized reproduction of this article is prohibited.

Congenital segmental dilatation of the colon

https://www.ajol.info/index.php/aps/article/download/168912/158379

Congenital segmental dilatation of the colon

Abstract

Similar works

Full text

Available Versions

AJOL - African Journals Online