Vernix caseosa peritonitis – no longer rare or innocent: a case series

A 40-year-old nulliparous Caucasian woman was delivered by an uncomplicated elective lower segment caesarean section (LSCS) at 39 weeks gestation for breech presentation after an unremarkable antenatal course. She developed generalised peritonism, dyspnoea and pyrexia (39.3°C) 5 days later. A neutrophilic leucocytosis (white cell count (WCC) 15 × 10⁹/L) was present but both an abdominopelvic CT and CT pulmonary angiogram (CTPA) were normal. At laparotomy, cheese-like fibrinous debris was found throughout the peritoneal cavity. Peritoneal lavage was performed and an omental biopsy obtained after exclusion of other pathologies and on suspicion of VCP. Histological examination showed acute fibrinous inflammation of the serosal surface and early epithelioid granulomata associated with fetal squamous cells. Antibiotics were commenced and her symptoms rapidly resolved allowing discharge 5 days post-laparotomy. Five weeks later, she was readmitted to hospital for management of recurrent abdominal pain. All her blood tests and an upper abdominal ultrasound were normal. She was managed conservatively and discharged 3 days later. Ten weeks post-delivery, she represented for a second time, now with a persistent discharge from the edge of the abdominal incision. An abdominal wall wound sinus originating superficial to the rectus sheath was identified and excised allowing a rapid resolution of her symptoms. A collection of fetal squamous cells and a granuloma containing VC was observed within the sinus tract that was lined with inflammatory granulation tissue containing aggregates of histiocytes and multi-nucleated giant cells. Two years later, she has continued to have ongoing pain culminating in a recent laparoscopic excision of a VC abscess on the anterior abdominal wall adjacent to the liver, confirmed on histopathology.

A 32-year-old primiparous Caucasian woman had previously had a normal vaginal delivery but underwent elective LSCS at 39 weeks gestation for breech presentation. She had had a normal medical and antenatal history and although the LSCS was uncomplicated, she developed abdominal pain, flu-like symptoms and myalgias 3 days post-delivery. A diagnosis of urinary tract infection was made on the sixth postoperative day as she now complained of dysuria and her temperature was 41°C. Oral antibiotics were commenced and her condition improved, allowing her to be discharged. On day eight, she represented with further pyrexia, dysuria and severe abdominal pain requiring opiate analgesia. Urine culture was negative and an abdominopelvic ultrasound scan was normal. Once more, her symptoms settled with intravenous antibiotics and she was discharged a week later. She continued to experience intermittent episodes of pain culminating in a third admission 3 months postpartum. On this occasion, abdominopelvic CT and MRI identified an 8 cm inflammatory mass involving the wound. Laparotomy revealed a wound abscess and sinus formation tracking to the broad ligament that required extensive debridement and division of adhesions. Biopsies from the wound and inflammatory mass showed a chronic inflammatory granulomatous process associated with extravasated fetal squames and foreign material. A month later, she experienced further pain and dysuria. She underwent extensive investigation for thrombo-embolism, osteomyelitis, tuberculosis and further histological and microbiological investigation of wound biopsies that were normal. A repeat histopathological review of her previous biopsies diagnosed VCP. A urological opinion concluded that paravesicular inflammation was responsible for her urinary symptoms. In all, her pain continued for 7 months post-caesarean section before resolving.

A 43-year-old woman had had 12 first trimester miscarriages and terminations of pregnancy before the current pregnancy with an otherwise unremarkable medical and antenatal history. She underwent an uncomplicated emergency LSCS for failure to progress in the first stage of labour. Four days post-delivery, she developed shoulder-tip pain in association with abdominal distension, peritonism and pyrexia. An abdominopelvic CT was unremarkable and as her clinical picture was not improving despite analgesia and antibiotics, a laparoscopic investigation was undertaken. VC extruded through the Hassan port at entry and was also scattered throughout the peritoneal cavity (Figure 1, grey arrows). The procedure was converted to laparotomy through the Pfannenstiel incision, and adhesiolysis, lavage and peritoneal biopsy performed. Histology revealed an acute inflammatory exudate forming in response to fetal squames and keratin debris. Her recovery was slow and complicated by persisting pain and abdominal distension. By day 10, the abdominal distension had still not adequately settled despite nasogastric aspiration and parenteral nutrition. Consequently, a contrast CT was undertaken which showed a high-grade small bowel obstruction. A second laparotomy was performed – this time through a midline incision. The cause of the obstruction was identified as a large inflammatory mass of adhesions involving loops of small bowel. Adhesiolysis, omentectomy, appendicectomy and abdominal lavage were performed. Her appendiceal biopsy showed normal mucosa and underlying muscularis propria (Figure 2, black arrow), and a serosal inflammatory infiltrate (Figure 2, blue arrows) centred around fetal squamous cells (Figure 2, purple arrow). Interestingly, the inflammatory infiltrate was now mixed, comprising neutrophils, histiocytes (Figure 3, green arrows) and giant cells (Figure 3, black arrow) centred on aggregates of fetal squamous cells (Figure 3, purple arrow). Her symptoms rapidly resolved allowing her discharge home but she had subsequent admissions over the following 6-month period with subacute small bowel obstruction. On each of these occasions, she responded to conservative treatment and as yet, no further surgical interventions have been required.