Laparoscopic cholecystectomy in a patient with erythropoietic protoporphyria.
Erythropoietic protoporphyria (EPP) is an autosomal dominant defect in haem synthesis caused by defective ferrochelatase; the terminal enzyme. Excessive plasma protoporphyrin is deposited in the skin causing photosensitivity triggered by light wavelengths around 400nm (1). This corresponds to the absorption spectra of porphyrins, which are stimulated to produce free radicals and oxygen atoms (2).
EPP is usually detected before the age of 2, often following a history of screaming and pain when taken outside. Phototoxic burning may occur, although extreme blistering and oedema is more a feature of Gunther's disease--the rarer autosomal recessive form (3). Hepatic protoporphyrin accumulation may lead to liver failure (4).
Although the dangers associated with exposure to theatre lights at laparotomy have been reported (5,6), the possibility of phototoxicity during laparoscopy has never been explored.
A 35 year old woman presented for consideration of elective laparoscopic cholecystectomy. EPP was diagnosed after post-natal testing prompted by paternal carriage. Although her brother was severely affected, having undergone liver transplantation following hepatic failure, her own symptoms were purely cutaneous, marked photosensitivity causing pain and blistering.
Examination revealed mild scarring of the face and dorsal aspects of both hands, characteristic of EPP(3), but no other abnormal signs.
Abdominal ultrasonography (a routine prognostic tool in EPP) demonstrated a distended gallbladder containing multiple stones. The liver measured 14cm and exhibited normal echotexture. The CBD and biliary tree were of normal dimensions. Blood tests showed an isolated rise in ALT to 59u/L (0-50), and free protoporphyrin levels of 1.53[micro]mol/L (<0.01). All other blood tests were normal.
Expectant management risked future complications, including cholestasis. In combination with potential porphyric hepatotoxicity, this could have prompted catastrophic hepatic failure were the gallbladder left in situ. We therefore elected to proceed with prophylactic cholecystectomy.
We improvised a trial of the cutaneous effects of the laparoscopic light source to assess the possible intra-peritoneal consequences. The laparoscope was fixed 15cm from the exposed forearm for 20 minutes with the light source at full intensity. There was no immediate reaction. Following a few hours uneventful observation the patient was allowed home. Telephone follow up the next day confirmed no delayed phototoxicity. Surgery was thus scheduled.
General anaesthesia was achieved with agents previously used safely in EPP (7,8). Laparoscopy revealed a thickened gallbladder with stones impacted in Hartmann's pouch. The liver parenchyma was macroscopically normal. Dissection was complicated by an atypical waisted gallbladder, but exposure to the light source remained just 32 minutes.
The excised gallbladder contained 5 large pigment stones (fig 1).
[FIGURE 1 OMITTED]
Following an uneventful few hours on the ward, the patient was discharged and suffered no adverse events subsequently. In clinic 11 days later she was in good health and her scars were healing well.
High intensity operating lights are hazardous in EPP. One report describes severe 2nd degree burns to the abdominal wall after liver transplantation, later followed by wound dehiscence. The same case was further complicated by biliary fistulation leading to peritonitis, and duodenal ulceration requiring endoscopic adrenaline injection (5). A second report details a liver transplant complicated by phototoxic skin necrosis and multiple intestinal perforations (6).
Recent reports (1,7) describe complication-free aortic valve replacements and ventricular septal defect closures using yellow acrylate filters over theatre lights to eliminate wavelengths below 530nm. However, we are unaware of any reports on the effects of laparoscopic light sources on abdominal viscera.
Although the potential for internal phototoxicity from laparoscopy has not been formally evaluated, we felt that a trial of its cutaneous effects provided valid reassurance when considering a short procedure. As the main theatre lights were only utilised for a short time during peritoneal cannulation and port site closure, we did not use filters, although this is mandatory for longer exposure periods. Elimination of wavelengths below 530nm (the blue part of the spectrum) has raised safety concerns, as labels and monitors could feasibly be misread. However, no such incidents have been reported.
We used vecuronium, propofol, fentanyl and midazolam for anaesthesia, which have been reported as safe in porphyria (7,8). Other drugs are associated with porphyric crises, probably due to decreasing haem levels causing increased synthetic activity. These include common drugs such as diazepam, ketamine, pancuronium, thiopentone, enflurane and etomodate.
Although irrelevant in this case, if there is the potential for significant blood loss, circulating haemoglobin levels should be rigidly maintained with transfusions, avoiding the risk of intra-operative stimulation of haem synthesis precipitating a porphyric crisis (1).
Employing a simple strategy to evaluate the cutaneous reaction of an EPP patient to the laparoscopic light source gave maximal reassurance that laparoscopic cholecystectomy would not provoke intra-peritoneal complications. Our approach was appropriate when considering a short procedure, given the potential for disaster with expectant management. However, further research is necessary to validate the use of laparoscopy in EPP patients for longer or more complex procedures.
(1.) Asokumar B, Kierney C, James TW, Amato J, Tuman K. Anaesthetic management of a patient with erythropoietic porphyria for ventricular septal defect closure. Paediatr Anaesth 1999; 9: 356-358
(2.) Goldstein BD, Harber LC. Erythropoietic porphyria: Lipid peroxidation and membrane damage associated with photohaemolysis. J Clin Invest 1972; 51: 892-902
(3.) Murphy GM. Diagnosis and management of the erythropoietic porphyrias. Dermatologic Therapy 2003; 16: 57-64
(4.) Murphy GM, Hawk JLM, Corbett MF. The UK Erythropoietic Protoporphyria register: a progress report. Br J Dermatol 1985; 113 (Suppl. 29): 11-30
(5.) Shehade SA, Chalmers RJG, Prescott RJ. Predictable and unpredictable hazards of erythropoietic protoporphyria. Clin Exp Dermatol 1991; 16: 185-187
(6.) Bloomer JR, Weimer MK, Bossenmair IC. Liver transplantation in a patient with protoporphyria. Gastroenterology 1989; 97: 188-194
(7.) VYotsumoto G, Masuda H, Iguro Y, Kinjo T, Matsumoto H, Sakata R. Aortic valve replacement in a patient with erythropoietic protoporphyria. Ann Thoracic Surg 2003; 75: 1003-1005
(8.) Sneyd J, Kremier-Birnbaum M, Lust MR. Use of sufentanil and atracurium anesthesia in a patient with acute porphyria undergoing coronary artery bypass surgery. J Cardiothorac Vasc Anesth 1995; 9: 75-78
Authors: Thomas Roe and Ian S Bailey
Location: Southampton General Hospital, Southampton, UK
Citation: Roe T, Bailey IS. Laparoscopic Cholecystectomy in a Patient with
Erythropoietic Protoporphyria. JSCR. 2010 9:3
Article Date: November 2010
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|Author:||Roe, Thomas; Bailey, Ian S.|
|Publication:||Journal of Surgical Case Reports|
|Article Type:||Case study|
|Date:||Dec 1, 2010|
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