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Case Report: Red Urine After Day Care Strabismus Surgery

Caroline, Pregardien MD*; Marie-Cécile, Nassogne MD, PhD*; Demet, Yuksel MD, PhD*; Francis, Veyckemans MD

doi: 10.1213/XAA.0000000000000430
Case Reports: Case Report
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In the absence of surgery on the urinary tract, the emission of red urine after anesthesia should be considered as a diagnostic emergency because it can be a sign of hematuria, hemoglobinuria, blood transfusion reaction, significant myoglobinuria, or porphyria.

This case describes the management of a 12-year-old boy who presented red urine at the day care unit after strabismus surgery.

*Departments of Anesthesiology, Paediatric Neurology and Ophtalmology, Cliniques Universitaires Saint-Luc, Brussels, Belgium; and Department of Anesthesiology, Hôpital Jeanne de Flandre, Lille, France.

Accepted for publication August 25, 2016.

Funding: None.

The authors declare no conflicts of interest.

Address correspondence to Pregardien Caroline, MD, Cliniques Universitaires Saint-Luc, Ave. Hippocrate 10, 1200 Brussels, Belgium. Address e-mail to caroline.pregardien@uclouvain.be.

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CASE DESCRIPTION

This 12-year-old Caucasian boy was scheduled for ambulatory strabismus surgery. Preoperative history revealed a first strabismus surgery at the age of 7. Previous anesthetic record documented an uneventful short general anesthesia with sevoflurane. Also the patient was followed by an orthopedic surgeon for Achilles tendon retraction, treated conservatively with sparks and physiotherapy. General anesthesia was induced by mask with sevoflurane, followed with propofol 1 mg/kg and sufentanil 0.1 µg/kg, and maintained with sevoflurane in air and oxygen, the airway being secured with a laryngeal mask. Surgery lasted 30 minutes and no neuromuscular relaxants were used. Anesthesia and recovery were uneventful with no muscular rigidity, tachypnea, tachycardia, arrhythmias, or hyperthermia being observed. The patient was discharged at the day care unit after receiving IV paracetamol 15 mg/kg and ketorolac 0.5 mg/kg.

Three hours after anesthetic induction, when about to be discharged home, the patient passed red urine (Figure) without any other complaints. When specifically asked about muscular pain, the patient complained of mild myalgias in his legs. Vital parameters recorded at that time were HR: 101 bpm, noninvasive blood pressure: 140/70 mm Hg, and Spo2 99%, but body temperature was not measured. Samples of blood and urine were immediately sent to the laboratory to determine whether red urine was caused by (1) hematuria, (2) hemoglobinuria, (3) myoglobinuria, or (4) coloration because of a dye present in food or medications. A urinary dipstick made at the bedside was positive (+++) for hemoglobin. Urinary microscopic analysis was negative for red blood cells: this excluded hematuria. The blood tests showed elevated creatine kinases (CK): 80,255 UI/L (normal [nl] < 200), lactate dehydrogenase: 2399 UI/L (nl < 250), aspartate aminotransferase: 1017 UI/L (nl 15–40), and alanine aminotransferase: 419 UI/L (nl 10–40). The association of severe hyperCKemia with red urine and increased liver enzymes led to the diagnosis of rhabdomyolysis with myoglobinuria. Hemoglobin: 15.1 g/dL (nl 11–14.5), bilirubin 0.3 mg/dL (nl < 1.2), and K+ 4.29 mmol/L were in the normal limits.

Figure.

Figure.

To prevent renal failure, the patient received aggressive fluid replacement (100 ml/kg/24 h) with Plasmalyte® and glucose 5% and bicarbonate (30 mEq/L) for urine alkalinization. The CK level peaked at 24 hours postoperatively (97,778 U/L), but no electrolyte disturbance or renal dysfunction were observed. A repeat targeted history did not reveal any familial history of neuromuscular or metabolic disorders. However, the patient mentioned frequent muscle cramps treated with oral magnesium and a first episode of red urine during a school camp, which resolved spontaneously. This event was not linked with fever, and the patient admitted had been more active than usual because he was not so sporty because of the gait disturbance caused by Achilles tendon retraction. Neurological investigation revealed enlarged calves and a slightly positive Gower maneuver. An electrocardiogram showed signs of left ventricular hypertrophy (LVH), but an echocardiogram was considered normal without any signs of LVH.

To determine the cause of rhabdomyolysis, an extensive laboratory investigation including serology, dosage of organic acids, and plasma acylcarnitines was performed, but no specific anomaly could be detected.

The clinical presumptive diagnosis of Becker muscular dystrophy (BMD) was confirmed a few weeks later by molecular biology studies, which revealed a deletion of the dystrophin gene involving exons 13 to 42. Subsequent genetic analysis of the patient’s mother showed that she is not a carrier of the involved deletion.

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DISCUSSION

In the absence of surgery on the urinary tract, the emission of red urine after anesthesia should be considered as a diagnostic emergency because it can be a sign of hematuria (bleeding?), hemoglobinuria (hemolysis?), significant myoglobinuria (muscle damage?), porphyria, transfusion reaction, or coloration by a dye. Porphyria produces red urine (porto wine) only some time after the urine is exposed to air: this diagnosis could thus be excluded in our case. The microscopic analysis of urinary sediment allowed us to exclude hematuria. Because a cross-reaction exists between myoglobin and hemoglobin on the urinary dipstick, the 2 proteins could not be differentiated at the bedside. The association of red urine with severe hyperCKemia led to the diagnosis of subacute rhabdomyolysis.

Rhabdomyolysis is characterized by the elevated levels of CK to at least 5 to 10 times the upper limit of normal associated with pigmenturia and muscle pain. The presentation varies widely between patients and ranges from asymptomatic to a life-threatening condition with hyperkalemia, cardiac arrhythmia, acute renal failure, and disseminated intravascular coagulation. The presence of colored urine is a sign of major rhabdomyolysis because myoglobin appears in the urine when its serum concentration is greater than 1.5 mg/dL and becomes visible when its urinary concentration is greater than 100 mg/dL.1 We did not measure urinary myoglobin concentration because it had no added value for further differential diagnosis.

In the context of anesthesia, rhabdomyolysis can be caused by malignant hyperthermia, or reveal a metabolic or muscular disease. The initial management should include a symptomatic treatment to prevent complications and starting investigations to obtain a diagnosis.1–3

In our case, the malignant hyperthermia (MH) Clinical Grading Scale,4 a method for estimating the qualitative likelihood of a MH reaction gave a raw score of 15 (elevated CK >10,000 after anesthesia without succinylcholine): with a MH rank of 3, the likelihood of a MH crisis was somewhat less than likely.

Differentiation between anesthesia-induced rhabdomyolysis (AIR) and MH may be difficult. However isolated rhabdomyolysis or acute hyperkalemic cardiac arrest with no signs of systemic hypermetabolism strongly suggests AIR. AIR is believed to be triggered primarily by the administration of succinylcholine and/or an inhalational anesthetic agent. Its underlying pathophysiology is unknown. It has been postulated that an abnormal glycoprotein-dystrophin complex, the muscle membrane–stabilizing framework, results in an unstable sarcolemma. Exposure to a trigger stresses the muscle cell membrane and further increases the sarcolemmal instability and permeability, resulting in an influx of Ca2+ and leak of intracellular K+ and CK from myocytes. An abnormal nitrosylation of the RYR1 ryanodine receptor (mutations of which cause MH) could also be involved. These proposed mechanisms may explain the hyperkalemia, hyperthermia, tachycardia, and rhabdomyolysis observed in these patients.4,5

Because an underlying myopathy was suspected,1 other specific investigations were started: they showed normal serum acylcarnitines and urinary organic acids and could exclude a metabolic disease. Genetic analysis to search for a Becker myopathy revealed a deletion in the dystrophin gene involving exons 13 to 42 in Xp21.2. This confirmed the clinical diagnosis of Becker muscular dystrophy (BMD).

Muscular dystrophies (MDs) are rare X-linked recessive inherited disorders in which the dystrophin-glycoprotein complex is abnormal. Patients with Duchenne muscular dystrophy (DMD) present a complete loss of dystrophin. They suffer from progressive muscular degeneration beginning in the early childhood with a rapid degradation to severe muscle atrophy by adolescence. BMD, characterized by a partial loss of dystrophin, progresses at a slower rate and first symptoms usually appear in adolescence or later. The risks related to anesthesia for MD patients include, in addition to respiratory and cardiac complications, rhabdomyolysis and hyperkalemic cardiac arrest.4,5

A literature search revealed several case reports of AIR, resulting in perioperative cardiac arrest in boys with BMD. In 2 boys aged 6 and 18 years, the diagnosis of BMD was known, but in 2 younger patients aged 3 months and 3 years, BMD was diagnosed subsequent to the adverse event.6 Because of the delayed appearance of clinical signs and symptoms in patients with BMD, it is possible that an anesthetic adverse event leads to the diagnosis of myopathy. All children presenting for sedation or general anesthesia should be evaluated preoperatively regarding their motor development. Any delay in motor development or hypotonia should prompt suspicion of a subclinical myopathy and should warrant a neurological evaluation.

Our patient presented an isolated rhabdomyolysis, but these case reports emphasize that rhabdomyolysis can be complicated by hyperkalemic cardiac arrest. Early recognition and adequate treatment of rhabdomyolysis are of outer importance.

In conclusion, this “healthy” teenager posted for elective strabismus surgery developed anesthesia-induced rhabdomyolysis following a short exposure to sevoflurane in the context of paucisymptomatic undiagnosed BMD. In case of fast-track ambulatory surgery, instruction should be given to the parents to come back to the hospital if their child passes red- or cola-colored urine at home.

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CONSENT FOR PUBLICATION

The parents of the patient gave written permission for authors to publish the report.

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DISCLOSURES

Name: Pregardien Caroline, MD.

Contribution: This author helped complete the manuscript.

Name: Nassogne Marie-Cécile, MD, PhD.

Contribution: This author helped complete the manuscript.

Name: Yuksel Demet, MD, PhD.

Contribution: This author helped complete the manuscript.

Name: Veyckemans Francis, MD.

Contribution: This author helped complete the manuscript.

This manuscript was handled by: Raymond C. Roy, MD.

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REFERENCES

1. Zutt R, van der Kooi AJ, Linthorst GE, Wanders RJ, de Visser M. Rhabdomyolysis: review of the literature. Neuromuscul Disord. 2014;24:651659.
2. Pedrozzi NE, Ramelli GP, Tomasetti R, Nobile-Buetti L, Bianchetti MG. Rhabdomyolysis and anesthesia: a report of two cases and review of the literature. Pediatr Neurol. 1996;15:254257.
3. Klingler W, Lehmann-Horn F, Jurkat-Rott K. Complications of anaesthesia in neuromuscular disorders. Neuromuscul Disord. 2005;15:195206.
4. Hayes J, Veyckemans F, Bissonnette B. Duchenne muscular dystrophy: an old anesthesia problem revisited. Paediatr Anaesth. 2008;18:100106.
5. Gurnaney H, Brown A, Litman RS. Malignant hyperthermia and muscular dystrophies. Anesth Analg. 2009;109:10431048.
6. Poole TC, Lim TY, Buck J, Kong AS. Perioperative cardiac arrest in a patient with previously undiagnosed Becker’s muscular dystrophy after isoflurane anaesthesia for elective surgery. Br J Anaesth. 2010;104:487489.
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