Vernix caseosa peritonitis (VCP) is a rare complication following cesarean section (CS) or vaginal delivery. Only 34 cases have so far been reported in the literature with two of these occurring after vaginal delivery. Because VCP commonly occurs following CS, it should always be suspected whenever a postpartum woman presents with acute abdomen following CS. Postvaginal delivery VCP occurrence is almost unheard of with the first such case reported by Sadath et al. in 2013. In this particular case report, we highlight the diagnostic and management dilemmas before VCP could finally be confirmed.
A 29-year-old woman, para 3, presented to our emergency department with progressive difficulty in breathing associated with generalized lethargy and poor appetite. She was at day 5 post uneventful spontaneous vaginal delivery (SVD). Clinically, she was in shock with blood pressure at 95/60 mmHg, tachycardic (130 bpm), and severely tachypneic (the respiratory rate at 38/min). She remained afebrile (37. 2°C) with good oxygen saturation (98%) on the nasal cannula. Her lungs were normal although her abdomen was mildly distended but with no features of peritonitis. Her uterus was also well contracted at 14 weeks in size. She had leukocytosis (white blood cell of 15.2 × 103/uL) with features of acute renal failure (urea 22.5 mmol/L; creatinine 340 umol/L) and remarkably high C-reactive protein (318 mg/L). Arterial blood gases showed partially compensated metabolic acidosis with high lactate 4.6 mmol/L. Her chest radiography showed perihilar heterogeneous opacities although the costophrenic angle was spared.
The patient's preliminary bedside ultrasound showed the presence of minimal free fluid in the pelvis. She was initially managed as septic shock secondary to community-acquired pneumonia and admitted to the intensive care unit (ICU) for ventilatory support and inotropes. Pulmonary embolism was ruled out based on an urgent computed tomography (CT) pulmonary angiogram done on the day of admission.
When the progressive accumulation of ascites was ascertained through serial abdominal ultrasound, the surgical and obstetric team was then consulted for the possibility of a septic abdomen. Despite no other abdominal signs apart from ascites, a CT scan of the abdomen and pelvis was conducted on day 2 of admission which subsequently revealed gross ascites with small bowel thickening and collapsed consolidation of both lower lobes of the lungs with effusion. All other organs were found normal [Figure 1].
Abdominal paracentesis was conducted when a diagnosis of primary peritonitis was initially considered. The peritoneal fluid appeared turbid with serum albumin ascites gradient at 12 g/L, total protein 6736 mg/L, glucose 3.4 mmol, lactate dehydrogenase 7388 unit/L, and polymorphonuclear cell count 3906 cells/uL. A microscopic examination demonstrated moderate pus cells but with no organism detected on Gram staining. A test for acid-fast bacilli organisms also turned out negative. However, despite these results, secondary peritonitis was suspected since the patient was responding poorly to conservative management in addition to her extremely high ascitic polymorphonuclear cell count. She subsequently underwent diagnostic laparoscopy and laparotomy and peritoneal washout on day 3 of ICU admission.
Intraoperatively, 1.2 L of turbid ascitic fluid were found dispersed all over the abdominal quadrants with cheesy exudates indicated mainly in the pelvic cavity region [Figure 2]. Although pancreas and retroperitoneal tissues appeared edematous, all other abdominal and pelvic organs were otherwise normal with no peritoneal deposits detected. The cheesy exudates and part of “crumpled” greater omentum were sampled for histopathological examination. The patient's immediate postoperative diagnosis was decided as either primary peritonitis or acute pancreatitis. Her recovery was slow; nonetheless, she was deemed well enough to be taken out of ICU 10 days later and subsequently discharged home after 2 weeks.
The histopathological examination of the greater omentum revealed features of acute chronic inflammation. The exudates yielded anucleated squames and keratin debris, with several detached stratum corneum-like materials with a basket weave pattern. These findings subsequently led to the diagnosis of VCP [Figure 3].
Our report of VCP following an uneventful SVD is, to our best of knowledge, the third reported case of VCP occurring post-SVD and the first of its kind to be reported in ASEAN.
Vernix caseosa, a creamy-like whitish cutaneous material covering the skin of a newborn, is produced by fetal sebaceous glands. It acts as the epidermal barrier for the newborn and is formed completely in the final trimester. Vernix exhibits a nonlamellar lipid matrix containing hydrated corneocytes with no intercorneal desmosomal connection. The vernix structure thus exhibits “pasta and cheese” morphology with a “mobile” architecture.
Spillage of amniotic fluid into the maternal abdominal cavity is inevitable during manipulation of the fetus in CS or from uterine perforation. However, the occurrence of this phenomenon is difficult to explain in uncomplicated SVD cases such as ours. Davis et al. proposed the idea of retrograde flow of vernix caseosa through the Fallopian tube during vaginal delivery whereby the spillage into the peritoneal cavity could stimulate an inflammatory reaction leading to an increase in vascular permeability and subsequently peritonitis as observed in this case. However, due to the very limited number of reported cases, such events should be regarded as insignificant since peritonitis only develops in some instances. The exact pathophysiology is still poorly understood although one theory is that the hypersensitivity reaction might be due to either antenatal or previous pregnancy sensitization.
In general, VCP has no specific clinical features compared to another acute abdomen. Patients usually present with generalized abdominal pain associated with fever and leukocytosis typically within 10 days of delivery although this might also occur between the 3rd and 5th week. Danawar et al. reported a case that presented initially with shortness of breath in addition to abdominal pain and fever which then progressed into acute respiratory distress syndrome. This mimics to an extent the presentation of our case. Respiratory embarrassment or failure therefore should be regarded as a potential sequelae or spectrum of disease following a VCP diagnosis. The majority of cases were negative cultures either from the blood or urine. The only positive culture as reported by Herz et al. was from peritoneal fluid which grew B. Melaninogenicus. In all these cases, ultrasound or CT scan demonstrated the presence of intra-abdominal fluid or collection with occasional peripherally enhancing nodules over the liver and within the pelvis.
The diagnosis of VCP should be considered in all postpartum women presented with acute abdomen regardless of the absence of specific laboratory and imaging features. This is to avoid delay in diagnosis and for control of sepsis. Early diagnostic laparoscopy and/or laparotomy with peritoneal lavage has been shown to have a significant impact on recovery and control of sepsis. Awareness of such diagnosis could also avoid unnecessary organ resection. Chambers et al. demonstrated good outcomes in their series of 20 cases of VCP following CS where out of all the reported cases, 17 patients (85%) had laparotomy and organ resection, while the remaining had a laparoscopy (n = 2) and aspiration (n = 1). The latter approach guided by ultrasound may also be considered in uncompromised and stable patients as proposed by Danawar et al. and Myers and Fernando. Despite this, we believe the presence of peritonitis with sepsis warrants a definitive approach such as diagnostic laparoscopy and/or laparotomy to rule out other more common pathologies.
Steroid therapy has also been administered to treat VCP as reported by several case series. Adjuvant treatment appears to enhance clinical outcomes by suppressing inflammatory response and facilitating recovery. Mahmoud et al. reported no recurrence or complications with good outcomes following such treatment.
In conclusion, diagnosis of VCP should be suspected in any postpartum woman presented with peritonitis and ascites. Emergency diagnostic laparoscopy and/or laparotomy and lavage must be considered, especially in the management of unstable patients. Intraoperatively, the appearance and sampling of the cheesy material are critical to confirm the diagnosis of VCP. Awareness of such a diagnosis will also avoid the resection of unnecessary organs. Postoperatively, steroid therapy has been shown to be beneficial although with limited evidence.
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The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initial will not be published and due efforts will be made to conceal the identity, but anonymity cannot be guaranteed.
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