Atypical presentation of perforated peptic ulcer disease in a 12-year-old boy

Abstract

A 12-year-old boy was referred to the surgical unit with 4 h history of severe lower abdominal pain and bilious vomiting. No other symptoms were reported and there was no significant medical or family history. Examination revealed tenderness in the lower abdomen, in particular the left iliac fossa. His white cell count was elevated at 19.6×10⁹/L, with a predominant neutrophilia of 15.8×10⁹/L and a C reactive protein of <0.3 mg/L. An abdominal X-ray revealed intraperitoneal gas and a chest X-ray identified free air under both hemidiaphragms. Subsequent diagnostic laparoscopy identified a perforated duodenal ulcer that was repaired by means of an omental patch. The case illustrates that although uncommon, alternate diagnoses must be borne in mind in children presenting with lower abdominal pain and diagnostic laparoscopy is a useful tool in children with visceral perforation as it avoids treatment delays and exposure to excess radiation.

Background

In a recent multicentre European study, the prevalence of peptic ulceration was 8.1% in children presenting with abdominal pain, the majority of patients being males in the second decade of life.¹ Helicobacter pylori infection and non-steroidal anti-inflammatory drug ingestion are the main aetiological risk factors in the paediatric age.² The classic presentation of patients with peptic ulcers is one of epigastric pain, often associated with vomiting.

Perforated peptic ulcer disease in children is rare, seen in only 5% of cases, and is usually associated with a preceding history of typical pain, and presentation with generalised peritonitis. In the largest study in the literature, 52 cases of perforated duodenal ulcer disease were reported over a 20-year period.³ All patients in this series reported a history of abdominal pain and 94.2% had signs of peritonitis at presentation.

As with all acute abdominal emergencies, rapid diagnosis and prompt treatment are the keys to a successful outcome, this being of particular importance in cases of visceral perforation. Faced with radiological evidence of perforation but an uncertain origin, options include cross-sectional imaging or immediate surgery. Diagnostic laparoscopy, as selected, excludes the radiation exposure of abdominal CT as well as its associated time delay. It also allows direct visualisation of the whole peritoneal cavity, thorough evacuation of food material and gastric secretions as well as providing direct visualisation of the perforation and facilitating repair.

Case presentation

A 12-year-old boy presented to the emergency surgical intake via the out of hours general practitioner service with very severe lower abdominal pain that woke him from sleep. The pain was constant in nature, scoring 10 out of 10 in severity, but did not radiate and no exacerbating factors were reported. The pain was associated with vomiting but no alteration in bowel habit. There was no medical or family history of note. He had no urinary or respiratory symptoms, took no medications and lived with four siblings who were all well.

On examination, he appeared flushed, with tenderness in the lower abdomen and peritonism that was markedly worse over the left iliac fossa. He was tachycardic with a heart rate of 140 bpm, blood pressure of 110/89 mm Hg, a temperature of 36.6°C and a respiratory rate of 20 bpm. Peripheral intravenous access was established and a standard blood profile sent for evaluation. The child was maintained nil per mouth and provided with adequate analgesia and antiemetics. Abdominal and chest radiographs were also requested.

Blood work revealed an elevated WCC at 19.6×10⁹/L (neutrophilia of 15.8 × 10⁹/L) but a normal CRP of <0.3 mg/L. The abdominal X-ray revealed intraperitoneal air and free air was seen under both hemidiaphragms in the chest radiograph (figures 1 and 2). A diagnosis of perforated viscus was established, and given the location of the pain in the lower abdomen, the perforation was believed to originate from the appendix or a Meckel's diverticulum.

Figure 1.

Abdominal X-ray demonstrating free intraperitoneal air as arrowed.

Figure 2.

Erect chest X-ray showing bilateral subdiaphragmatic air (arrow).

Treatment

The patient was consented for diagnostic laparoscopy and to proceed appropriately dependent on the diagnosis. Laparoscopy revealed a large volume of turbid fluid tracking to the pelvis and a 0.5 cm perforation in the anterior wall of the first part of the duodenum was observed. The perforation was repaired with an omental patch and the peritoneal cavity thoroughly washed with warm saline.

Outcome and follow-up

His postoperative recovery was unremarkable and he was discharged 6 days later on empirically prescribed H. pylori eradication therapy. Prior to discharge a serum gastrin level was sent, and returned as being normal. At follow-up, he was symptom free and was prescribed a maintenance dose of 20 mg omeprazole. He was also referred to a paediatric gastroenterologist for on-going care.

Discussion

The current case is unusual in that the location of pain was atypical, there being no preceding upper abdominal pain, and the clinical signs were limited to the lower abdomen, specifically the left iliac fossa. The existing literature would suggest that the majority of children with perforated peptic ulcers report severe abdominal pain with evidence of generalised peritonitis.^{1 3}

Right iliac fossa pain as a presentation of a perforated peptic ulcer has been documented.⁴ Indeed, the eponym Valentino's syndrome has been applied to this presentation and relates to the famous actor Rudolph Valentino who underwent an appendicectomy for suspected appendicitis but then developed multiorgan failure and died. At autopsy, a perforated peptic ulcer was identified as the cause of his initial presentation.

The likely mechanism accounting for lower abdominal pain rather than epigastric pain, as confirmed by laparoscopy, is that gastric contents descend under gravity along the paracolic gutters. However, it is uncertain why in this case the fluid preferentially gathered in the left iliac fossa. A detailed review of the published English language literature by means of a comprehensive electronic search of MEDLINE and manual review of the bibliographies of relevant papers failed to identify a previously documented similar presentation of perforated peptic ulcer disease.

In the largest study to date, the mean age for paediatric perforated peptic ulcer disease was 14.2 years, with 90% being adolescents.³ The majority of children (>80%) are males, with most reporting a predisposing risk factor such as abdominal pain of greater than 3 months duration; underlying medical illness; family history of peptic ulcer disease; active smoker and alcohol use.³

In the case reported herein, the preoperative diagnosis was of perforated viscus but the origin was unclear. Faced with this clinical scenario, there are two available options namely to try and define the defect preoperatively with further imaging or to proceed to surgical exploration. In a study of 85 patients with visceral perforation, CT scan was able to accurately identify the point of perforation in 86% of cases,⁵ and while there are no series specifically looking at diagnostic laparoscopy in the evaluation of visceral perforation, a series of 1320 patients undergoing evaluation for abdominal pain showed a diagnosis was established in 90% of cases.⁶ Furthermore, laparoscopy changed the preoperative diagnosis in 30% of cases, and allowed for immediate laparoscopic operation in 83% with the remaining 7% converted to an open operation.

In the current paediatric case, with a lesser range of differential diagnoses available for the perforation, rather than requesting a CT scan, a decision was made to progress immediately to laparoscopy. This decision omitted the radiation exposure and reduced the interval from admission to definitive management. Reducing the time interval delay from presentation to surgery with paediatric perforated peptic ulcers, as with all surgical conditions, is associated with a reduction in morbidity and mortality.³ In adults with left iliac fossa pain and intraperitoneal air present, perforated diverticular disease becomes an important consideration and CT may be of value in determining the need/urgency of surgery and so taking into account each case independently is important.

It is clear from the literature that perforated peptic ulcer disease is frequently not considered in the differential diagnosis of a child with peritonism leading to delays in management.^{3 7 8} It is also clear from a large Danish registry report that delays in diagnosing and treating perforated ulcers is associated with poorer outcome, with each hour leading to a 2.4% decreased probability of survival.⁹

The published series illustrate that there is no consensus as to the investigation of children with abdominal pain, with significant intercentre variation. In the current case, the abdominal and chest radiographs confirmed free intraperitoneal gas, and so rather than investigating using radiological means, a laparoscopy was performed to allow diagnosis and management within a reduced time frame.

After managing the acute presentation of peptic ulceration in the paediatric patient, it is important to treat, if present, with appropriate eradication therapy.³ Indeed, evidence from a systematic review and meta-analysis of this approach has suggested empirical treatment with H. pylori eradication therapy is superior to antisecretory treatment alone.¹⁰ Other risk factors such as hypersecretory states should also be sought and treated. All children should be referred for endoscopic evaluation to ensure the ulcer has healed.¹¹

Learning points.

• Peptic ulcer disease is not uncommon in the paediatric population accounting for 8.1% of patients investigated for abdominal pain; however, ulcer perforation is rare.

• Suspect perforated peptic ulcer in adolescents who present with acute abdominal pain and peritoneal signs, in particular if upper abdominal pain has been reported over the preceding months.

• Once visceral perforation is diagnosed in a child, diagnostic laparoscopy with a view to definitive surgery would appear to be the appropriate option to expedite treatment and reduce delays.