Anesthetic considerations in a patient with Cornelia ...

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2 blade) it was possible to see aditus laryngis (Cormack. Lehane score 3). Intubation was done in first attempt using.
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use  of  electrocautery  in  a  gastric  banding  gastroplasty  by  laparoscopic  approach.[3]  Perzanowki  has  reported  sudden  onset VF during use of unipolar electrocautery near angle of  His during a gastric bypass surgery.[4] Yan et al. have reported  the occurrence of VF following application of electrocautery  to  left  diaphragm  during  a  laparoscopic  subphrenic  mass  resection and suggested capacitive coupling to be involved in  generation of a low frequency current.[1] This case highlights the possible complication of intra-operative  VF with the use of electrocautery during liver resection. Such  accidents can be prevented using argon or fibrin glue to control  the bleeding from the raw surface of liver. Their limitation  though is high cost. In our experience of more than 150 cases  of liver resections in last 8 years this is the first such event.  Since then we are using electrocautery at a much lower energy,  i.e., 20 W as a preventive measure.  Strict vigilance, continuous alert monitoring and availability of  advanced resuscitation equipments should be ensured during  liver resections. Akhil Agarwal, Rajeev Lochan Tiwari,  Sundeep Jain1 Departments of Anaesthesia and 1Gastrointestinal, HPB and Bariatric  Surgery, Fortis Escorts Hospital, Jaipur, Rajasthan, India Address for correspondence: Dr. Akhil Agarwal,  G-73, Shyam Nagar Extension, Jaipur - 302 019,  Rajasthan, India.  E-mail: [email protected]

References 1. 

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Yan  CY,  Cai  XJ,  Wang  YF,  Yu  H.  Ventricular  fibrillation  caused  by  electrocoagulation  in  monopolar  mode  during  laparoscopic  subphrenic mass resection. Surg Endosc 2011; 25:309-11. Fu Q, Cao P, Mi WD, Zhang H. Ventricular fibrillation caused by  electrocoagulation  during  thoracic  surgery.  Acta  Anaesthesiol  Scand 2010; 54:256. Klop WM, Lohuis PJ, Strating RP, Mulder W. Ventricular fibrillation  caused  by  electrocoagulation  during  laparoscopic  surgery.  Surg  Endosc 2002; 16:362. Perzanowski C. Ventricular fibrillation resulting from diaphragmatic  stimulation  during  gastric  bypass  surgery.  Obes  Facts  2012;  5:  648-50.

Anesthetic considerations  in a patient with Cornelia  de-Lange syndrome Cornelia  de-Lange  syndrome  (CdLS)  is  a  relatively  rare,  genetically  heterogeneous,  and  sporadic  disease.[1]  There  is  an  estimated  prevalence  of  1  in  10,000  to  1  in  30,000.  In  Denmark,  there  are  65  persons  known  with  CdLS (www.cdl.dk/kort_om_cdl.html). Two-third of CdLS  individuals die within their 1st year of living, primarily due to  aspiration pneumonia. Later in life mortality is mostly due to  infections and bowel obstruction. The syndrome typically has  clinical  manifestations  of  primordial  growth  failure,  mental  retardation,  neurosensory,  musculoskeletal,  craniofacial,  cardiopulmonary, and gastrointestinal abnormalities.[2] A  49-year-old  woman,  height  140  cm  and  weight  24  kg  [Figure  1],  diagnosed  with  CdLS  was  hospitalized  with  difficulty  in  swallowing  and  with  severe  dehydration.  She  was  posted  for  gastroscopy  under  general  anesthesia.  She  was mentally retarded with only high-pitched growling cries,  almost blind and deaf, growth retarded with craniofacial and  abnormal  extremities.  As  newborn,  she  had  a  ventricular  septal  defect  with  spontaneous  closure  and  suffered  from  repeated pneumonias. Presently she does not have any signs  or symptoms of epilepsy or cardiopulmonary compromise. Preanesthetic checkup was challenging because the patient  is mentally retarded and nonco-operative. Adequate airway  assessment  was  not  possible  despite  anticipating  a  difficult  airway with high risk of aspiration. Based on medical history,  talking  to  her  caretaker  and  considering  previous  CdLS  case  reports,  a  basic  anesthetic  plan  was  made.  With  the 

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DOI:   10.4103/0970-9185.161729

Figure 1: This is a photo of a 49-year-old Cornelia de-Lange syndrome patient.  It  is  easy  to  see  the  external  abnormal  anatomy.  It  was  impossible  to  see  the  mouth-pharyngeal and -laryngeal anatomy before she was anesthetized. Consent  to use photo given by Bente Mills (Caretaker)

Journal of Anaesthesiology Clinical Pharmacology | July-September 2015 | Vol 31 | Issue 3

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patient  lying  in  the  lateral  position,  we  monitored  with  pulse-oximetry, electrocardiography, and noninvasive blood  pressure.  Awake  fiberoptic  intubation  was  impossible  due  to  the  lacking  cooperation.  Anticipating  regurgitation,  hypoplastic  larynx,  and  difficult  airway,  we  kept  suction,  difficult  intubation  trolley,  various  sizes  of  laryngoscope  blades, and endotracheal tubes ready. She was induced using  sevoflurane  and  kept  on  spontaneous  respiration.  Mouth  and  oro-pharynx  was  sprayed  with  lidocaine  spray  (1%)  3  times.  With  laryngoscope  (MacIntosh  laryngoscope  size  2  blade)  it  was  possible  to  see  aditus  laryngis  (Cormack  Lehane score 3). Intubation was done in first attempt using  an endotracheal tube stylet. After intubation the patient was  maintained on sevoflurane, remifentanil infusion, and volume- controlled  ventilation.  Gastroscopy  was  impossible  due  to  oropharyngeal abnormalities. The awakening was prolonged  with periods of insufficient spontaneous respiration. With the  use of capnography, pulse-oximetry and tidal volume she was  extubated uneventfully (still sleeping) after 1 h, observed in  the postoperative care unit and after 4 h discharged to the  general ward without any adverse events. At the postoperative  care unit she was shielded, observed with pulse-oxymetry and  noninvasive blood pressure. There was a suction device ready  in case of aspiration. Her caretaker was present during the  entire period except when she was fully anesthetized. Cornelia de-Lange syndrome is a rare disease. Anesthesiologist  encountering such patients is rare. When encountered, such  patients are challenging.[2] Anticipated difficult airway, use  of  awake  fiberoptic  intubation,  hyperactive  airway,  risk  of  aspiration, convulsion, cardiac arrhythmias, prolonged apnea  with the use of suxamethonium, malignant hyperthermia with  use of halothane and nitrous oxide, and prolonged awakening  are well-described.[1,2] Drugs such as sevoflurane, isoflurane,  ketamine, etomidate, and narcotics as induction agents with  dose adjustments after renal function are suggested.[1,2] Background  knowledge  of  such  a  syndrome,  considering  patient’s  past  and  present  medical  history  and  getting  information from the patient’s caretaker is important. Induction  with sevoflurane using lidocaine spray for the airways with the  patient on spontaneous respiration eases laryngoscopy and  intubation. It is also the safest way of induction in an unknown  airway anatomy with a nonco-operative and agitated patient.  It is suggested to use stomach acid neutralizing drugs such  as Sodium citrate if possible. Use of safer anesthetic drugs,  reducing procedural and or anesthesia time with anticipation  of  potential  complications  and  adequate  precautions  will  definitely have favorable anesthetic outcomes. Awareness of  the importance of keeping the patient asleep during extubating  will minimize the risk of agitation and complications in the  post  anesthetic  process.  It  is  also  recommended  and  well- 420

described[3] that there should be, for the patient, a well-known  person at all time to minimize agitation and misconceptions. Lars Vestergaard, Nilanjan Dey, Robert Winding Department of Anesthesia, Hospital Unit Vest,   Region of Midtjylland, Denmark Address for correspondence: Dr. Lars Vestergaard,   Department of Anesthesia, Hospital Unit Vest,   Region of Midtjylland, Denmark.   E-mail: [email protected]

References 1. 

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Washington  V,  Kaye  AD.  Anesthetic  management  in  a  patient  with  Cornelia  de  Lange  syndrome.  Middle  East  J  Anesthesiol  2010;20:773-8. Takeshita  T,  Akita  S,  Kawahara  M.  Anesthetic  management  of  a  patient  with  Cornelia  De  Lange  syndrome.  Anesth  Prog  1987;34:63-5. Shulman SM, Polepalle S. Spinal anesthesia for a deaf-mute patient  undergoing prostatic surgery. Anesthesiology 1993;78:590-1. Access this article online Quick Response Code:

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DOI:   10.4103/0970-9185.161732

Anesthetic management  of insulinoma Sir,

Insulinoma  is  a  rare  tumor  of  beta  pancreatic  cells  with  an  incidence  of  1-4/million.[1]  Whipple’s  triad,  which  is  pathognomic of insulinoma, consists of recurrent hypoglycemic  symptoms, plasma glucose 