Intussusception encephalopathy

Gayle T. Case 3: Sudden Altered Mental Status in an 11-month-old Boy. Pediatr Rev. 2020 Feb;41(2):88-89.

An 11-month-old boy presents to an emergency department (ED) after multiple witnessed episodes that were concerning for seizures. His parents note that he was in his normal state of health last night but this morning started becoming increasingly fussy. He then developed a blank stare and went limp. This happened 1 more time where he was seen to have perioral cyanosis. There were no convulsions, noted abnormal movements, or incontinence. He was quiet for a few minutes after this occurred but started to cry afterward. He was then taken to the community ED. He again developed a blank stare and went limp, which was witnessed by the care team on arrival in the ED. The family denies any fever or diarrhea, although he has had 2 episodes of nonbilious, nonbloody emesis after his staring episode. There was no witnessed ingestion or concern for nonaccidental or accidental trauma. He had been taking fluids well, with good urine output. There are no sick contacts at home, he does not attend child care, and there has been no recent travel. He is not taking any medication at home, has age-appropriate development, has not been hospitalized, and has received all of his vaccines.

Physical examination reveals a drowsy-looking pale infant with normal vital signs. The patient is circumcised. He was noted to be hypotonic during the episodes, but his neurologic examination findings are now normal. All other physical examination findings are normal.

Laboratory results are significant for a mild leukocytosis (white blood cell count, 15,200/μL [15.2×109/L]) and mild thrombocytosis with a platelet count of 473×103/μL (473×109/L). He had normal findings on a comprehensive metabolic panel, urinalysis, and urine toxicology screen looking for acetaminophen or other drugs he could have gotten into in the home. Blood and urine cultures were performed because there was concern that he could be in early sepsis, and the results are pending. Head computed tomography and chest radiography were normal. The diagnosis is determined after additional studies.

Discussion

The patient was transferred to a tertiary children’s hospital for evaluation by pediatric neurology. At the time of transfer the differential diagnosis included a new seizure disorder, encephalitis, unwitnessed ingestion, and head injury. On transfer, the receiving ED physician decided to order abdominal radiography and ultrasonography. The ED physician wanted to expand the differential diagnosis beyond neurologic complaints because the infant was now no longer having neurologic symptoms but was still acting fussier than usual, per mom. The combination of emesis, fussiness, and that the child was afebrile made the provider concerned for the diagnosis of intussusception. Ultrasonography showed ileocolonic intussusception extending to approximately the level of the proximal transverse colon. The boy then went directly to interventional radiology for reduction, which was successful. The patient was found to return back to baseline behavior and started being playful and interactive. A few hours later he surprisingly again became increasingly fussy, so was imaged again with ultrasonography. This was secondary to the concern that intussusception could have recurred, which happens in approximately 10% of all intussusception cases. His repeated ultrasonography showed that the intussusception was resolved. He was observed overnight and was able to go home the following morning, tolerating a regular diet…

Lessons for the Clinician

Patients usually present with intermittent, crampy abdominal pain, sometimes accompanied by bringing the knees to the chest.

The triad that is considered classic—intermittent colicky pain, currant jelly stool, and emesis—is a late finding of this diagnosis.

This is an important diagnosis to think about particularly in children and infants who have an unexplained change in their mental status, particularly those younger than 1 year.

Children younger than 1 year are also more likely to present with emesis and heme-positive or clearly bloody stools.

Importantly, infants can also present without any accompanying signs of abdominal pain and typically will be afebrile.

Goetting MG, Tiznado-Garcia E, Bakdash TF. Intussusception encephalopathy: an underrecognized cause of coma in children. Pediatr Neurol. 1990 Nov-Dec;6(6):419-21.

Abstract

Intestinal intussusception is a common cause of bowel obstruction in infancy and early childhood. Typically the presenting signs and symptoms are referable to the abdomen. On occasion the most prominent presenting feature is depressed level of consciousness. We describe 3 patients who presented with coma associated with intussusception.

Aygün F, Aydın PÖ, Emre Ş, Uzunoğlu SŞ, Saltık S, Çam H. A rare case with encephalopathy. Turk Pediatri Ars. 2016;51(3):169–172. Published 2016 Sep 1. doi:10.5152/TurkPediatriArs.2016.3460

The patient whom we presented in this article was referred to us, because his neurological picture deteriorated and the etiology could not be elucidated. At the time of admission, the airway, respiration and circulation were assessed rapidly and the vital signs were monitorized. No prominent pathology was described in the history except for previous upper respiratory tract infection. His hemodynamic status was stable and body temperature was normal. He was followed up in another center before being internalized in intensive care unit and the laboratory tests and imaging methods performed there revealed no pathology. When a palpable mass was found on physical examination, urgent abdominal ultrasonography was performed to elucidate the etiology and ileocolic invagination was detected. After sugery, the patient’s consciousness returned to normal completely and additional investigation or treatment was not required.