Vernix caseosa peritonitis (VCP) can occur following contamination of the peritoneum with amniotic fluid containing vernix caseosa, a white paste found on foetal skin. It is a rare condition with 35 cases reported since first described in 1976.[[1]]

Case report

A 32-year-old primigravida Caucasian woman underwent a caesarean section at 41+3 weeks for foetal distress. Meconium-stained liquor was noted. She was discharged two days later. The pregnancy was otherwise uncomplicated, with close monitoring required for her quiescent ulcerative colitis controlled with infliximab.

On day 5, she presented with severe abdominal pain, fever, and raised inflammatory markers. Antibiotics were administered for presumed endometritis without initial improvement. Ultrasound showed no retained products of conception. Computed tomography (CT) demonstrated features of ileus and possible peritonitis without perforation or other clear cause. She began improving and was discharged 1 week later on oral antibiotics.

Eight days later she represented in near identical fashion. CT now showed multiple small disseminated intra-abdominal abscesses and a probable contained ascending colon perforation. Multiple blood cultures were negative. Despite trialling conservative management with antibiotics for five days, she remained febrile and an interval CT showed no improvement (see Figure 1). Laparoscopy converted to midline laparotomy was performed. Findings included a large collection beneath the Pfannenstiel incision with adherent omentum, small bowel and sigmoid colon; multiple diffuse inflammatory adhesions, multiple whitish peritoneal nodules (biopsied; see Figure 2), uterine fundus defect and a pinpoint colonic perforation near the hepatic flexure. Interventions involved suture repair of the colonic perforation, loop ileostomy formation, appendicectomy, uterine fundus repair and extensive washout.

View Figures 1–3.

The patient gradually clinically and biochemically improved. Culture of intra-operative aspirates were negative. She discharged 16 days following surgery. Histology findings were consistent with VCP as seen in Figure 3. At 6-month clinic review, she remains asymptomatic and awaits ileostomy reversal.

Discussion

Most VCP cases occur after caesarean section where spillage of amniotic fluid occurs routinely. However, the true aetiology is not understood, given that VCP develops rarely and hypersensitivity reactions may play a role.[[1]] Patients commonly present 3–35 days postpartum with abdominal pain, peritonism, fever and leucocytosis.[[2]] Investigations for an infective source are negative and imaging findings are initially normal or non-specific. Typically, these unexplained unwell patients proceed to exploratory laparotomy where cheese-like white plaques are found diffusely on peritoneal surfaces.[[2]] Underlying organ pathology is not identified. However, due to erroneously attributing peritoneal findings to their involvement, unnecessary appendicectomy, colectomy, hysterectomy and salpingo-oophorectomy have occurred.[[3,4]] Biopsy later confirms VCP by identifying inflammatory infiltrates commonly with foreign-body giant cell reaction associated with anuclear squamous cells and lanugo hair shafts.[[5]]

In most respects, this case is classical of those described. However, this appears to be the first case to report an associated colonic perforation, though the exact timing and aetiology are unclear. In three cases who underwent segmental colectomy for suspected perforation, no perforation was identified.[[6–8]] An adherent inflammatory reaction causing focal erosion remains possible. Iatrogenic injury is considered unlikely from the caesarean section or laparotomy given prior CT findings of possible perforation. Any ulcerative colitis contribution is uncertain given unknown disease activity, though she remained symptomatically controlled.

Like most cases, the underlying pathology was not initially suspected by the treating teams. This is undesirable, as early diagnostic surgical exploration and washout is the mainstay of management in the non-improving patient.[[1,3]] This can be achieved successfully laparoscopically.[[5]] Intra-operative recognition of VCP will prevent unnecessary organ resection and prolonged antibiotics which are unlikely beneficial, unless rare organ injury is present.

This case reinforces the need to improve awareness and early recognition of VCP to ensure appropriate management in a timely fashion. It remains to be determined whether there is a true association between VCP and bowel perforation.