Pediatric Anesthesia and Critical Care Journal 2014; 2(1):48-51 doi:10.14587/paccj.2014.12

Sevoflurane in child with carpus callosum agenesis syndrome, a series of four cases

N. Kumar

Consultant, Department of Anesthesia and Intensive Care, Naryana Nethralaya Eye Hospital, Narayana Hrudalaya Campus, Bangalore, India.

Corresponding author: N. Kumar, Consultant, Department of Anesthesia and Intensive Care, Naryana Nethralaya Eye Ho- spital, Narayana Hrudalaya Campus, Bangalore, India. Email: [email protected]

Key points

The clinical features most commonly seen in patients with agenesis of the carpus callosum syndrome are microcepha- ly, , seizures, developmental delay or retardation, hypotonia, spasticity and multiple somatic anomalies including arthrogryposis, eye anomalies, in the and facial dysmorphisms. Main issues of anaesthetic concerns are difficult airway, altered respiratory mechanics, myopathy, gastric reflux,interaction of poly- pharma therapy for seizures and anaesthetic agents, associated cardiovascular disorders. The response to general anae- sthesia with sevoflurane and muscle relaxants and its postoperative outcomes were observed in these unreported cases.

Abstract me, Shapiro syndrome, acrocallosal syndrome, septo- Agenesis of the (ACC) occurs when optic dysplasia (optic nerve hypoplasia), Mowat-Wilson the band of tissue connecting the two hemispheres of the syndrome and Menkes syndrome4. We report four cases brain does not develop typically in utero. It has been of carpus callosum agenesis syndrome undergoing se- correlated with inherited genetic factors, prenatal infec- ven procedures under general anaesthesia with sevoflu- tion or injuries, prenatal toxic exposures, structural rane. Common issues of anaesthetic concerns are diffi- blockage by cysts or other brain abnormalities, metabo- cult airways, altered respiratory mechanics, myopathy, lic disorders fetal alcohol syndrome, craniofacial ab- gastric reflux,interaction of polypharma therapy for sei- normalities, and chromosome errors such as unequal zures and anaesthetic agents, associated cardiovascular chromosome x inactivation or deletion of the short arm disorders,response to general anaesthesia with sevoflu- of Chromosome 11. It is diagnosed sometimes acciden- rane and muscle relaxants and its postoperative outco- tally presenting with a broad range of clinical manifesta- mes were observed. tions and no unique prognosis.2 The clinical features Keywords: Sevoflurane, carpus callosum agenesis syn- most commonly seen in patients with agenesis of the drome, difficult intubation, pediatric. corpus callosum or hypoplasia of the corpus callosum Case reports are , macrocephaly, seizures, developmen- Case 1. One and half month old female baby weighing tal delay or retardation, hypotonia, spasticity and multi- 3kg was posted for trabeculectomy and trabaculotomy ple somatic anomalies including arthrogryposis, eye under general anaesthesia. Patient was born at full term anomalies, syringomyelia in the spinal cord and facial with normal delivery, cried immediately after birth wei- dysmorphisms.3 Some syndromes that frequently inclu- ghing 2.5 kg. Patient had facial dysmorphism, microce- de ACC are Aicardi syndrome, Andermann syndro- phaly, craniosynostosis, congenital bupthalmos of left

Kumar. Sevoflurane and carpus callosum agenesis 48

Pediatric Anesthesia and Critical Care Journal 2014; 2(1):48-51 doi:10.14587/paccj.2014.12 eye, right sided facial palsy, micrognathia, low set ears, she was on antibiotics. Patient had uncontrolled seizures sacral hairs and global developmental delay. Complete and was on long term multiple antiepileptic (sodium hemogram, liver and renal profile was normal. ECHO valproate,liveracetam and vigabatrin) drugs with maxi- showed normal cardiac function. The sonography of ab- mum dose. Sodium valproate level was normal. Patient domen showed mild pelvicalyceal fullness across left was posted for electroretinography to rule out vigaba- kidney. MRI of brain showed complete carpus callosum trin toxicity under general anaesthesia. The procedure agenesis and colpocephaly. The procedure took about took about 60 minutes. 130 minutes. Case 4. 10yr old female weighing 15 kg was posted for Case 2. Ten months old female baby weighing 9kg was bilateral cataract extraction under general anaesthesia at posted for bilateral cataract under general anaesthesia at a week interval. Patient was born at full term with nor- a week interval. Patient was born at full term with nor- mal delivery, cried immediately after birth. Patient had mal delivery, cried immediately after birth weighing 3 severe intentional tremors, global developmental delay, kg. Patient had bilateral cataract, central hypotonia, glo- spastic paraperesis, ataxia without seizures. Complete bal developmental delay and lactose intolerance .There hemogram, renal and liver profile was normal. Cardiac was a recent history of staphylococcal skin scalded syn- function was normal. MRI of brain shows diffuse hy- drome three months back before surgery. Complete he- pomyelination of white matter of both cerebrum and ce- mogram, liver and renal profile was normal. Ig G anti- rebellum, anterior and posterior limbs of bilateral inter- bodies for cytomegalovirus and rubella were strongly nal capsule and hypoplastic carpus callosum. Visual positive. The karyotype was normal. ECHO showed evoked potential, Somatosensory evoked potential and normal cardiac function. MRI of brain showed hypo- brainstem evoked potential was normal. The procedure plastic carpus callosum with mild . MRI took about 90-100 minutes e for each eye. of spinal cord showed syringomyelia in cervical and All patients were shifted to operation theatre with stan- dorsal spinal cord sparing a small portion of upper dor- dard fasting protocol. All antiepileptic were continued sal cord. Procedure took around 100 minutes. on morning of the surgery. All patients were accompa- Case 3. Three year old female weighing 20kg was po- nied with their parents into the operation theatre to re- sted for electroretinography under general anaesthesia. duce the anxiety. Parameters such as electrocardigraphy, Patient was born at full term with normal delivery, cried non invasive blood pressure, saturation and temperature immediately after birth weighing 3.3 kg. Patient had probes were applied. Inhalation induction was done with seizures, myoclonic jerks, macrocephaly, quadriparesis, sevoflurane in graded incremental manner upto 8 vol % global developmental delay and symptomatic west syn- via facemask with 100% oxygen, while monitoring the drome. Complete hemogram, renal and liver profile was hemodynamic parameters. Intravenous line was secured normal. MRI of brain showed complete carpus callo- in all the patients except in three year old child requiring sum agenesis, colpocephaly, fusion of bilateral thalami. multiple attempts. Intravenous glycopyrrolate 0.08 Urine amino acid negative and ketones negative. EEG mg/kg, fentanyl 1.5 mcg/kg was given. Intubation was showed lateralized seizures from left hemisphere. Vi- visualized and intubated with appropriate endotracheal sual evoked potential showed mild conduction block in tube without muscle relaxant in all patients except in left side. Gastoesophageal reflux disease was diagnosed one and half month baby where the malampatti score owing to repeated lower respiratory tract infections. Il- was 3. Succinylcholine 1 mg/kg was given in that pa- leostomy was done 2 month back. The last episode of tient and intubated with the help ofstylet. Ryles tubes of lower respiratory tract infection was one week ago and appropriate size was inserted and fixed through nasal

Kumar. Sevoflurane and carpus callosum agenesis 49

Pediatric Anesthesia and Critical Care Journal 2014; 2(1):48-51 doi:10.14587/paccj.2014.12 route in all patients, suctioned and connected to the sum or hypoplasia of the corpus callosum collector bag. All Vital parameters were monitorted and are microcephaly, macrocephaly, seizures, developmen- maintained throughout the procedure. Patients were ca- tal delay or retardation, hypotonia, spasticity and multi- refully positioned in supine position. All the pressure ple somatic anomalies including arthrogryposis, eye points were padded with cotton. Euthermia was main- anomalies, and facial dysmorphisms.3 Although only tained. Paracetomol suppository 15 mg/kg was inserted one quarter of these children will have midline facial to decrease the postoperative pain and pyrexia. Patients anomalies, this is the single most frequent category of were maintained with oxygen, nitrous oxid (1:1) and se- somatic malformation3. Colpocephaly is a cephalic di- voflurane in closed circuit. Extubation was done in fully sorder that refers to the disproportionate enlargement of awake condition. Propofol one mg/kg was given 5 mi- the occipital horns of the lateral ventricles and is usually nutes before extubaion in 3 year and 10 year child to diagnosed early after birth due to seizures. It is a non- avoid emergence delirium. All patients maintained satu- specific finding and is associated with multi- ration in the postroperative peroid. All were shifted to ple neurological syndromes, including agenesis of the pediatric intensive care unit for 24 hours. Ryles tube corpus callosum, , , was removed after 4 hours of extubation as oral fluids and microcephaly5. The neonate with agenesis of the was allowed. No adverse events were noted in the po- corpus callosum is most often recognized by identifica- stoperative period. All patients were discharged on third tion of the accompanying somatic or facial anomalies3. postoperative day. The slightly older infant may also be identified by ac- Discussion companying anomalies or during the evaluation of sei- Agenesis of the corpus callosum occurs when the band zures3. Both neonates and infants may have feeding dif- of tissue connecting the two hemispheres of the brain ficulties and swallowing apraxia. In children the usual does not develop typically in utero. It has been correla- clinical presentation is developmental delay, difficulty ted with inherited genetic factors, prenatal infection or in school, or seizures, and the agenesis of the corpus injuries, prenatal toxic exposures, structural blockage by callosum is identified incidentally as a result of the neu- cysts or other brain abnormalities, metabolic disorders roimaging studies performed as part of the evaluation3. and chromosome errors such as unequal chromosome x Agenesis of the corpus callosum is also seen as a feature inactivation or deletion of the short arm of Chromosome of many chromosomal and genetic mental retarda- 11. It is diagnosed sometimes accidentally presenting tion syndromes as well as several hereditary metabolic with a broad range of clinical manifestations and no diseases where the clinical presentation is dominated by unique prognosis.2 The clinical manifestations of ano- the primary disease3. Some syndromes that frequently malies involving the corpus callosum are more depen- include ACC are Aicardi syndrome, Andermann syn- dent on the degree of disturbance of development of the drome, Shapiro syndrome, acrocallosal syndro- brain in general and midline structures in particular than me, septo-optic dysplasia (optic nerve hypopla- the absence of the corpus callosum itself3. Focal cortical sia), Mowat-Wilson syndrome and Menkes syndrome. dysplasias such as zones of disorganized lamination oc- The main issues of anaesthetic concern were difficult cur and may be epileptogenic3. Furthermore, when airways, altered respiratory mechanics, myopathy, ga- the callosal defect is only part of a more pervasive di- stric reflux, interaction among polypharma therapy for sorder, the clinical picture is usually dominated by the seizures and anaesthetic agents, associated cardiovascu- primary condition3. The clinical features most com- lar disorders,response to general anaesthesia with sevo- monly seen in patients with agenesis of the corpus callo- flurane and muscle relaxants and its postoperative out-

Kumar. Sevoflurane and carpus callosum agenesis 50

Pediatric Anesthesia and Critical Care Journal 2014; 2(1):48-51 doi:10.14587/paccj.2014.12 come. These patients have hard of hearing, vision im- temperature, seizures was done in postoperative period pairment and impaired face processing for pain. So, the in the intensive care unit attender was accompanied with the child to reduce an- Conclusions xiety during induction and to avoid injury and assess Sevoflurane was safely used as an induction and main- pain in the post opearative period. Difficult intravenous tainence agent in patients with carpus callosum agenesis cannulation was anticipated and measures for central syndrome.Trachea was intubated without muscle rela- cannulation was considered as these patients will be on xant with sevoflurane and fentanyl combination in ma- multiple antiepileptic drugs. Fibre optic intubation and jority of cases (6/7cases). Carefull positioning, tempera- difficult intubation cart was always be made ready since ture monitoring, adequate ventillation, preventing aspi- these patients usually have micrognathia, short neck, ration by inserting ryles tube, assessing pain with hemo- and spasticity. Use of succinylcholine was avoided in dynamic parameters and parental presence and conti- view of myopathy. Use of non depolarizing muscle re- nuing all antiepileptic drugs in pre and post period was laxant was also avoided initially in view of difficult in- very helpful in successful management of cases with tubation. So it was better that intubate without muscle carpus callosum agenesis syndrome. relaxation. Use of fibre optic bronchoscopy was consi- References dered if found unsuccessfull. Inserting ryle tube after the 1. Njiokiktjien C. Absence of the corpus callosum: cli- intubation and keeping it throughout the procedure was nicopathological correlations In: Ramaekers G, very helpful to prevent aspiration as these patients have Njiokiktjien C, eds Pediatric Behavioral neurology vol. swallowing difficulties and gastric reflux. Carefull posi- 3: The child's corpus callosum. Amsterdam: Suyi Publi- tioning was done as these cases have cervical instability cations, 1991; 235-250. or torticollis, contractures, weakness/hypotonia, move- 2. Bedeschi MF, Bonaglia MK, Grasso R, Pellegri A, ment disorders and seizures. Care was taken to avoid Garghentino RR, Battaglia MA, Panarisi AM, Di Rocco hyperventilation/hypocarbia that leads to reduction of M, Ballotin U, Bresolin N, Bassi MT, Borgatti R. Age- CBF and worsening metab/EEG. Sevoflurane was used nesis of the corpus callosum: clinical and genetic study as both induction and maintainence as it is known for in 63 young patients. Pediatr Neurol. 2006; 34:186-93. rapid induction, hemodynamically stable, less incidence 3. Aminoff MJ, Swaab FB, Sarnath DF, Curatolo HB. of malignant hyperthermia, short duration of surgery Malformations of nervous system: Handbook of Clinical and rapid recovery. Emergence delirium was prevented Neurology, Elsevier. 87/3 series 2008; pg 83-85. by giving propofol 1 mg/kg 10-15 minutes before extu- 4. ACC - What is agenesis of the corpus callosum ? bation. Care in interpretation of cause or nature of twit- University of Maine. Retrieved April 11, 2010. ching, tonicity, shivering, confusion, and anaesthetics 5.Volpe P, Paladini D, Resta M, Stanziano A, Salvatore, with effects on seizures should be considered.Interaction M, Quarantelli M, Gentile M. (2006). Characteristics, among polypharma therapy for seizures, anaesthetic associations and outcome of partial agenesis of the cor- agents and global developmental delay response in the pus callosum in the fetus. Ultrasound In Obstetrics & child was kept in the mind. Possibilty of status epilepti- Gynecology: The Official Journal Of The International cus and need for post operative ventilation was also be Society of Ultrasound in Obstetrics And Gynecolo- kept in mind. Cautious monitoring of vit parameters, gy 27:509–516.

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