Table of Contents  
CASE REPORT
Year : 2017  |  Volume : 10  |  Issue : 1  |  Page : 64-66  

A rare case of Stevens- Jonhson Syndrome with attention deficit hyperkinetic disorder and seizure disorder for circumcision: An anesthetic challenge


Department of Anaesthesiology, Dr. D. Y. Patil Medical College, Hospital and Research Centre, Dr. D. Y. Patil Vidyapeeth, Pune, Maharashtra, India

Date of Web Publication9-Jan-2017

Correspondence Address:
Dr. Deepasri Chowdhury
Department of Anesthesiology, Dr. D. Y. Patil Medical College, Hospital and Research Centre, Pimpri, Pune - 411 018, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0975-2870.197900

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  Abstract 

Stevens–Johnson syndrome (SJS) is an acute reaction to medications. It is a severe form of erythema multiforme. It is characterized with vesiculobullous eroded lesions of the skin and mucous membrane. Anesthetic management pertains to the drugs that can be used for anesthesia and preventing injury of oropharynx and larynx due to lesions. We report a rare case of SJS with attention deficit hyperkinetic disorder and seizure disorder for circumcision.

Keywords: Attention deficit hyperkinetic disorder, seizure disorder, Stevens–Johnson syndrome


How to cite this article:
Chowdhury D, Khatavkar S, Samuel M. A rare case of Stevens- Jonhson Syndrome with attention deficit hyperkinetic disorder and seizure disorder for circumcision: An anesthetic challenge. Med J DY Patil Univ 2017;10:64-6

How to cite this URL:
Chowdhury D, Khatavkar S, Samuel M. A rare case of Stevens- Jonhson Syndrome with attention deficit hyperkinetic disorder and seizure disorder for circumcision: An anesthetic challenge. Med J DY Patil Univ [serial online] 2017 [cited 2024 Mar 28];10:64-6. Available from: https://journals.lww.com/mjdy/pages/default.aspx/text.asp?2017/10/1/64/197900


  Introduction Top


Stevens–Johnson syndrome (SJS) is an uncommon acute exfoliating disease causing involvement of the skin and mucous membrane. Widespread, often confluent purpuric macules (spots) or atypical flat targets predominantly on the trunk are the cutaneous patterns in SJS.[1] It is a blistering disorder that is usually more severe than erythema multiforme. Initial presentation is often sore throat, malaise, fever, erosions, small blisters that develop on dusky, purpuric maculae, or atypical target lesions.[2] It can be life threatening in the acute phase. For an anesthetist, the management of airway, fluids, venous access, and anesthetic agents to be used for induction and pain management present as challenges.[3] Treatment is based on nonspecific and symptomatic means. The latter is most important for patients with large amount of skin detachment requiring intensive care in specialized units. Furthermore, sequelae such as strictures of mucous membranes and symblepharon, which may lead to long-lasting impairment, should be prevented.

Attention deficit hyperactivity disorder (ADHD) is a developmental condition of inattention and distractibility, with or without accompanying hyperactivity.[4] ADHD can appear as early as the age of three and can continue till adulthood in some cases. Seizure disorders are characterized by episodes of uncontrolled electrical activity in the brain. Seizure disorders can be hereditary, or they can be caused by environmental factors.


  Case Report Top


A 7-year-old male child of 25 kg with a history of fever with chills for 5 days was treated in a secondary care center with tablet chloroquine and tablet paracetamol orally before reporting to our hospital. The mother (informant) said that he developed multiple hyperpigmented lesions over the face, forearm, abdomen, back, eyes, and genitals after taking two doses of the medications and thereafter the medications were immediately stopped. The relatives then took discharge against medical advice and came to our hospital where he was directly admitted into the Pediatric Intensive Care Unit (PICU). Initially, the eruptions were small pinpoint and contained white fluid. These later increased in size and number. The patient developed multiple ulcers in the oral mucosa and lips. The patient was started on injection ceftriaxone, injection linezolid both intravenously and supportive measures such as injection wysolone intravenous and intravenous fluids. No reaction was seen.

On taking detailed history, it was found that the patient was a known case of seizure disorder since 2 years of age and was on oral tablet sodium valproate. He had two episodes of generalized tonic–clonic seizures in the past 10 days. Birth history of the child revealed that he was born by full-term lower section cesarean section for breech presentation followed by a Neonatal Intensive Care Unit stay for neonatal jaundice. He had a history of delayed milestones and even at 7 years would only speak by syllables. For his hyperactive behavior and low attention span, the child was taken to a psychiatrist 2 years ago where he was diagnosed to have ADHD.

Considering the above history, a diagnosis of SJS with seizure disorder and ADHD was made. The patient was kept in the PICU for 15 days and was treated with adequate hydration, syrup levetiracetam, syrup cyclosporin orally, and topical ophthalmic eye drops. Soon after the acute phase of SJS had subsided, he was posted for circumcision to treat paraphimosis that had developed while in the PICU.

All routine investigations were within normal limits except for electroencephalograph that showed epileptic foci at the frontotemporal region.

The patient was given fitness under the American Society of Anaesthetists Grade III and posted for emergency circumcision in view of paraphimosis.

In the operating theater, monitors were attached carefully over the skin that did not have areas of sloughing and blisters. The patient was premedicated with injection glycopyrrolate 0.1 mg intravenously, injection midazolam 0.5 mg intravenously, injection ondansetron 2.5 mg intravenously, injection hydrocortisone 40 mg intravenously, and injection fentanyl 30 mcg given intravenously. The patient was preoxygenated for three minutes with 100% oxygen by face mask. Induction was done with injection propofol 60 mg intravenously. Thereafter, injection suxamethonium 50 mg was given intravenously, and the patient was intubated gently with 5.5 uncuffed endotracheal tube avoiding trauma to the delicate mucosa of the airway. Air entry was checked bilaterally, and the patient was maintained on oxygen: Nitrous oxide (50%:50%), sevoflurane (0.8%–1%), and injection atracurium 12.5 mg intravenously. Intraoperatively [Figure 1], patient's hemodynamic parameters were within normal limits. Reversal was done with injection neostigmine 1 mg intravenously and injection glycopyrrolate 0.2 mg intravenously after respiratory efforts were noted. Extubation was done gently. Postoperative vitals were stable, and protective reflexes were present. The patient was conscious and responding to verbal commands. Oxygen saturation was 100% on room air.
Figure 1: Original picture of the patient intraoperatively

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  Discussion Top


SJS is an acute disease of the skin and mucous membranes characterized by skin lesions which have the tendency for recurrence and show symmetrical extension, especially in extremities.[3] It is a rare disorder with an incidence rate of 1%–6%,[5] and the mortality is 3–18%.[6] Less than 10% of the skin in SJS is inflamed with macules, papules, vesicles, bullae, and lesions may occur throughout the body with involvement of the eyes, skin, gut, and kidneys.[7]

Drugs are an important cause of SJS, but infections or a combination of infections and drugs has also been implicated.[8] More than 100 drugs have been implicated; however, most commonly used drugs such as sulfonamides, anticonvulsant agents, allopurinol, cyclooxygenase inhibitors, barbiturates, and sedatives are commonly associated with this condition. Whether nonsteroidal anti-inflammatory drugs, analgesic agents, and nonsulfonamide antibiotics are associated with it or not is controversial.[9] Drugs commonly used in anesthesia such as glycopyrrolate, midazolam, ondansetron, fentanyl, and neostigmine have rarely been implicated in SJS.

Our patient was on sodium valproate. This drug has been associated with SJS but to a lesser extent as compared to other antiepileptogens.[10] Pathophysiology of the disease is said to be by the activation of CD8+ T-cells that produce a cytolytic protein granulysin which causes the epidermal destruction. The severity of the disease is also correlated with the concentration of granulysin found in the blister fluid.[11]

Anesthetic implication of SJS includes difficult orotracheal intubation due to oral mucosal erosions, difficult monitoring because of skin lesions, pleural blebs which could rupture and lead to pneumothorax, myocarditis, and pericarditis. Temperature monitoring is needed during episodes of fever. Patients of SJS are also more prone to Raynaud's phenomena.[12] Supraglottic airway devices such as laryngeal mask airway (LMA) could have been used, but we felt placement of an LMA could be more traumatic to the delicate oral mucosa, especially for a prolonged period. Airway edema also presents as a problem. Prompt diagnosis, identification, and early withdrawal of all suspect drugs are the most important preliminaries in the management of such patients. Patient care must be undertaken in specialized intensive care units, with the same main types of therapy as for burns: Warming of the environment, correction of electrolyte disturbances, administration of a high caloric enteral intake, and prevention of sepsis. Efficacy of drugs used in some case reports is difficult to evaluate: Intravenous immunoglobulins, cyclosporin, cyclophosphamide, pentoxifylline, and thalidomide have all been tried.[9]

To summarize, SJS is a rare clinical entity that poses a dilemma for the anesthetist. The anesthetic complications faced in our case was the choice of analgesic drugs and induction agents as the patient had history of seizures, airway manipulation due to ulcers on the mucous membrane and raw skin lesions that made intravenous access, and monitor placement difficult. Keeping the above factors in mind, the patient was managed successfully with no adverse sequelae.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Bastuji-Garin S, Rzany B, Stern RS, Shear NH, Naldi L, Roujeau JC. Clinical classification of cases of toxic epidermal necrolysis, Stevens-Johnson syndrome, and erythema multiforme. Arch Dermatol 1993;129:92-6.  Back to cited text no. 1
    
2.
Robert S, Oliver MC, Bruce UW. Cutaneous drug reaction. In: Harrison's Principles of Internal Medicine. 15th ed. New Delhi: McGraw Hill Education Publishers; 2003. p. 336-9.  Back to cited text no. 2
    
3.
Kalhan SB, Ditto SR. Anesthetic management of a child with Stevens-Johnson syndrome. Cleve Clin J Med 1988;55:467-9.  Back to cited text no. 3
    
4.
Moffitt TE, Houts R, Asherson P, Belsky DW, Corcoran DL, Hammerle M, et al. Is adult ADHD a childhood-onset neurodevelopmental disorder? Evidence from a four-decade longitudinal cohort study. Am J Psychiatry 2015;172:967-77.  Back to cited text no. 4
    
5.
Roujeau JC, Kelly JP, Naldi L, Rzany B, Stern RS, Anderson T, et al. Medication use and the risk of Stevens-Johnson syndrome or toxic epidermal necrolysis. N Engl J Med 1995;333:1600-7.  Back to cited text no. 5
    
6.
Ghislain PD, Roujeau JC. Treatment of severe drug reactions: Stevens-Johnson syndrome, toxic epidermal necrolysis and hypersensitivity syndrome. Dermatol Online J 2002;8:5.  Back to cited text no. 6
    
7.
Smith GB, Shribman AJ. Anaesthesia and severe skin disease. Anaesthesia 1984;39:443-55.  Back to cited text no. 7
    
8.
Kim HI, Kim SW, Park GY, Kwon EG, Kim HH, Jeong JY, et al. Causes and treatment outcomes of Stevens-Johnson syndrome and toxic epidermal necrolysis in 82 adult patients. Korean J Intern Med 2012;27:203-10.  Back to cited text no. 8
    
9.
Ferrell PB Jr., McLeod HL. Carbamazepine, HLA-B*1502 and risk of Stevens-Johnson syndrome and toxic epidermal necrolysis: US FDA recommendations. Pharmacogenomics 2008;9:1543-6.  Back to cited text no. 9
    
10.
Naveen K, Arunkumar J, Hanumanthayya K, Pai V. Stevens-Johnson syndrome induced by sodium valproate monotherapy. Int J Crit Illn Inj Sci 2012;2:44-5.  Back to cited text no. 10
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11.
Mockenhaupt M. The current understanding of Stevens-Johnson syndrome and toxic epidermal necrolysis. Expert Rev Clin Immunol 2011;7:803-13.  Back to cited text no. 11
    
12.
Garg R, Khanna P, Sinha R. Perioperative management of patients for osteo-odonto-kreatoprosthesis under general anaesthesia: A retrospective study. Indian J Anaesth 2011;55:271-3.  Back to cited text no. 12
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