Introduction

Acute appendicitis is the commonest surgical condition among children, and the cause remains elusive (1). The appendix is a tubular structure at the caecum with an average length of 4.5 cm in neonates and 9.5 cm in adults (2). The base of the appendix is wider in neonates and infants; therefore, the chances of luminal obstruction are rare (2). We present an unusual case of a premature neonate with perforated appendicitis where diagnosis was made intraoperatively. This is an uncommon phenomenon with atypical presentations.

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Case presentation

Our patient was a premature male baby who was referred to us with presenting complaints of being born before term and had experienced difficulty in breathing since birth. This baby was a second twin, born vaginally and had an APGAR score of 8 and 10 in the 1st and 5th min, respectively. The mother reported that the pregnancy was uneventful. At admission, diagnosis of a preterm baby at 32 weeks of gestation age by Fin storm was made as well as a diagnosis of idiopathic respiratory distress syndrome. The patient was started on intravenous ampicillin and gentamycin to prevent infections, started on oxygen therapy, and given intramuscular vitamin K to prevent hemolysis.
On the 5th day it was reported that the baby was breastfeeding poorly, had started to develop jaundice, had gradual abdominal distension and failure to pass stools. The patient was started on phototherapy for jaundice. An urgent plain erect abdominal X-ray was done which revealed free air under the right hemi-diaphragm (Fig. 1). 
The baby was then taken for an emergency laparotomy where intraoperatively approximately 25 mL of ascitic fluid contaminated with fecal matter and a hyperemic appendix with a perforation at its tip were found (Fig. 2). Other viscera were normal. An appendectomy was done and a biopsy taken for histopathology (which later confirmed appendicitis, Fig. 3). A thorough lavage of the abdominal cavity was done and an abdominal drain inserted. Postoperatively the baby developed surgical site infection on the 8th day that was dressed daily and the patient recovered. On day 18 the patient was discharged from hospital. The baby was then seen at the neonatal outpatient clinic 2 weeks after discharge with good recovery and growth.
Written informed consent was obtained from the patient’s mother for publication for this case report and accompanying images. A copy of the consent is available for review by the editor-in-chief of this journal.

 

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Click to view figure 2

Click to view figure 3

 

Discussion

Acute neonatal appendicitis is rare and the rate of perforation is higher in premature infants, resulting in peritonitis (2,3). The incidence of neonatal appendicitis has been reported to be 0.04–0.2% by El-Gohary (3); this is infrequent because of the wide appendix at the base, soft liquid diet, recumbent posture, and infrequent viral infections (3,4). El-Gohary also observes that of all reported cases, 25–50% occur in premature infants and this is due to their underdeveloped immunity, thin appendiceal wall, and indistensible caecum (3,5). Similar to our case, the diagnosis of perforation of the appendix is usually made operatively (3).
Primary neonatal appendicitis may present as an isolated entity where the etiology remains unclear, but secondary causes include Hirschsprung’s disease, necrotizing enterocolitis, meconium plug syndrome, or cystic fibrosis (2,4–6). Complications such as perforation are due to the thin-walled appendix, and the infection spreads to the whole abdomen due to the small omentum and small capacity of the abdominal cavity; therefore, early diagnosis and management are important to reduce morbidity and mortality (4). Complications are also partly because of the atypical clinical presentations of acute appendicitis (and perforation) compared with what would be elicited in older children and adults (4–6). As reported by Gupta (4), different symptoms include vomiting and abdominal distension (as was present in our case). Pneumoperitoneum due to a perforated appendix was also present in our case (Fig. 1); however, this is reported to be a rare finding by Ali et al. (7). The features of intestinal obstruction in our patient area result of peritoneal contamination causing intestinal ileus.
Surgery remains the preferred management for a perforated appendix, as was done in our case (6). Surgery includes appendectomy with an abdominal wash out, and the appendix taken for histological analysis to rule out other causes of perforation (2,6). Generally, the management outline of appendicitis is by clinically evaluating the signs and symptoms, with laboratory and radiological adjuncts; with the use of the Alvarado and pediatric appendicitis score scoring system, low-risk patients can be discharged with watchful waiting (2). C-reactive protein and computed tomography are considered gold standards with relatively high sensitivity and specificity to confirm clinical suspicion of appendicitis (1,8). Currently, laparoscopic surgery has become increasingly popular among pediatric surgeons along with other means such as percutaneous drainage of appendicular abscesses (2).

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Conclusion

Acute appendicitis is a rare entity in neonates and due to the uncommon presentation and diagnosis is difficult to make therefore causes delay in the management which may lead to high morbidity and mortality.

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Consent

Written informed consent was obtained from the patient’s mother for publication for this case report and accompanying images. A copy of the consent is available for review by the chief editor of this journal.

 

Authors’ contributions
JL came up with the idea, drafted and prepared the manuscript. AH, DW and JL performed the operation and reviewed patient’s medical records. AS prepared and reported the radiological films. PA prepared the histology slides and the histopathology results, and RP and DM provided the technical input. All authors read and approved the final manuscript.

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Competing interest

The authors declare they have no competing interests.

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Acknowledgement

The authors would like to thank the mother of the child for permission for the information to be shared for further learning purposes.

 

References

1. Kumar J, Shepherd G, Abubacker M, et al. Trends in incidence of acute appendicitis in children. Acad J Pediatr and Neonatol. 2017;3(5):27–29.
2. Almaramhy HH. Acute appendicitis in young children less than 5 years. Ital J Pediatr. 2017;43(1):15.
3. El-Gohary MA. Neonatal appendicitis with perforation: A case report. J PediatrSurg Case Rep. 2014;2(7):353–354.
4. Gupta V, Sharma SB. Neonatal appendicitis with perforation: A case report and review of literature. J Indian AssocPediatr Surgeons. 2005;10(3):179.
5. Tumen A, Chotai PN, Williams JM, et al. Neonatal perforated appendicitis attributed to localized necrotizing enterocolitis of the appendix: A review. J Neonatal Surg. 2017;6(3):60.
6. Sabrina JD, Tan CW, Nallusamy M. Neonatal perforated appendicitis. J PediatrSurg Case Rep. 2018; 38:66–68.
7. Ali R. Perforated appendicitis with massive intraperitoneal free air in a two-month-old baby. J Surg Pakistan (International). 2015; 20:38–39.
8. Marzulli P, Germany C, Krauss BS, et al. Appendicitis in children less than five years old: A challenge for the general practitioner. World J Limpidity. 2015:4(2):19