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Colonic Perforation in a Nursing Infant as the Initial Presentation of Hirschsprung’s Disease. Is There a Relation with Prokinetics Use?

Nestor Martínez Salcedo¹, Paulino Martinez Hernandez-Magro¹,Claudia Robledo Monterrubio²

¹Department of Pediatric Surgery, Santa Fe Hospital, Celaya Guanajuato, México

²Department of Pediatrics, Santa Fe Hospital, Celaya Guanajuato, México

 

Correspondence:

Paulino Martínez Hernández Magro

Guadalupe No 205, Consultorio 202

Colonia Centro

CP. 38000 Celaya Guanajuato, México

Phone: +52 4616162123

E-mail: This email address is being protected from spambots. You need JavaScript enabled to view it.

 

Abstract

Perforation is a rare initial presentation in Hirschsprung’s disease. Early diagnosis and treatment are a challenge for the surgeon. A possible relation with the use of prokinetics should be studied. We present the case of a female infant, 50 days old, with constipation from birth treated with cisapride and trimebutine that subsequently developed irritability, bloating and vomiting. Plain abdominal X-ray revealed intra-abdominal free air and dilated bowel loops. She underwent exploratory laparotomy finding generalized purulent peritonitis, with small abscess in pelvic cavity and punctiform single sigmoid perforation. Partial colectomy and colostomy was performed. Prokinetics could contribute to the development of perforation in Hirshsprung’s disease, further work is required to examine whether the risk of perforation is increased in patients with Hirshsprung’s disease who are treated with prokinetics.

Keywords: bowel perforation, Hirschsprung’s disease, prokinetics

 

Background

Gastrointestinal perforation during the neonatal period is a challenge for pediatric surgeons, commonly attributed to many causes, mainly necrotizing enterocolitis [1].

Hirschsprung’s disease (HD) is caused by the absence of ganglion cells in distal bowel, extending proximally for varying distances, which can be a cause of intestinal obstruction, as well as perforation associated with obstruction in neonatal period [2]. Perforation not associated with necrotizing enterocolitis is a rare initial presentation in HD [2, 3].

Literature review indicates the rarity of bowel perforation in HD and the treatment difficulty but there are no reported cases that suggest an association with the use of prokinetics.

Case Presentation

We present the case of a female patient, nursing infant, delivered by cesarean section due to cephalopelvic disproportion with 3,300 kg birth weight, breast-fed and presented with constipation from birth. At 50 days old, she is evaluated by a physician who prescribed Trimebutine and Cisapride for constipation. Four days later the patient presents irritability, bloating and vomiting, without enteral signs. The patient has basal hypoventilation, a distended and tender abdomen, hypoactive bowel sounds, with signs of peritoneal irritation, digital rectal exploration shown straight narrow without fecal impaction, with sensation of entering into a tight finger glove.

Laboratory tests reported: hemoglobin 10 g/ml, leukocytes 5.150/mm3, neutrophils 60%, bands 4%, platelets 250.000/mm3, chlorine 102,56 mmol/L, sodium 139,5 mmol/L and potassium 3,59 mmol/L. Plain abdominal X-ray revealed intra-abdominal free air and dilated bowel loops (Fig. 1A and 1 B).

Figure 1A and 1B - Plain abdominal X-ray revealed intra-abdominal free air and dilated bowel loops

Fig 1a Fig 1b

Based on clinical, radiographic and laboratory findings we decided to perform an exploratory laparotomy finding generalized purulent peritonitis with abundant fibrinous exudates on bowel loops in both paracolic sliders, with small abscess in the pelvic cavity and punctiform single sigmoid perforation (Fig. 2A and 2 B).

Figure 2A and 2 B - Abundant fibrinous exudates on bowel loops with generalized purulent peritonitis (A) and punctiform single sigmoid perforation (B).

Fig 2a Fig 2b

A sigmoidectomy with terminal colostomy in barrel shotgun was performed. The postoperative period was uneventful. The patient started passing gas through colostomy at day 3, started being breast fed which was adequately tolerated, and was discharged at 5 days with good outcome. Histopathological study reported hypoganglionosis and colonic perforation with acute peritonitis (Fig. 3A and 3B).

Figure 3A and 3B – Histopathology exam showing hypoganglionosis in myenteric plexus.

Fig 3a Fig 3b

The patient was well during the 2-months follow-up and she is waiting for a definitive operation.

Discussions

Prenatal diagnosis of HD has not been possible. Clinical symptoms are variable and make very difficult to diagnose HD in the neonatal period and that is why an early diagnosis may not be always possible. In case of emergency surgery, seromuscular biopsies performed intraoperatively must be carried out to make the definitive diagnosis [4].

Perforation is a rare complication of HD and occurs in 3.2-4% of patients [1, 2]. Perforation is most common in the proximal colon (ileo-cecal area) in 68% of cases but can also occur in the appendix in 18% [2, 5] or terminal ileum [6, 7]. Other authors reported perforations in transversal colon. It can occur in both aganglionic and ganglionic portions [8].

The most common presenting symptoms are poor oral feeding, progressive abdominal distension with bilious vomiting, tachypnea, failure or delayed passage of meconium or bleeding per rectum [6, 7].

Delayed diagnosis of HD in newborn infants not only increases serious morbidities in the neonatal period, but also indirectly affects their long term outcome. Clinicians need to be vigilant in the recognition of HD in newborns and infants, and provide appropriate care accordingly, to prevent the detrimental complications [9]. Success rate is based on making an early diagnosis and collaboration with well-equipped neonatal intensive care units. In our country, we have failed to make an early diagnosis, most patients with constipation are treated symptomatically without knowing the cause, which causes incorrect and delayed treatment of HD [10].

Pathogenesis of perforation is still unknown and most studies have proposed that inflammation may play a major role in its development. As the disease progresses, the lumen of the intestine become filled with fibrinous exudates and this causes increased risk for perforation [2]. Other authors suggest that the lack of features of necrotizing enterocolitis in colonic perforations due to HD support the fact that perforation occurs due to increased intraluminal pressure from distal obstruction rather than inflammatory process [5, 8].

In our case, the patient was apparently in good condition until she started to be treated with prokinetics, what we believe could be a factor predisposing to perforation. One of the causes of perforation in patients with anorectal malformation is that the downstream obstruction leads to increased intraluminal pressure, and this, along with the muscular deficiency is probably responsible for more frequent rupture of the rectum [11]. In patients with diverticular disease of the colon is well known that conditions that predispose to an increased intraluminal pressure or reduced resistance of the diverticular mucosa can lead to perforation [12]. In this view, excessive colonic segmentation may increase intracolonic pressures and the stress forces acting on the diverticular mucosa [13]. The functional obstruction caused by HD and colonic segmentation by the action of prokinetics could increase intracolonic pressure, in fact prokinetics are contraindicated in cases of obstruction [14]. Colonic perforation associated to neostigmine administration has been documented [15, 16]. Further work is required to examine whether the risk of perforation is increased in patients with HD who are treated with prokinetics.

Treatment of the perforation depends on the clinical condition of the patient following fluid resuscitation and the initiation of broad spectrum antibiotics and generally requires a stoma construction at the point where ganglion cells are identified. Bowel continuity reconstruction with a definitive surgical treatment may include one of the techniques of transanal endorectal pull-through operation using open or laparoscopic approach [17, 18].

Conclusions

Intestinal perforation is a rare presenting form of Hirschsprung’s disease and it is seen especially in long segment disease. Early recognition of perforation contributes to successful management. Prokinetics could contribute to the development of perforation and they should be used with caution.

 

 

References

1. Almoutaz A. E, Mostafa H. Management and Outcome of Neonatal Bowel Perforation. Ann Pediatr Surg 2008; 4: 83-88.

2. Gözaydin N, Kaya M, Boleken M.E, Kanmaz T, Yücesan S. Intestinal perforation in Hirschsprung’s disease: A report of three cases. Gazi Med J 2005;16 (3): 136-138

3. Talishinskiy T, Tomita S. Intestinal perforation in preterm infant as an unusual initial presentation of Hirschsprung’s disease. J Ped Surg Case Rep 2013; 1: 381-382.

4. Polley T.Z.Jr, Coran A.G. Hirschsprung's disease in the newborn: An 11-year experience. Pediatr Surg Int 1986; 1: 80-83.

5. Newman B, Nussbaum A, Kirkpatrick J.A. Jr. Bowel Perforation in Hirschsprung’s Disease. AJR Am J Roentgenol 1987; 148:1195-1197.

6. Patel R.V, Lawther S, Mccallion W.A. Discordant monozygotic total colonic Hirschsprung’s disease presenting with neonatal isolated ileal perforation. BMJ Case Rep 2013;27 pii: bcr2013200743

7. Stringer M.D, Drake D.P. Hirschsprung’s disease presenting as neoanatal gastrointestinal perforation. Br J Surg 1991; 78: 188-189.

8. Singh S, Rawat J, Wakhlu A, Kureel S.N, Pandey A. Six-year retrospective analysis of colonic perforation in neonates and infants: Single centre experience. Afri J Paediatr Surg 2012; 9: 102-105.

9. Lee C.C, Lien R , Chian M.C ,Yang P.H, Chu S.M, Fu J.H, Lai J.Y. Clinical impacts of delayed diagnosis of Hirschsprung’s disease in newborn infants. Pediatr Neonatol 2012; 53: 133-137.

10. De la Torre Mondragón L, Torres Manjarrez T. Enfermedad de Hirschsprung. Estudio de 50 casos. Problemas de diagnóstico y tratamiento en México. Acta Pediatr Mex 1999; 20: 237-239

11. Tong WD, Ludwig KA. Neonatal colon perforation due to anorectal malformations: Can it be avoided? World J Gastroenterol 2013;19(25): 3915-3917.

12. Piekarek K, Israelsson L.A. Perforated colonic diverticular disease: the importance of NSAIDs, opioids, corticosteroids, and calcium channel blockers. Int J Colorectal Dis 2008; 23:1193–1197.

13. Gravante G, Yahia S. Medical influences surgical outcomes: Role of common medications on the risk of perforation from untreated diverticular disease. World J Gastroenterol 2013;19: 5947-5952.

14. Wu G.Y. the pharmacology of prokinetic agents and their role in the treatment of gastrointestinal disorders. IJGE 2003; 1:6-13.

15. Mc Donald CR, Tonkin D, Hewett P. Colonic perforation associated with neostigmine administration. J Surg Case Rep 2013;7: rjt040.

16. Mollema R, Spijkstra J.J,Polderman K.H, Gelissen HPMM, Girbes A.R.J. Perforation of the colon after administration of neostigmine. J Intens Care Med 2004; 30:730.

17. Thomson D, Allin B, Long A-M, Bradnock T, Walker G, Knight M. Laparoscopic assistance for primary transanal pull-through in Hirschsprung’s disease: a systematic review and meta-analysis. BMJ Open 2015; 5:e006063.

18. Hosseini SM, Gholamzadeh S, Zarenezhad M. New method of transanal pull through operation in patients with Hirschsprung’s disease. Afr J Paediatr Surg 2014; 11: 94-5.

Figures 1A and 1B - Plain abdominal X-ray revealed intra-abdominal free air and dilated bowel loops