Death caused by appendicular knotting and small-bowel strangulation in a child: A case report and review

Introduction

Acute appendicitis and small-bowel obstruction is a relatively common emergency in paediatrics. Indeed, acute appendicitis can be linked to small-bowel adynamic ileus or adhesion. However, mechanical small-bowel strangulation secondary to appendicular knotting as a complication of acute appendicitis is an extremely rare event in adults and even more so in young infants.

Here, a fatal case of a very rare complication of a common surgical pathology is reported. After a review of the literature, only three references to small-bowel strangulation secondary to appendicular knotting in young infants were found. These cases are listed and explained. Some aspects regarding the pathological types of appendicitis producing small-bowel obstruction are reviewed. The statements of the cause and manner of the deaths are discussed. To the best of the authors’ knowledge, this is the first fatal case of appendicular knotting and small-bowel strangulation in a child reported in the literature.

Case report

The present case relates to a 23-month-old girl who entered the emergency department of a private medical centre with a 3-day history of colic-type abdominal pain associated with vomiting, hyperthermia and irritability. The patient was described as in a stable condition, with mucocutaneous pallor, abdominal tenderness and signs of dehydration. She was hospitalised, and support with intravenous fluids was initiated. A blood test showed a white blood-cell count of 12,500/mm³ and glycosuria. The patient’s condition deteriorated progressively, and she died less than 24 hours after admission. No diagnostic imaging or any surgical intervention was performed. The emergency doctors stated that death was secondary to gastroenteritis. The case turned judicial at the request of the parents who claimed lack of clarity in the diagnosis. An attorney requested a medico-legal autopsy to clarify the cause and manner of death.

A complete medico-legal autopsy was performed. External examination showed an anatomically normal infant without any evidence of trauma, with signs of medical intervention given by venepunctures in both upper limbs and signs of dehydration. The infant appeared eutrophic, adequately developed and well nourished. Internal examination documented severe distension of small intestinal loops and haemorrhagic peritoneal fluid of 100cc. The appendix measured 6 cm and looked inflamed; the tip adhered firmly to the wall of the ileum, forming a knot (Figures 1–2). There was a trapped jejunal-ileal segment that showed torsion and severe ischemic changes. Gangrene extended up to 70 cm from the ileocecal junction. Other findings included bilateral pleural effusion and shock lung. The vitreous confirmed hydro-electrolytic imbalance due to low levels of sodium and potassium.

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Figure 1.

An appendicular knot formed by adherence of the appendix to part of the terminal ileum. There was also torsion of the bowel.

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Figure 2.

The inflamed tip of the appendix was documented as being adherent to the ileum. Ischemic changes in last loop of the ileum are evident.

Histology showed peri-appendicular tissue with predominantly inflammatory mononuclear cell infiltrates, with numerous lymphoid aggregates; extensive areas of recent haemorrhage to the jejunal/ileal wall; and extensive mixed predominantly inflammatory mononuclear cell infiltrates and haemorrhagic areas to the peritoneum. The changes were consistent with peri-appendicitis, severe transmural haemorrhage of the small bowel and peritonitis.

Discussion

Acute appendicitis is one of the most common indications for abdominal surgery in paediatrics, with a peak incidence in the second decade of life. However, acute appendicitis in the first years of life is an uncommon event but with a high incidence of early perforation in younger patients. ¹

According to Alloo et al., ² appendicitis is rarely considered as a clinical diagnosis in children younger than three years of age. They conducted a review of 28 years’ experience of a single paediatric surgeon in academic practice, and found that 27 children younger than three years old comprised 2.3% of all children with appendicitis in this series. Abdominal pain, tenderness and vomiting were described as hallmarks of diagnosis. Interestingly, the mean age was 23 months, and they documented very high morbidity (59% complications), which may be attributed to three- to five-day delay in diagnosis. According to this study, the main misdiagnosis was precisely gastroenteritis.

Intestinal obstruction including the small bowel secondary to appendicitis is a complication widely referenced in the literature. According to Bandhari, ³ after a thorough review of the literature, intestinal obstruction secondary to appendicitis could be classified into (1) adynamic (small-bowel ileus), (2) mechanical (without strangulation), (3) strangulation of the intestine and (4) intestinal obstruction due to mesenteric ischemia.

The adynamic type (small-bowel ileus) is described as the most common type (seen in 1–5% of appendicitis), followed by mechanical intestinal obstruction without strangulation as a result of compression or traction of the small bowel trapped in an appendicular mass or abscess. Mesenteric ischemia, produced when the appendix adheres to a mesentery near the ileocolic artery and causes gangrene of the terminal ileum, is described as the rarest. ³

An inflamed appendix causing strangulation of the small bowel, sometimes described as an appendicular tourniquet, ⁴ appendiceal knotting, ⁵ appendiceal tie syndrome ⁶ or appendix constrictor ring, ⁷ is a very unusual entity, with only a few cases described in the literature, almost all of them in adults.

Mechanical obstruction with or without strangulation may result from the appendix wrapping around a bowel loop, or adhesion of the appendicular tip with the small bowel, cecum or posterior peritoneum forming a ring-like structure known as an appendiceal knot. A portion of the small bowel usually herniates through that ring or knot, forming a closed-loop obstruction with or without strangulation. ⁴ Assenza et al. describe a mechanism in which the tip of the inflamed appendix adheres to the bowel wall, producing small-bowel obstruction due to a kink in a loop. ⁸

In 2016, Chowdary ⁹ emphasised that this is a very rare condition and found only 16 cases reported in the literature. Interestingly, all of them were in adults, with only one fatality – a first case described by Naumov ¹⁰ in 1963, who died from sepsis six days after surgery.

Cases reported in infants are even more infrequent. Only three cases of appendicular knotting causing small-bowel strangulation have been described in the literature, none of them fatal. In 2002, Yang and Lee ¹¹ described the case of a 19-month-old boy with an unusual long necrotic appendix, knotting the terminal ileum. This resulted in the strangulation of a segment of the terminal ileum. In 2013, Sarkis ¹² described a case of a 21-day-old male neonate with the last 20 cm of ileum trapped under the appendix, the tip of which was adherent with the mesentery of the small bowel. In 2014, Mirza and Saleem ¹³ described a case of a two-year-old boy in which the appendix was found encircling a loop of the terminal ileum like a band. The tip of the appendix was adherent with the cecum, and the entrapped ileum was gangrenous. None of the three cases listed above were fatal.

In our case, there was an appendiceal knot (ring-like structure) through which a 70 cm portion of the jejunum-ileum was herniated. As in the case reported by Chaterjee, ⁴ the appendix tip appeared attached to the wall of a terminal portion of the ileum. The entrapped small bowel showed torsion and clearly appeared gangrenous. Post-mortem histological findings showed peri-appendicitis with predominantly inflammatory mononuclear cell infiltrates with numerous lymphoid aggregates. This last finding is essential, as is known that in young children, mesenteric lymphadenitis of viral origin can affect the lymphoid tissue of the appendix. ¹⁴

A reasonable reconstruction of events could be that lymphoid hyperplasia of viral aetiology caused the appendix to adhere to adjacent structures such as ileum, causing the knotting, peri-appendicitis/peritonitis and then intestinal obstruction and ischemia. It can also be hypothesised that the inflamed appendix had wrapped around the ileum before clinical presentation of intestinal obstruction and ischemia. The cause of death in this infant was classified as a complicated acute appendicitis: intestinal obstruction secondary to appendicular knotting. As the formation of an appendiceal knot is the result of acute appendicitis, the manner of death was deemed to be natural. To the best of the authors’ knowledge, no other fatal cases of appendicular knotting in children have been reported in the literature.

Despite the advances in radiology and clinical laboratory test accuracy, many studies still show significant discrepancies between clinical and post-mortem diagnoses. ¹⁵ A complete autopsy (both clinical and medico-legal), complemented by a consistent histological examination, remains an invaluable tool to document known suspected pathologies and undiagnosed medical complications, especially those considered unusual. Promoting autopsy would be a necessary step for health systems, giving doctors and health services an educational and an audit tool to improve quality and reducing mortality.