Intussusception as a rare clinical presentation of a child with type 1 diabetes and diabetic ketoacidosis

Abstract

Intussusception in children is mainly idiopathic or due to a viral aetiology. Occasionally, pathological lead points like Meckel diverticulum or rarely metabolic causes like hyperglycaemia can result in formation of an intussusception. We describe the case of a boy with diabetic ketoacidosis presenting with abdominal pain. Despite correction of acidosis, the gastrointestinal symptoms persisted for which an ultrasonography of the abdomen was performed. It revealed an ileo-ileal intussusception, which was confirmed by a CT scan. He underwent an exploratory laparotomy. After reduction of the intussusception, a Meckel diverticulum was found as the lead point that was then resected. We present a case of a child with type 1 diabetes having an intussusception due to Meckel diverticulum, which was probably made worse by the decreased intestinal motility due to hyperglycaemia. Intussusception should thus be suspected in patients with diabetic ketoacidosis if the pain is persistent despite correction of acidosis.

Background

Intussusception is defined as an invagination or prolapse of a part of the intestine into the adjoining segment leading to bowel obstruction. The small bowel is involved in majority of the paediatric cases and rarely large bowel involvement can also be seen. It is commonly seen in children aged6–18 months of age and is mainly idiopathic (90% of the cases, no aetiology can be identified) or secondary to some viral infection.¹ However, in older children and adults, intussusceptions are more likely to have a pathological lead point such as Meckel diverticulum, benign polyp or intestinal tumours like lymphomas. Rarely, decreased intestinal motility due to metabolic derangements like hyperglycaemia, metabolic acidosis and hypokalaemia has also been implicated in the pathogenesis of an intussusception.²

In children with newly diagnosed type 1 diabetes mellitus, the very first clinical presentation could be in the form of diabetic ketoacidosis (DKA); DKA is reported in 15%–70% of children at onset of diabetes.³ Abdominal pain is a frequent manifestation of DKA and sometimes the pain may be very severe like an acute abdomen. The abdominal pain in diabetic children during ketoacidosis is due to ischaemia caused by metabolic acidosis. This presentation of an acute abdomen can sometimes lead to a wrong diagnosis such as urinary tract infection, appendicitis or intussusception until diagnosis of diabetes and ketosis becomes evident.⁴ We report a case of persistent abdominal pain in a 10-year-old boy with newly diagnosed type 1 diabetes and DKA, which was later on diagnosed to be due to an ileo-ileal intussusception.

Case presentation

Our patient was a 10-year-old boy; only child born to non-consanguineous parentage. The child was previously well and did not require any hospitalisations prior to this admission. There was a family history of autoimmune conditions with father and paternal grandmother having autoimmune hypothyroidism, for which both were on thyroxine supplementation. There was also a history of type 2 diabetes mellitus in paternal grandfather controlled on oral hypoglycaemic agents.

Our patient presented to us with the reports of repeated episodes of non-bilious vomiting and pain in abdomen since 1 day. In addition, parents also reported increased frequency of urination, increased intake of oral fluids, secondary nocturnal enuresis and weight loss since 2 months. The weight loss documented was around 6 kg. On examination, he was conscious, lethargic but responding to oral commands. He was afebrile, pulses were well felt but there was tachycardia with a pulse rate of 122 bpm and blood pressure readings were within the reference range. The child also had acidotic breathing with a respiratory rate of 34/min. In addition, signs of dehydration like sunken eyes, decreased skin turgor and dry oral mucosa were present. Head and neck examination were normal. He had normal heart sounds and there was no gallop and breath sounds were also normal. Abdominal examination revealed a soft abdomen, which was tender to touch in all the quadrants, but no guarding or rigidity were present and normal bowel sounds were heard on auscultation.

Investigations revealed that blood sugar concentration was 580 mg/dL (32.2 mmol/L) and there was the presence of four plus or large ketones on urinary dipstick assessment. Blood gas analysis revealed severe metabolic acidosis (pH—6.92 and bicarbonate—5.6 mEq/L or mmol/L). Other investigations including complete blood counts, renal, liver and thyroid function tests were normal. His glycosylated haemoglobin A1C was high, 15.6% or 147 mmol/mol. With the above clinical picture and laboratory investigations, a diagnosis of diabetes mellitus and severe DKA was made and the patient was started on intravenous isotonic 0.9% normal saline infusion and regular insulin infusion (1 unit/kg/hour) according to the International Society for Paediatric and Adolescent Diabetes 2018 protocol.³ Within 48 hours of intravenous insulin and fluid therapy, acidosis and dehydration were resolved. The blood sugar readings were maintained between 100 and 150 mg/dL (5.5–8.3 mmol/L) with 5% dextrose in 0.9% normal saline and intravenous insulin infusion at 0.05 units/kg/hour. As acidosis had resolved, C-peptide and GAD-65 antibody tests were requested to determine the type of diabetes. The C-peptide levels were low, 0.10 ng/mL (33.1 pmol/L) and GAD 65 antibody test was positive, 53.96 IU/mL confirming the diagnosis of type 1 diabetes.

Despite the correction of acidosis and fluid depletion, his abdominal pain persisted. For this, he underwent an ultrasonography of the abdomen, which revealed the presence of a probable ileo-ileal intussusception, which was confirmed by a CT scan (figure 1). The CT scan also showed proximal small bowel distension due to the presence of ileo-ileal intussusception and presence of minimal ascites. He underwent an emergency exploratory laparotomy. After reduction of the intussusception, during the procedure, a Meckel diverticulum was found approximately 35 cms proximal to the ileocaecal junction. This Meckel diverticulum served as the lead point of the intussusception. The distal ileum showed pregangrenous changes and the intraperitoneal fluid was serous in nature. The ileum with Meckel diverticulum was resected and sent for histopathological analysis, following which an end to end ileal reanastamosis was performed in two layers with absorbable sutures. The vascularity of the intestine prior to reanastomosis was assessed clinically by the colour, pulsations and peristaltic activity. The intraperitoneal fluid sent for culture analysis did not show any microbial growth. The histopathology report of the surgical specimen sent revealed a Meckel diverticulum measuring 5 cms in length, which showed a perforation 1 cm from the junction. On the cut surface of the Meckel diverticulum, polypoidal growth was noted 3 cm away from proximal end. Section from Meckel diverticulum showed dense mixed inflammatory infiltrate composed of lymphoplasmacytes and neutrophils, extending up to serosa with severe oedema and congestion consistent with Meckel diverticulitis.

Figure 1.

CT scan abdomen of a 10-year-old child with type 1 diabetes melitus. The scan shows ileo-ileal intussusception (indicated by an arrow) and proximal small bowel obstruction.

There were no complications and postoperative period was uneventful. As the child was kept nil by mouth for few days post the surgical procedure, sugars were initially maintained by titration of intravenous fluids and intravenous regular insulin infusion. He was shifted to subcutaneous basal bolus insulin therapy (rapid-acting insulin analogue aspart and long acting insulin analogue glargine) when oral fluids and feeds were started and tolerated.

Outcome and follow-up

On being shifted to the basal bolus insulin therapy (1 unit/kg/day), the sugars were well maintained. The child and the family were educated about the management of type 1 diabetes (blood sugar monitoring, insulin injection techniques, diet, exercise, management of complications including hypoglycaemia and hyperglycaemia and importance of regular follow-up and testing) by the diabetes team. As the child was stable, abdominal pain had subsided and there were no other complications, he was discharged on seventh day post the surgery. He followed up with us after 15 days during which he had gained 4kg weight and was active and cheerful and sugar readings were maintained between 90 and 180 mg/dL or 5 and 10 mmol/L.

Discussion

Intussusception is a medical emergency, if it is not detected and treated early, it can lead to serious life-threatening complications like necrosis of the bowel, perforation, peritonitis, sepsis and eventually can also lead to mortality. The classical clinical triad of intussusception (colicky abdominal pain, ‘currant jelly’ stools and bilious vomiting) is seen only in one-third of paediatric patients. Hence, an imaging (ultrasonography or CT scan) is necessary to diagnose intussusception in cases where the triad is not present and to confirm in cases where there is a strong clinical suspicion.⁵

Abdominal pain, a frequent manifestation of DKA, is seen in 40%–75% of the patients. The cause of abdominal pain in these patients could be due to a variety of reasons such as mesenteric ischaemia due to severe metabolic acidosis, which is precipitated by volume depletion or secondary to impaired gastrointestinal motility (oesophageal, gastric, intestinal and gallbladder).⁴ In most patients, correction of metabolic acidosis and fluid deficit leads to a resolution of the abdominal symptoms. However, if abdominal pain persists in these patients despite the corrective measures, organic causes such as pyelonephritis, pancreatitis, hepatitis, peritonitis or other causes of acute abdomen should be kept in mind.⁴ Similarly, in our patient, despite correction of acidosis and dehydration, the abdominal pain persisted and hence he was evaluated for organic causes and was diagnosed to have an intussusception.

There are only a handful of case reports in literature describing intussusception in patients with type 2 diabetes and DKA; all of them are in adults.^{2 6 7} The main reason postulated in all these case reports is the role of acute or longstanding hyperglycaemia on the function and motility on the gastrointestinal tract. Hyperglycaemia can result in decreased motility in any part of the gastrointestinal tract. The most common motility disorder reported in type 1 diabetes is delayed gastric emptying or gastroparesis, which is prevalent in 40% of patients with long-standing diabetes.⁸ Similarly, small intestinal motility may also be affected in type 1 diabetes either due to poor glycaemic control or due to acute hyperglycaemia as demonstrated in the study by Byrne et al.⁹ The study showed that acute hyperglycaemia leads to delayed transit time in the jejunum as well decreased magnitude of jejunal contractions on intestinal manometry. Thus, reduced small bowel motility essentially acts as a safeguard in hyperglycaemic states to prevent absorption of glucose in the intestine, thereby preventing the worsening of the hyperglycaemia.² In our patient, in addition to hyperglycaemia altering the intestinal motility, the child also had a lead point in the form of a Meckel diverticulum leading to the development of intussusception. Meckel diverticulum is present in 2% of the population but only 4% will have complications in the form of bowel obstruction, diverticulitis or intussusception. Since most Meckel diverticuli are asymptomatic, diagnosis of intussusception due to Meckel diverticulum as a lead point is rare in all populations.¹⁰

To the best of our knowledge, no case report has been documented in children with type 1 diabetes mellitus having intussusception as the initial clinical presentation along with DKA. The intussusception in our child was probably due to the Meckel diverticulum acting as a lead point, which was made worse by the impaired small bowel motility due to the hyperglycaemia and metabolic acidosis. Due to the similarity of gastrointestinal symptoms in both DKA and intussusception, treating clinicians need to have a high index of suspicion, especially when faced with disproportionate or persistent abdominal pain despite correction of the acidosis in the paediatric population.

Learning points.

• Despite correction of acidosis and dehydration in a child with diabetic ketoacidosis, if the abdominal pain is disproportionate or unremitting, other causes of acute abdomen like intussusception should be kept in mind.

• Acute or chronic hyperglycaemia is known to cause decreased intestinal motility, which could be responsible for the development of an intussusception in children with type 1 diabetes.

• In children with diabetes having intussusception, in addition to metabolic abnormalities like hyperglycaemia, hyperkalaemia and acidosis, organic lesions like Meckel diverticulum (which act as a lead point) should also be explored.