An interesting perioperative rendezvous with a case of Henoch-Schonlein purpura.

Henoch-Schonlein purpura (HSP) is an autoimmune, multisystem, acute vasculitis of childhood commonly involving the skin, gut, joints and the kidneys. Fatal complications involving various systems can occur in this disease and careful perioperative management is advocated. We report here the occurrence of postoperative bradycardia and the successful perioperative management of a 12-year-old boy with HSP for diagnostic laparoscopy.

INTRODUCTION

Henoch-Schonlein purpura (HSP) is a multisystem, self-limiting, nongranulomatous, autoimmune immunoglobulin A (IgA) mediated small vessel vasculitis primarily affecting children.[12] HSP mainly affects children between 4 and 11 years of age in up to 90% cases.[3] The disease is characterized by a clinical tetrad of nonthrombocytopenic palpable purpura, abdominal pain, arthritis, and renal involvement.[3] The etiology is unclear, but the disease most commonly occurs in spring and is associated most frequently with recent respiratory tract infections in 90% cases. Other precipitating factors may be medications (nonsteroidal anti-inflammatory drugs, angiotensin-converting enzyme inhibitors, antibiotics), food allergies, immunization, insect-bites.[345] There are very few reports on cases of HSP encountered by anesthesiologists. Furthermore, no standard anesthetic guidelines have been suggested for patients suffering from HSP.[6] We present here the perioperative management of a patient with HSP for diagnostic laparoscopy.

CASE REPORT

A 12-year-old boy weighing 25 kg was admitted to our hospital with severe colicky periumbilical pain associated with recurrent bilious vomiting. He had a history of cough and fever since 1 month, which was treated. He developed a palpable purpura over legs and buttocks for which he was put on topical betamethasone cream. He also developed bilateral knee joint pain since 15 days [Figure 1].

Figure 1

Palpable purpura with pedal edema seen on the lower limb

He appeared poorly built and pale. There was a diffuse, nonblanching palpable purpura over buttocks and both lower limbs. Pulse rate (PR) was 80/min and regular. Blood pressure (BP) was 100/60 mm Hg in the upper limb. Abdominal examination showed diffuse tenderness and no organomegaly. Respiratory and cardiovascular systems were normal.

Laboratory investigations revealed a hemoglobin of 12 mg/dl, normal renal and liver function tests, prothrombin time and serum electrolytes. Urine examination revealed 6-8 pus cells and 8-10 red blood cells/high power f ield (HPF) and protein++; 24 h urine protein was 234 mg/dl. Ultrasonography of abdomen and pelvis showed left kidney size 9 × 4 × 13 cm, ectopic and fused with the lower part of right kidney. Left renal fossa was empty. Other organs were normal. A provisional diagnosis of HSP was made. Laboratory facilities for immunoglobulin examinations were not available and were hence not done.

The patient was posted for elective diagnostic laparoscopy for recurrent abdominal pain. After premedication with injections ondansetron 2 mg, glycopyrronium 0.1 mg, midazolam 1 mg and fentanyl 50 μg intravenously, he was induced with intravenous propofol 50 mg and atracurium 12 mg. Tracheal intubation was performed with 6 mm internal diameter endotracheal tube. Anesthesia was maintained with oxygen, nitrous oxide (3:5) and injection atracurium. Positioning was carefully done to prevent injury and skin necrosis. Intraabdominal pressure monitoring was done. End tidal carbon dioxide level was maintained at 25-30 cm H2O, BP at 100/60 mm Hg, PR at 80/min and SpO2 100%. The surgeons performed a laparoscopic appendectomy. The trachea was extubated after reversal. Patient was shifted to the pediatric intensive care unit for observation.

The patient continued to have abdominal pain even on the second postoperative day. The palpable purpura soon extended up to the trunk and upper extremities. He developed hematuria, scrotal swelling, facial puffiness with periorbital edema and bilateral pedal edema up to the knee. He was in agony with a with a bradycardia (PR of 54/min) and an irregular rhythm. BP was 120/70 mm Hg and SpO2 100%. Abdominal examination revealed signs of minimal free fluid, an increased abdominal girth with bowel sounds present. Urine examination showed protein 3+, 8-12 red blood cells/HPF. An electrocardiogram showed bradycardia and two-dimensional-echocardiography revealed normal findings. Serum electrolytes including sodium and potassium levels, serum creatinine and other investigations were within the normal limits.

He was started on intravenous dexamethasone 0.15 mg/kg for 3 days and then changed over to oral prednisolone 10 mg once daily. Intravenous fluids, antibiotics, oral paracetamol 250 mg twice daily were given. The bradycardia and irregularity of PR continued up to the sixth postoperative day. He improved by the seventh postoperative day and was shifted to the ward. Renal biopsy was done, which showed features suggestive of glomerulonephritis with <50% crescents. He was discharged with advice of regular follow-up, oral prednisolone 10 mg twice daily with tapering after 1 week and tablet enalapril 2.5 mg once a day. He is now being followed up regularly in our hospital every 15 days [Figure 2].

Figure 2

Palpable purpura over the upper limb

DISCUSSION

Henoch-Schonlein purpura is a multisystem vasculitis with multi organ involvement.[1] In our case too, four systems were involved-skin, joints, gastro intestinal system and renal system. Acute pediatric abdomen is a very common clinical problem with HSP sometimes leading to laparotomy.[34] Intussusception is the most common surgical complication. Our case too presented with abdominal complaints thus necessitating a laparoscopy.

Renal involvement is usually noted within a few days to 1 month after the onset of systemic symptoms; however renal symptoms usually present late. Hematuria is the common and earliest symptom. Proteinuria may also be seen. In HSP cases, attention should be paid to decreasing the risk of tissue compression and necrosis over pressure points such as that associated with positioning, BP cuff and endotracheal intubation.[1245] Fortunately, no complications of tissue compression were seen in our case. Perioperative management of liver and kidney function is very important for anesthetized patients with HSP.[5] A renal biopsy can be done in patients in whom diagnosis is uncertain or who have severe renal involvement as it is an invasive procedure. The percentage of glomeruli showing crescents is the most important prognostic factor. There were <50% crescents in our case.

According to diagnostic criteria of European League against Rheumatism and Pediatric Rheumatology European Society, palpable purpura plus one feature among the following suggests the diagnosis of HSP.[2]

Diffuse abdominal pain

IgA deposition in any biopsy

Arthritis/arthralgia

Renal involvement (hematuria/proteinuria).[2]

Our patient fulfilled all the above criteria for HSP.

Henoch-Schonlein purpura is self-limiting in 94% of children and 89% adults.[2] Symptomatic treatment is sufficient in case of only rash and joint pain. Oral steroids like prednisolone or methylprednisolone can be started and tapered down.[25] Immunosuppressive drugs (cyclophosphamide, azathioprine, cyclopropane A, mycophenolate mofetil) in combination with high dose pulse steroids are recommended for rapidly progressive glomerulonephritis and hemorrhages in the lungs and brain.[2] Veins can be deep seated because of long-term corticosteroid treatment and hence difficult cannulation is a possibility. Perioperative steroid cover can be instituted in those on treatment with steroids.[7] Nevertheless, our patient was not on preoperative steroids.

Neurological, pulmonary, cardiac and genitourinary complications may occur in HSP.[3] Diffuse life-threatening alveolar hemorrhage and resulting hypoxemia can occur. Perioperative alveolar hemorrhage in HSP has not yet been reported, but nevertheless should be kept in mind and postoperative ventilator support should be kept ready.[78]

Cardiac involvement is extremely rare. Atrioventricular block and arrhythmias with need for ventricular pacing and death from cardiac arrest have also been described.[9] Our patient too had bradycardia with irregular rhythm perioperatively from the second day. Central nervous system vasculitis, posterior reversible encephalopathy syndrome, hypertensive encephalopathy, cerebral venous thrombosis, subdural hematoma, subarachnoid hemorrhage, neuro-ophthalmological complications and myelopathy are the neurological manifestations that have been rarely reported in adults with severe HSP.[10] These should also be kept in mind, while handling the case perioperatively.

CONCLUSION

Monitoring and maintaining perioperative renal, liver and cardiac functions is important in HSP cases. The disease can worsen postoperatively. Meticulous preoperative evaluation with details of drug therapy, careful intraoperative technical care to avoid tissue compression and appropriate postoperative vigilance and care will avoid morbidity and mortality in cases of HSP.