Fatal phenytoin-related toxic epidermal necrolysis: case report

Toxic epidermal necrolysis is a rare but often fatal hypersensitivity reaction to numerous agents, including most anticonvulsants. The authors present a case of fatal phenytoin-related toxic epidermal necrolysis in a patient who was given prophylactic anticonvulsant therapy after he sustained a moderately severe closed head injury. The typical course and current management of toxic epidermal necrolysis are reviewed, as are the indications for the prophylaxis of posttraumatic epilepsy.     

A 12-year retrospective study of non-burn skin loss (burn-like syndromes) at a tertiary burns unit in a developing country

BACKGROUND: A retrospective study of the presentation, etiology, and prognosis of non-burn epidermal loss managed at the Lagos University Teaching Hospital Nigeria over a 12-year period. MATERIALS AND METHODS: Admission records of patients managed for non-burn skin loss were retrieved from the medical records. Demographic details of the patients, the initial diagnosis, final diagnosis, treatment and outcome of treatment was noted. RESULTS: A total of 23 patients were identified, 17 (74%) had idiosyncratic drug reactions. Of this 17, 6 (26%) had Steven Johnson Syndrome, 6 (26%) had Steven Johnson Syndrome/toxic epidermal necrolysis while 5 (22%) presented with toxic epidermal necrolysis. Three of the five patients with toxic epidermal necrolysis died. The age range of patients with idiosyncratic adverse drug reactions was 2-28 years, mean, 10.18+/-1.44 years and male to female ratio of 1:1.83. The body surface area involved ranged from 8 to 78%; mean 26.65+/-6.08%. The agents suspected for the reactions were Co-trimoxazole (41.2%) and combination of Co-trimoxazole, and Fansidar (17.6%). Other conditions seen were two (9%) Staphylococcal Scalded Skin Syndrome, three (13%) had Necrotizing Faciitis, one of whom was HIV positive and died. One (4%) patient presented with pemphigus vulgaris. The presentation and management of the patients was discussed.     

Improved burn center survival of patients with toxic epidermal necrolysis managed without corticosteroids.

Fifteen consecutive patients with toxic epidermal necrolysis or the Stevens-Johnson syndrome managed without corticosteroids after transfer to the burn center (group 2) are compared to a previous consecutive group of 15 who received high doses of these drugs (group 1). Group 2 had a 66% survival, which was a significant improvement compared to the 33% survival in group 1 (p = 0.057). In group 1, mortality was associated with loss of more than 50% of the body surface area skin. In group 2, mortality was related to advanced age and associated diseases. Age, extent of skin loss, progression of skin loss after burn center admission, incidence of abnormal liver function tests, and the incidence of septic complications were not significantly different in the two groups (p greater than 0.10). The incidence of detected esophageal slough was similar in both groups. Nonsteroid (group 2) management was associated with a decreased incidence of ulceration of gastrointestinal columnar epithelium, Candida sepsis, and an increased survival after septic complications. The combined experience of these 30 patients suggests that corticosteroids are contraindicated in the burn center management of toxic epidermal necrolysis and the Stevens-Johnson syndrome.     

Subtotal pancreatectomy for cancer: closure of the pancreatic remnant with staplers

This paper presents a 2-year series of 26 consecutive pancreatectomies for periampullary cancer where the pancreatic tail was closed with a stapler in order to avoid complications related to a pancreatico-digestive anastomosis. The follow-up period was 14 months or more. Seven patients developed operative complications. Pancreatic fistulas developed in 3 patients. The fistulas closed spontaneously in 2 of the patients after 2-4 months. Intraabdominal abscesses developed in 4 patients and required surgical drainage. In 1 of these patients, the abscess eroded a large vessel with a fatal outcome resulting in an operative mortality rate of 3.8%. A transient postoperative gastric stasis was observed in seven patients. Postoperative hospital median stay was 27 days (range 10-83 days). Eighteen patients have died after 4-30 months in recurrent disease and seven patients are alive after a follow-up period of 15-29 months. Pancreatic endocrine function seemed well preserved; diabetes mellitus has developed in only one patient. In conclusion, it appears that subtotal pancreatectomy with closure of the pancreatic remnant with staples gives a low morbidity and mortality. Although the conclusion should be tempered by the small number of patients, the results justify continued evaluation of this technique with long-term follow-up.     

Burn unit management of toxic epidermal necrolysis.

Toxic epidermal necrolysis is the name given to a group of dermatologic disorders characterized by a separation of epidermis and dermis with a subsequent skin slough. The denuded areas have the appearance of a second-degree burn. The complications of infection, negative nitrogen balance, severe pain, and emotional instability are identical to those seen in the patient with major burns. There are difficulties in patient management and advantages in burn unit care. As with the major burn, care of the patient with skin loss from toxic epidermal necrolysis is extremely complex, requiring the expertise of a burn team along with that of the dermatologist.     

Visceral artery pseudoaneurysm in necrotizing pancreatitis: incidence and outcomes

BackgroundVisceral artery pseudoaneurysms (VA-PSA) occur in necrotizing pancreatitis; however, little is known about their natural history. This study sought to evaluate the incidence and outcomes of VA-PSA in a large cohort of patients with necrotizing pancreatitis.MethodsData for patients with necrotizing pancreatitis who were treated between 2005 and 2017 at Indiana University Health University Hospital and who developed a VA-PSA were reviewed to assess incidence, presentation, treatment and outcomes.ResultsTwenty-eight of 647 patients with necrotizing pancreatitis (4.3%) developed a VA-PSA between 2005 and 2017. The artery most commonly involved was the splenic artery (36%), followed by the gastroduodenal artery (24%). The most common presenting symptom was bloody drain output (32%), followed by incidental computed tomographic findings (21%). The median time from onset of necrotizing pancreatitis to diagnosis of a VA-PSA was 63.5 days (range 1–957 d). Twenty-five of the 28 patients who developed VA-PSA (89%) were successfully treated with percutaneous angioembolization. Three patients (11%) required surgery: 1 patient rebled following embolization and required operative management, and 2 underwent upfront operative management. The mortality rate attributable to hemorrhage from a VA-PSA in the setting of necrotizing pancreatitis was 14% (4 of 28 patients).ConclusionIn this study, VA-PSA occurred in 4.3% of patients with necrotizing pancreatitis. Percutaneous angioembolization effectively treated most cases; however, mortality from VA-PSA was high (14%). A high degree of clinical suspicion remains critical for early diagnosis of this potentially fatal problem.     

Toxic epidermal necrolysis after phenytoin usage in a brain trauma patient

Toxic epidermal necrolysis is a drug-induced, rare, but life-threatening skin eruption. The main differential diagnoses are drug-induced erythema (hypersensitivity syndrome), acute graft-versus-host disease, staphylococcal scalded skin syndrome, and toxic shock syndrome. Because the therapy for toxic epidermal necrolysis and acute graft-versus-host disease differs largely from the others, it is necessary to make an accurate diagnosis. In addition to a detailed medical history, skin biopsy is mandatory because the skin eruptions are not always unequivocal. Discontinuation of the causing agent is crucial, and treatment in specialized intensive care units or burn units is supportive. Currently there is no specific treatment for toxic epidermal necrolysis. Advantages from corticosteroids, plasmapheresis, intravenous immunoglobulin, cyclophosphamide, cyclosporin, and N-acetylcysteine still remain to be established by controlled trials, or have failed to prove a benefit (thalidomide). The patient presented here demonstrates the difficulties in diagnosing toxic epidermal necrolysis in a critically ill patient. A short overview of the pathogenesis and the management of toxic epidermal necrolysis is provided.     

Cutaneous reactions to antibacterial agents

Antibacterial agents may cause a variety of untoward reactions. Some range from transient, mild erythema to toxic epidermal necrolysis, often resulting in disability and death. Both in vivo and in vitro tests are becoming useful for the diagnosis of the causative agent in drug eruptions. The drug hypersensitivity syndrome may be associated with thyroid abnormalities often occurring months after the drug has been withdrawn. Symmetrical small joint polyarthritis, fever, and malaise may be the presenting findings in a patient with drug-induced lupus erythematosus. Exanthematous drug eruptions without high fever, mucosal involvement, or joint symptoms often resolve without discontinuation of the drug. The differential diagnosis of Stevens-Johnson syndrome and toxic epidermal necrolysis depends on the percentage of epidermal detachment.     

The place of Sugiura operation for portal hypertension and bleeding esophageal varices

Fifty patients with portal hypertension and bleeding varices aged 10 months to 72 years were treated with a modified Sugiura, nonshunt operation (n = 26) or shunting procedures (n = 24) in accordance with the following predetermined therapeutic protocol: after resuscitation and diagnostic endoscopy, an emergency mesocaval shunt procedure was carried out if bleeding could not be stopped (group 1, n = 10). When bleeding could be stopped, the patients underwent full investigation and were then treated with either the distal splenorenal (DSR) shunt if the criteria of Warren were satisfied (group 2, n = 14) or with a modified Sugiura procedure in all other circumstances (group 3, n = 26). Patients were evaluated at 1.5 to 6 years. The rates for operative deaths, recurrent hemorrhage, encephalopathy, late deaths, and actuarial patient survival at 6 years were as follows: 20%, 30%, 30%, 20%, and 60% for group 1; 14.3%, 14.3%, 14.3%, 7.2%, and 79% for group 2; and 7.7%, 3.4%, 0%, 0%, and 93% for group 3, respectively. Within 3 months after the Sugiura operation, varices disappeared in 95% of patients and hypersplenism was relieved in all. Major complications were gastric and esophageal leaks in two patients (fatal in one) and temporary dysphagia in six. We conclude that the modified Sugiura nonshunt operation is probably the preferable treatment for variceal hemorrhage in the nonalcoholic patient because it is effective in arresting hemorrhage, has low operative mortality, low recurrence rate, no encephalopathy, and excellent survival rates.     

Upper airway involvement in Stevens–Johnson syndrome and toxic epidermal necrolysis

BackgroundStevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare life-threatening hypersensitivity conditions associated with epidermal detachment and mucositis. The indication for flexible nasoendoscopy (FNE) and overall predictive factors for early intubation are unclear.ObjectivesTo describe the incidence of airway involvement and the key indicators for intubation in our SJS or TEN patient cohort. To determine the association between FNE findings and early intubation.MethodsA retrospective review of 45 patients with biopsy proven SJS or TEN admitted to an Australian tertiary burns centre from 2010 to 2017.ResultsThirty-five patients were diagnosed with TEN (77.8%), followed by overlap syndrome (SJS-TEN) (n = 6, 13.3%) and SJS (n = 4, 8.9%). Twenty (44.4%) patients were intubated; and all 20 had a diagnosis of TEN (100.0%) (p < 0.05). Intubated patients had a higher increase in total body surface area percentage(%) from day 1–3 [10.0% (IQR 0.0–23.8%)] and a longer length of stay [26.0 days (IQR 12.5–34.0)], compared to non-intubated patients [0.0% (IQR 0.0–4.0%)], [10.0 days (IQR 6.0–14.0)] (p < 0.05) respectively. The main indications for intubation were to facilitate operative and dressing management (47.4%) followed by airway involvement (26.3%). FNE was performed on 32 patients (71.1%), however FNE findings did not significantly influence intubation rates.ConclusionMore than half (n = 20, 57.1%) of the 35 patients diagnosed with TEN underwent intubation, mainly to facilitate operative and dressing management. FNE was performed on most patients, however there was no clear association between FNE findings and early intubation.     

Laparoscopic pancreatic cystogastrostomy

The purpose of the review was to evaluate the feasibility and outcome of laparoscopic pancreatic cystogastrostomy for operative drainage of symptomatic pancreatic pseudocysts. A retrospective review of all patients who underwent laparoscopic pancreatic cystogastrostomy between June 1997 and July 2001 was performed. Data regarding etiology of pancreatitis, size of pseudocyst, operative time, complications, and pseudocyst recurrence were collected and reported as median values with ranges. Laparoscopic pancreatic cystogastrostomy was attempted in 6 patients. Pseudocyst etiology included gallstone pancreatitis (3), alcohol-induced pancreatitis (2), and post-ERCP pancreatitis (1). The cystogastrostomy was successfully performed laparoscopically in 5 of 6 patients. However, the procedure was converted to open after creation of the cystgastrostomy in 1 of these patients. There were no complications in the cases completed laparoscopically and no deaths in the entire group. No pseudocyst recurrences were observed with a median followup of 44 months (range 4-59 months). Laparoscopic pancreatic cystgastrostomy is a feasible surgical treatment of pancreatic pseudocysts with a resultant low pseudocyst recurrence rate, length of stay, and low morbidity and mortality.     

胰腺假性囊肿诊治体会

回顾性分析近１５年来对２３例胰腺假性囊肿的治疗情况。保守治愈５例,均为近期患急性胰腺炎者;手术治疗１８例,其中外引流１例,内外流１４例,囊肿切除术３例。结果 无手术死亡,外引流术后胰瘘１例,囊肿切除术后复发１例,内引流术后无严重并发症出现。认为急性囊肿应观察６周,有些病例有自行消散的可能,慢性囊肿一经确诊即行内流引治疗,内引流是目前较理想的有效手术方式。     

Intense Pulsed Light Treatment of Persistent Facial Hypermelanosis Following Drug-Induced Toxic Epidermal Necrolysis

Background. Cutaneous hyperpigmentation is one of the most cosmetically disturbing sequel of drug-induced toxic epidermal necrolysis. Intense pulsed light is a promising tool for treating some melanocytic lesions.
Objective. The objective was to assess the effect of intense pulsed light in treating post-toxic epidermal necrolysis facial hypermelanosis.
Methods. Two Caucasian men aged 35 and 50 years presented with long-standing (32 and 39 years) severe hypermelanosis of the face after sulfonamide-induced toxic epidermal necrolysis. They were treated by intense pulsed light. Cutoff filters of 550, 590, and 615 nm were employed for five intense pulsed light sessions at 4-week intervals. The treatment was characterized by energy fluence of 25 to 32 J/cm ² , pulse width of 2.2 to 3.2 ms, and double- to triple-pulse mode respecting a 30-ms delay. Before intense pulsed light treatment, and 2 months after the fifth intense pulsed light session, clinical photographs and skin biopsies were performed in combination with quantitative narrow-band remittance spectrophotometry of melanin pigmentation. Patients were clinically followed-up for 8 months after the end of the treatment.
Results. In both patients, clinical, histologic, and spectrophotometric assessments showed an average of 80% decrease in the hypermelanosis. No clinical recurrence of the hypermelanosis developed during the 8-month follow-up after intense pulsed light treatment. No major persistent side effects were experienced, especially hypopigmentation.
Conclusion. Intense pulsed light appears to be effective and safe for treating post-toxic epidermal necrolysis hypermelanosis in Caucasian patients.     

Pancreatogastrostomy: a safe drainage procedure after pancreatoduodenectomy

The purpose of this study was to evaluate the role of pancreaticogastrostomy as an alternative method of restoring pancreaticointestinal continuity after pancreaticoduodenectomy. Since 1975, 45 patients have undergone pancreaticogastrostomy after pancreaticoduodenectomy at our institution. Pancreaticoduodenectomy was performed for pancreatic carcinoma (24 patients), ampullary carcinoma (8 patients), duodenal carcinoma (4 patients), common bile duct carcinoma (4 patients), pancreatic islet cell carcinoma (1 patient), trauma (1 patient), extensive colon carcinoma (1 patient), chronic pancreatitis (1 patient), and gastroduodenal artery aneurysm (1 patient). There was one operative death, for an overall operative mortality rate of 2%, and seven patients had major postoperative complications, for an overall morbidity rate of 15%. No pancreatic anastomotic leaks or other complications related to the pancreaticogastrostomy occurred. Twenty-four patients have died of recurrent carcinoma, with a mean survival of 25 months (range, 5 to 66 months), and 20 patients are alive and well, with a mean follow-up of 27 months (range, 2 to 106 months). Eight of these patients are alive 2 or more years after operation and four do not have exocrine pancreatic insufficiency. This experience confirms that pancreaticogastrostomy is a safe method of pancreatic drainage after pancreaticoduodenectomy and suggests that it may have technical advantages and therefore merits more widespread application.     

Emergency laparotomy in patients with AIDS.

The presentation, operative management and final diagnosis were reviewed in 28 patients with AIDS (27 men and one woman) who underwent emergency laparotomy. On clinical and radiological examination, six patients showed features of toxic megacolon, five patients had small bowel obstruction, six patients had localized peritonitis and three had perforated viscus with generalized peritonitis. The most common disease processes were acute colitis in seven patients (associated with cytomegalovirus (CMV) infection in six), intra-abdominal lymphoma in five patients, acute appendicitis in five patients (associated with CMV infection in two), and atypical mycobacterial (MAI) infection in four patients. Two perioperative deaths occurred; one in a patient with acute pancreatitis and a second with generalized peritonitis. Later deaths were due to progression of AIDS, and patient survival at 1 month, 3 months and 6 months was 89 per cent, 64 per cent and 48 per cent, respectively. Lower operative mortality than in previously reported series may be due to earlier intervention in CMV toxic megacolon. Surgery, however, conferred less benefit in patients with acute abdominal pain from MAI infection or lymphoma. With careful patient selection, emergency laparotomy may achieve worthwhile palliation in patients with AIDS.     

Treatment of toxic epidermal necrolysis and a review of six cases

Six cases of drug-induced toxic epidermal necrolysis treated in a burns unit are presented. The mean skin loss was 67.3 per cent of the total body surface area. Two patients developed renal failure and two had ocular symptoms. The mortality rate was 50 per cent, with two patients dying from septicaemia and one from respiratory and renal failure. The diagnosis of toxic epidermal necrolysis can be confirmed by skin biopsy. We recommend that this disease is treated in a burns unit so that both adequate wound care and essential intensive supportive treatment can be given. Antibiotics are indicated only for specific infections such as septicaemia or pneumonia. Steroids have been shown to increase greatly the mortality from septic complications and are not recommended. The mortality ranges from 10 per cent to 70 per cent and bad prognostic factors include increasing age, greater than 50 per cent of body surface skin loss and neutropenia.     

Risk of gallstone-related complications in necrotizing pancreatitis patients treated with a step-up approach: The experience of two tertiary care centers

BackgroundA minimally invasive step-up approach to necrotizing biliary pancreatitis often requires multiple interventions, delaying cholecystectomy. The risk of gallstone-related complications during this time interval is unknown, as is the feasibility and safety of cholecystectomy after minimally invasive step-up treatment. In this paper, we analyzed both.MethodsNecrotizing pancreatitis patients treated with a minimally invasive step-up approach who underwent interval cholecystectomy at 2 tertiary care centers between 2014 and 2019 were included. Gallstone-related complications prior to cholecystectomy were examined, as were surgical approaches to cholecystectomy and complications. Necrotizing pancreatitis patients treated without mechanical intervention were also examined.ResultsSeven of 31 patients developed gallstone-related complications between minimally invasive step-up treatment initiation and cholecystectomy. One patient developed biliary colic. Six patients developed acute cholecystitis. Two of these patients also developed choledocholithiasis, and 1 developed cholangitis, all requiring endoscopic retrograde cholangiopancreatography. Cholecystectomy was performed laparoscopically in 27 of 31 patients. One patient required open conversion, and 3 patients underwent planned cholecystectomy during another open operation. Four patients developed postoperative complications. Two of 14 necrotizing pancreatitis patients treated without mechanical intervention developed recurrent pancreatitis while awaiting cholecystectomy.ConclusionOver 20% of necrotizing pancreatitis patients treated by a minimally invasive step-up approach developed gallstone-related complications while awaiting cholecystectomy. Laparoscopic cholecystectomy is feasible and safe in the great majority of necrotizing pancreatitis patients treated by a minimally invasive step-up approach.     

The complications of pancreatectomy.

This paper analyses the early postoperative complications after 285 pancreaticoduodenectomies performed during the past 15 years in the Surgical University Clinic, Mannheim. There were 235 partial (Whipple) and 52 total pancreatectomies performed for pancreatic and periampullary tumors (181 patients) and complicated chronic pancreatitis (104 patients). A total of 92 complications requiring relaparotomy in 42 patients ended fatally in nine patients. The overall operative and hospital mortality rate was 3.1%. The most frequent and most dangerous were complications at or around the pancreaticojejunal anastomosis, which occurred 25 times with five deaths. Postoperative hemorrhage was seen in 16 patients; endoscopic treatment in four patients and operation in 12 patients was successful in stopping the bleeding in all but one patient. Eight biliary fistulae either ceased spontaneously (3 patients) or after operative reintervention (5 patients) without any mortality. Control of these complications depends on four lines of approach: (1) before operation: optimal preparation of the jaundiced patient including endoscopic transpapillary decompression of the common duct; (2) during operation: a meticulous and standardized technique is mandatory; (3) after operation: continuous observation in the surgical intensive care unit is essential for the timely detection of possible complications; and (4) early reintervention can salvage the great majority of these patients with deleterious complications.     

Extensive colonic necrosis complicating acute pancreatitis

Segmental ischemic gangrene of the colon is a rare complication of acute pancreatitis. Three patients with long-segment colonic necrosis complicating acute pancreatitis are reported. At operation all three patients had extensive retromesocolic necrolysis associated with colonic arterial thrombosis. Colonic resection and intestinal exteriorization with peripancreatic drainage were performed, with survival of two patients. Although diagnosis of colonic ischemia in the presence of acute pancreatitis is difficult, operative therapy affords reasonable cure when this severe problem is recognized.