ABDOMINAL CONDITIONS © 2010 SNL All rights reserved

A case series on intussusceptions in infants presenting with listlessness

Intussusception is characterised by , and in stools. However, in younger infants it may present with non-classical symptoms such as listlessness, pallor, decreased feeding, and being non-specifically unwell. Three cases of intussusception in young infants who presented with being listless and had some or no features to suggest a clinical diagnosis of intussusception are described which are designed to highlight the non-classical features of intussusception likely to be encountered in very young infants.

Siba P. Paul1 ntussusception is one of the most tachycardia and prolonged capillary refill MBBS, DCH Icommon surgical emergencies time he was given a fluid bolus of 20mL/kg Paediatric Trust Registrar encountered in infancy and early with 0.9% sodium chloride. He was [email protected] childhood. This is a condition where the intermittently responding to his parents proximal segment of the bowel telescopes but was not irritable. His cardiovascular 1 David C. A. Candy into the distal segment causing and respiratory examination was MBBS, MSc, MD, FRCP, FRCPCH, FCU obstruction1. The classic triad of symptoms otherwise normal. Consultant Paediatric Gastroenterologist consists of abdominal pain, vomiting and He was distressed by an abdominal blood in stools1. It is often seen in children Nikila Pandya2 examination and was found to be drawing aged four months to two years, with a peak up his legs and crying while being MD, DCH, FRCPCH incidence during four to nine months of Consultant Paediatrician examined. The provisional diagnosis at age2. However, infants may present with that point was either , Department of Paediatrics non-classical symptoms such as listlessness, or a suspected intussusception. A chest and 1 St Richard’s Hospital, Chichester decreased feeding, non-specifically abdominal film (FIGURE 1) was organised 2 Maidstone General Hospital, Maidstone appearing unwell, looking pale, etc. We which showed clear lung fields. The Keywords present three cases of infants with abdominal X-ray looked very abnormal intussusception, who presented with being with paucity of gas. Subsequently he intussusception; classic triad; hypotonia; listless and pale, with a view to heightening passed fresh mucousy blood per listless infant; ultrasonography; early the awareness of this condition. which resembled a ‘redcurrant jelly’ stool, weaning hence a clinical diagnosis of Key points Case 1 intussusception was made. He needed a A healthy six-month-old male infant, Paul S.P., Candy D.C.A., Pandya N. A case series on intussusceptions in infants presented with intermittently looking very presenting with listlessness. Infant 2010; pale and being poorly responsive over an 6(5): 174-77. eight-hour period. He had non-bilious 1. Intussusception is the most common vomiting on six different occasions and his in infants and mother reported blood spots in the nappy young children. earlier in the day. He was reported to be 2. The classic triad of symptoms consists fully immunised and had been weaned of abdominal pain, vomiting and early on to solid food at around four redcurrant jelly stool. months of age. 3. Symptoms such as lethargy, irritability, The initial assessment revealed a very listlessness may present early in the pale looking infant with a heart rate of illness with no abdominal signs. 158/min, temperature of 36.1°C, 4. Ultrasonography or contrast enemas are the preferred investigations to respiratory rate of 36/min, oxygen diagnose intussusception. saturation of 99% in air, blood glucose of 5. If reduction of intussusception is 6.7 mmol/L and a central capillary refill unsuccessful surgical intervention may time of three seconds. He had a patent be necessary. airway and was breathing comfortably. He 6. Chronic intussusception can present was given two litres per minutes of face FIGURE 1 Chest and abdominal X-ray shows with non-specific symptoms and failure mask oxygen due to his initial presentation evidence of abnormal gas pattern with to thrive. and diagnostic uncertainty. In view of the paucity of gas. Lung field appears clear.

174 VOLUME 6 ISSUE 5 2010 infant ABDOMINAL CONDITIONS further fluid bolus of 20mL/kg of 0.9% sodium chloride and was started on intravenous cefotaxime. The blood invest- igation results showed a C-reactive protein (CRP) of 9mg/L, white cell count of 14.5 x 109/L, platelets of 395 x 109/L and haemoglobin of 11.7 gm/dL. He was referred to the regional paediatric surgical unit where a diagnosis of intussusception was confirmed. He had a failed trial of reduction of the intussusception by hydrostatic pressure and needed an open surgical reduction. He made an uneventful recovery and was reported to be well at further follow-up.

Case 2 FIGURE 2 Abdominal X-ray shows evidence of FIGURE 3 Abdominal X-ray shows evidence of A healthy four-month-old female infant, abnormal gas pattern with a right-sided soft abnormal gas pattern with paucity of gas. presented with intermittently looking very tissue mass suggestive of intussusception. pale and listless for about six hours. She was reported to be not feeding well. She had no history of vomiting or diarrhoea, uneventful recovery. She was reported to no cough or cold, and did not have a . be well at further follow-up. She had had the first set of primary immunisations and had been weaned early Case 3 1 1 on to solid food at around 3 /2 months of A healthy 4 /2-month-old male infant age. The initial assessment revealed a very presented with intermittent shrill crying pale looking infant with a heart rate of episodes for about eight hours. He had had 148/min, temperature of 36.9°C, at five weeks of age and respiratory rate of 48/min, oxygen had laparoscopic pyloromyotomy, but saturation of 94% in air, blood glucose of represented a week later with vomiting 5.8 mmol/L and a central capillary refill which resolved with open pyloromyotomy. time of <2 seconds. She had a patent He had had non-bilious vomiting on two airway and was breathing comfortably in occasions during his present illness and a air. She was responding appropriately to blood streaked offensive stool earlier in the her parents and was not irritable. The evening. cardiovascular and respiratory The initial assessment by the casualty examination was otherwise normal. She officer revealed a pale looking infant, disliked an and listless with a heart rate of 158/min, was found to be drawing up her legs and temperature of 36.1°C, respiratory rate of crying while being examined. The 36/min, oxygen saturation of 99% in air, provisional diagnosis at this point was one blood glucose of 6.7 mmol/L and a central of sepsis or a suspected intussusception. capillary refill time of two seconds. There An abdominal film (FIGURE 2) was was no increased work of breathing. organised which showed multiple loops The review by the paediatric team of small bowel, with a soft tissue revealed an irritable but listless child with impression in the right side which was normal systemic examination. He disliked suspicious for an intussusception mass. an abdominal examination and had FIGURES 4 and 5 scan of the She remained stable and was given a tenderness on deep palpation. The shows classical ‘target’ sign of fluid bolus of 20mL/kg of 0.9% sodium provisional diagnosis at that point was intussusception. chloride. The blood investigation results gastroenteritis or a suspected intussuscep- showed a CRP of <1 mg/L, white cell count tion. An abdominal X-ray (FIGURE 3) was confirmed the diagnosis of intussusception of 12.2 x 109/L, platelets of 765 x 109/L and abnormal with paucity of gas. (FIGURES 4 and 5). haemoglobin of 10.9 gm/dL. Overnight he passed blood and faeces on He was referred to the regional She was referred to the regional one more occasion. He was kept nil by paediatric surgical unit where he had a paediatric surgical unit where a diagnosis mouth and given intravenous maintenance failed trial of reduction of the of intussusception was confirmed on fluids. His CRP was 12mg/L, white cell intussusception by air enema and needed abdominal ultrasound scan. She had a count 8.4 x 109/L, platelets 383 x 109/L and an open surgical reduction. He made an successful reduction of the intussusception haemoglobin 11.2 gm/dL. He had an uneventful recovery and was reported to be by hyrdrostatic pressure and made an abdominal ultrasound scan which well at further follow-up. infant VOLUME 6 ISSUE 5 2010 175 ABDOMINAL CONDITIONS

Discussion infants and these generally present early1,9. Patients often present with non-specific Historical background symptoms such as looking mottled or pale, Intussusception was first described by irritability, decreased appetite, listlessness 3 4 Barbette of Amsterdam in 1674 . Hunter or lethargy9. Clinicians may face a in 1789 discussed the surgical pathology of diagnostic dilemma in children presenting intussusception. The exact year of surgical solely with neurologic manifestations and reduction of intussusception remains may investigate for causes of sepsis or controversial – 1784 according to neurological conditions thus delaying the Hutchinson but 1672 according to diagnosis of intussusception9. In this group Ashhurst4. In 1876 Hirschsprung first FIGURE 6 Possible foci for intussusception. the may be entirely described the reduction of intussusception normal except for the infant being 5 by hydrostatic pressure methods . In July lethargic or listless. 1999, reports of intussusception associated Intussusception should be considered in with the rotavirus vaccine led to the differential diagnosis of young children suspension of the programme6. presenting with lethargy and/or hypotonia, even though the classical signs may be Epidemiology absent9. Intussusception is the commonest cause of FIGURE 7 Schematic diagram of Chronic intussusception10 presents with a acute intestinal obstruction in children intussusception. varying combination of symptoms 1,7 under two years of age with less than 25% Figures 6 and 7 were published in Essential , including abdominal pain, vomiting, of cases occurring in children aged more Problems, Diagnosis and Management, 4th Edition, weight loss/failure to thrive, diarrhoea, and than two years7. Intussusception has a male by Birkett et al, page 742, copyright Elsevier (2007). blood per rectum and is a treatable cause predominance with a male to female ratio of failure to thrive. It is an uncommon of 4:11. If left untreated intussusception has vasculitis, , Meckel’s diverticulum, condition and often diagnosed late. An been reported to be uniformly fatal within intestinal , , etc. abdominal mass may or may not be two to five days1. No clear racial Intussusception may also occur in palpable and the classic triad of symptoms predilection is reported, however children starvation (during religious fasts in certain is uncommon. with sickle cell disease may be more prone communities), dehydration secondary to to the condition. severe gastroenteritis, cystic fibrosis and in Diagnosis sickle cell crisis1,7. Interestingly a lead point Laboratory investigations are not helpful in Aetiology and associated conditions is often demonstrated in older children diagnosing intussusception. However, these The aetiology of intussusception remains presenting with intussusception1,7. may be helpful in differentiating the unclear and is idiopathic in most cases. ingestion in young condition from other causes like sepsis, There are two main theories forwarded viz children may lead to intussusception and metabolic disorders, etc. Also profuse dietary and infective. The dietary theory specific caution needs to be taken in the vomiting may result in arises from the observation that event of multiple magnet ingestion. imbalance which can be detected in intussusception largely occurs around the laboratory tests. time of weaning and early weaning has Clinical presentation Radiological investigations are the been considered to be a risk factor8. The The classic triad of intussusception mainstay for diagnosing intussusceptions. infective theory is probably explained by a consisting of abdominal pain, vomiting Plain radiographs are often the fastest history of mild respiratory or gastro- and bloody stools, is seen in less than one- investigation available and may show the intestinal tract infection preceding the third of patients1,7,9. Interestingly 75% of typical target sign (in right upper onset of intussusception, which may cause children have two symptoms while another quadrant, appears as a faint circle within a of abdominal lymph nodes 13% will have just one symptom. circle, similar to a doughnut), absence of and that of the Peyer’s patch8 (FIGURE 6). Abdominal pain typically precedes the the edge, crescent sign and bowel Many authors believe that a lead point in vomiting, occurs in paroxysms every 15 to obstruction. A normal abdominal X-ray the intestine allows a bowel segment 30 minutes and can last up to 15 minutes does not exclude intussusception. (intussusceptum) with its mesentry to during which time the child becomes Contrast or air enemas have traditionally telescope into the adjacent distal segment inconsolable and draws up the legs. been the diagnostic test and often can be (intussuscipiens) causing the obstruction1,9 Abdominal examination may be normal therapeutic in reduction of the (FIGURE 7). When these segments become early in the disease course. Later the intussusception (FIGURE 8). strangulated, mucosal bleeding can occur abdomen may become distended and Ultrasonography is fast becoming the thus producing the classic ‘redcurrant tender, and palpation may reveal a sausage investigation of choice and is often used as jelly’ stool. shaped mass in the right upper quadrant it is non-invasive with no radiation However a specific lead point is not while the lower quadrant feels empty involved and can also detect other found in 90% of cases and thus the dietary (Dance sign)1,7. pathologies. In a study of 141 children11 in and infection theories may play a role. Neurologic manifestations are Australia, 95 (67%) cases were diagnosed Cases where a lead point may be identified1 becoming a recognised entity in with ultrasonography and 31 cases (22%) can be seen in Henoch-Schönlein Purpura intussusception especially in very young were diagnosed with air or barium enema.

176 VOLUME 6 ISSUE 5 2010 infant ABDOMINAL CONDITIONS

Management professionals and the current national A child presenting with intussusception guidelines of weaning on to solid foods needs assessment and appropriate around six months of age may help to resuscitation as the initial presentation may decrease the occurrences of be unclear and may mimic other serious intussusception. An early diagnosis is pathologies. Some children presenting with associated with a better outcome. will need fluid resuscitation and in References non-specific sepsis-like presentations or cases where peritonitis or perforation is 1. Nelson K.A., Hostetler M.A. A listless infant with vomiting. Hospital Physician 2002; 40-46. suspected, therapy should be 2. Kong F.T., Liu W.Y., Tang Y.M. et al. Intussusception started. A nasogastric tube insertion may in infants younger than 3 months: a single center’s FIGURE 8 Barium enema showing experience. World J Pediatr 2010; 6(1): 55-59. be helpful. Intussusception. Non-surgical management includes 3. Madanur M.A., Mula V.R., Patel D. et al. reduction of intussusception with air Periampullary carcinoma presenting as duodenojejunal intussusception: a diagnostic and enema or with contrast enema5. Any type therapeutic dilemma. Hepatobiliary Pancreat Dis Int of hydrostatic reduction resolves the 2008; 7: 658-60. intussusception by exerting pressure on the 4. Lewis J.H. Jejunal intussusception of the newborn. apex of the intussusceptum to restore it to Am J Dis Child 1939; 58(3): 558-63. its original position12. Different studies 5. Bisset III G.S., Kirks D.R. Intussusception in infants highlight success rates between 50-95%. and children: diagnosis and therapy. Radiology 1988; 168: 141-45. Air enema can have success rates up to 6. Chang H.G.H., Smith P.F., Ackelsberg J. et al. 90% however 10% of patients go on to Intussusception, rotavirus and rotavirus develop intussusception again later. The vaccine use among children in New York State. success rates decreases with repeated FIGURE 9 Intussusception at surgery. Pediatrics 2001; 108(1): 54-60. Turner D., Rickwood A.M.K., Brereton R.J. attempts at using air enemas and surgical Figures 8 and 9 reprinted with permission from 7. Intussusception in older children. Arch Dis Child intervention may be necessary. eMedicine.com, 2010. Available at: 1980; 55: 544-46. Surgical management involves explora- http://emedicine.medscape.com/article/ 930708-overview. 8. Knox E.G., Court S.D.M., Gardner P.S. Aetiology of tory where the intussusception intussusception in children. BMJ 1962; 2(5306): is manually reduced (FIGURE 9). However, its reduction and also associated shock and 692-97. delayed presentations can cause bowel sepsis. There is a subsequent risk of small 9. Kleizen K.J., Hunck A., Draaisma J.M.Th. Neurologic ischaemia and necrosis necessitating a caused by adhesions in symptoms in children with intussusception. Acta up to 7% of cases. Paediatrica 2009; 98: 1822-24. resection of the bowel segment. Recurrence 10. Malakounides G., Thomas L., Lakhoo K. Just another of obstruction after a surgical reduction is case of diarrhea and vomiting? Pediatric Emergency uncommon. Conclusion Care 2009; 25(6): 407-10. Intussusception can present a diagnostic 11. Blanch A.J.M., Perel S., Acworth J.P. Paediatric Complications dilemma especially in younger children intussusception: Epidemiology and outcome. Emerg Med Austr 2007; 19: 45-50. With treatment the mortality rate may vary presenting with non-classical symptoms. 12. del-Pozo G., Albillos J.C., Tejedor D. Intussusception between 0 to 3% depending on the Early weaning practices need to be in children: current concepts in diagnosis and duration of onset of intussusception and addressed with caution by the healthcare enema reduction. Radiographics 1999; 19: 299-319.

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