Rev. Bras. Anestesiol. vol.65 número5

RevBrasAnestesiol.2015;65(5):414---416

REVISTA

BRASILEIRA

DE

ANESTESIOLOGIA

CLINICAL

INFORMATION

Anaesthesia

for

infant

with

Jarcho

Levin

syndrome:

case

report

Sukran

Geze

,

Ufuk

Arslan

,

Mustafa

Tusat

a_{DepartmentofAnesthesiologyandCriticalCare,FacultyofMedicine,KaradenizTechnicalUniversity,Trabzon,Turkey} b_{DepartmentofPediatricSurgery,FacultyofMedicine,KaradenizTechnicalUniversity,Trabzon,Turkey}

Received24September2012;accepted19December2012 Availableonline16October2013

KEYWORDS

Anaesthesia; infant; JarchoLevin syndrome

Abstract JarchoLevinsyndrome isarare disorder.Therearevarious vertebral andcostal

anomalies.Severe deformities andabnormal fusion ofribs andvertebraecause respiratory

insufficiencyandpneumonia.WepresentanaesthesiainapatientwithJarchoLevinsyndrome forvesicoureteralreflux.

©2013SociedadeBrasileiradeAnestesiologia.PublishedbyElsevier EditoraLtda.Allrights reserved.

PALAVRAS-CHAVE

Anestesia; Crianc¸a;

Síndromede

Jarcho-Levin

Anestesiaemcrianc¸acomsíndromedeJarcho-Levin:relatodecaso

Resumo AsíndromedeJarcho-Levinéumdistúrbioraroqueapresentaváriasanomalias

ver-tebrais e costais. Deformidades graves e fusões anormais das costelas e vértebras causam

insuficiênciarespiratóriaepneumonia.Apresentamosumcasodeanestesiaempacientecom

síndromedeJarcho-Levinpararefluxovesicuretral.

©2013SociedadeBrasileiradeAnestesiologia.PublicadoporElsevierEditoraLtda.Todosos direitosreservados.

Introduction

JarchoLevinsyndromeisararedisorderfirstdescribedby JarchoandLevinin1938.1_{Theprevalenceis0.25/10,000.}2

Vertebral anomalies are the forefront features of Jarcho

Levin syndrome. There are various vertebral and costal

anomalies.Hemivertebra,vertebralagenesis,butterfly

ver-tebrae, hypoplastic vertebrae and costovertebral fusion

defectsarefrequentlyencountered.2,3_{Neuraltubedefects,}

∗_{Correspondingauthor.}

E-mails:drgezes@yahoo.com,gezes@hotmail.com(S.Geze).

cardiovascular,genitourinary,andparenchymalorgan

disor-dersmayaccompanyinJarchoLevinsyndrome.

Hydrocephalus, hydroureteronephrosis,

meningomyelo-cele, atrial septal defect, ventricular septal defect,

renal agenesis---hypoplasia, polycystic kidney, anal

atre-sia, diaphragmaticherniation,oesophageal fistula,Meckel

diverticula, biphidius uvula are among the reported

anomalies.4_{Severedeformitiesandabnormalfusionofribs}

andvertebraecauserespiratoryinsufficiencyandrecurrent

respiratory tract infections.5 _{There is not any literature}

toanaesthesia in Jarcho Levinsyndrome. We present our

anaesthesiamethodinapatientwithJarchoLevinsyndrome

who underwent operation for vesicoureteral reflux with

accompanying vertebralanomalies, hydrocephalus, patent

foramenovaleandsecundumatrialseptaldefect.

AnaesthesiaforJarchoLevinsyndrome 415

Figure1 Therearecostovertebralfusiondefectsinright6---10 andleft7---10ribs.

Figure2 Butterflyvertebraeanomalyexistsinthoracic ver-tebrae.

Case

Thepatientwastwo-month-oldfemaleinfantof6kgweight.

The patient was born via spontaneous vaginal delivery

with 2900g birth weight from 29-year-old mother’s

sec-ond pregnancy. The patient had the diagnosis of Jarcho

Levin syndrome. Patent foramen ovale, secundum atrial

septal defect and hydrocephalus in addition to vertebral

and costal anomalies had been detected in the follow

up period (Figs. 1 and 2). An operation was planned for

the patient with the indication of vesicoureteral reflux.

Bilateral rales were osculated in the preoperative

phys-ical examination. A systolic murmur was detected in

the cardiovascular examination. The patient had grade 3

mallampatiscore.Sensorialandmotorfunctionswere

nor-malintheneurologicalexamination.Routinepreoperative

laboratory tests werenormal. The patientwasmonitored

by electrocardiography and peripheral oxygen saturation

in the operation room. Arrangements were made for the

patient with difficult airway before general anaesthesia.

Becausethe maskventilation wasnormal,mask induction

with oxygen and sevoflurane was performed. Intravenous

remifentanil0.5␮g/kgwasadministered.Lidocaine1mg/kg

wasappliedtoinhibitairwayreflex.LMAofsize1inserted.

Theanaesthesiawasmaintainedwithsevofluraneof2.5---3%

concentration,50% oxygen, 50% nitrous oxide.Soon after

theonsetofoperation,throughclosureofNO2,additional

remifentanil of 0.5␮g/kg was administered. Suppository

paracetamolafterinductionofanaesthesiaandintravenous

tramadolof0.5mg/kgpostoperativelywereadministeredto

maintainpostoperativeanalgesia.After45minfollowupat

thepostoperativecareunitthepatientwastransferredto

paediatricsurgeryclinic.

Discussion

JarchoLevinesyndromeis ararecongenital disorder

cha-racterized by distinctive vertebral anomalies. Vertebral

anomalies and defects in the costovertebral fusion result

in a smaller thoracic cavity and respiratory insufficiency.

Patients frequently experience serious respiratory

prob-lemsduetopneumoniaor restrictivetypelung diseaseat

most cases leading to early mortality.2,5 _{Also central}

air-wayanomaliesareencountered inthis syndrome.Smaller

thoraciccavityandinadequatelungmaturation,lower

res-piratory tract infections and airway problems complicate

anaesthesia required for any kind of operation in child

patients with Jarcho Levin syndrome.6 _{The patient had}

severe vertebral anomalies in our case (Figs. 1 and 2).

Thepatienthadrecovered postnatalpneumoniabut

inspi-ratory rales and secretions in the respiratory tract still

persisted.Inductionwasperformedbymaskinhalationand

thesurgicaloperationwasperformedunderLMA

anaesthe-sia without the need for intubation. Because the patient

wasnotintubated, musclerelaxant wasavoided

minimiz-ing the likely complications in the postoperative period.

LMAwasfirstdiscoveredin1980andhadawidespreaduse

thereafter in anaesthesia practice.It has been used with

successparticularlyin patientsdifficult tointubate.7---10 _In

our case, LMA was used for general anaesthesia for the

patientwhounderwentoperationfor vesicoureteralreflux

duetohydroureteronephrosis.Shortactinganaestheticand

analgesicagentsin LMAanaesthesiawasadvantageousfor

thesafetyofanaesthesiainourcase.Therewasno

respira-torycompromisepostoperatively.Therearereportsinthe

literatureindicatingthatLMAensuressatisfactoryairwayfor

patientsundergoinggeneralanaesthesia.

In our clinic, LMA anaesthesia had previously been

performed with success for a patient with Pierre-Robin

syndromeundergoingtracheotomy. Inliterature, LMA was

showntobeperformedwithcomfortandsuccessinpatients

difficulttointubate. Haraetal. reportedthatLMA wasa

promising anaesthesiamethod for a patientwithCHARGE

syndromewithairwayanomalies.10 _{Wecouldnotfinddata}

relevanttoanaesthesiainJarchoLevinsyndromein

litera-ture.Wesupposethatthelackofliteraturedataisduetothe

poorprognosisofthediseasebecauseofrespiratory

insuf-ficiency leading to early mortality in the infantile period

renderinganyoperationundergeneralanaesthesia

impos-sible.Recentlymoredevelopedmedicalcarehasimproved

theprognosisof JarchoLevinesyndrome andthepatients

hadthechanceofoperationindicatedforvariousproblems.

LMAwasevaluatedasapromisinganaesthesiamethodfor

416 S.Gezeetal.

Conflicts

of

interest

Theauthorsdeclarenoconflictsofinterest.

References

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3.Bannykh SI, Emery SC, Gerber JK. Aberrant Pax1 and Pax9 expression in Jarcho---Levin syndrome: report of two Cau-casian siblings and literature review. Am J Med Genet. 2003;120:241---6.

4.Kansal R, MahoreA, Kukreja S.Jarcho---Levin syndrome with diastematomyelia: a casereportand reviewof literature. J PediatrNeurosci.2011;6:141---3.

5.RobertsAP,ConnerAN,TolmieJL,etal.Spondylothorasicand spondylocostaldysostosis.JBoneJointSurgBr.1988;70:123---6. 6.Schulman M, GonzalezMT, Bye MR.Airway abnormalities in Jarcho---Levinsyndrome:areportoftwocases.JMedGenet. 1993;30:875---6.

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9.HaraY,HirotaK,FukudaK.Successfulairwaymanagementwith useofalaryngealmaskairwayinapatientwithCHARGE syn-drome.JAnesth.2009;23:630---2[EpubNovember2009]. 10.Kiernan F,Crowe S. Safe use of the classic laryngeal mask