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  • Adults-many of them are the same.
  • OBS and peds for students

    1. 1. Complex Health Challenges: A Baby’s Life Story
    2. 2. Jessica Potts is a 32 year old woman who is pregnant for the first time. She is working full time but she and her partner, Susan, are just making ends meet with the expenses for IVF.
    3. 3. Jessica is an average height and weight woman as is Susan. When they decided to have a child it was discovered that Susan has endometriosis and fibroids and can not get pregnant therefore Jessica will be carrying the child.
    4. 4. Treatment of infertility  Usually does not fully prevent conception, especially in mild to moderate cases  Infertility is more common in women with severe forms of disease  Treatments are varied  Surgical treatments are superior to hormonal or medical treatments when goal is enhanced fertility  Assisted reproduction may be used
    5. 5. After being unsuccessful with artificial insemination, the couple opt for in vitro fertilization, Jessica is successful with IVF therapy on the first attempt.
    6. 6. IVF  In vitro fertilization  Successfully used in 1978
    7. 7. IVF: Who May Benefit?  Women who have a blocked or damaged fallopian tube  Mild problem with male partner’s sperm  No cause identified for inability to conceive  Patient’s who have tried IUI or ovulation induction with no success
    8. 8. IVF Procedure  Fertility drugs: -gonadotrophin-releasing hormones (GnRH) -human-menopausal gonadtrophin (hMG) -human chorionic gonadotrophin (hCG).  Monitor blood hormone levels  Ultrasound scan  Remove ova through ultrasound-guided transvaginal retrieval or laparoscopy
    9. 9. IVF Procedure Cont’d  Eggs are mixed with sperm in dish and cultured in incubator  Dish checked in 2 days to see if eggs have been fertilized  Those kept are kept for a couple more days and checked again  Fertilized eggs form ball of cells-embryo  Healthiest embryo is inserted into uterus  Taking progesterone all along to thicken lining
    10. 10. IVF Procedure Cont’d  Progesterone given by injection, pessary (gel)  Endometrium too thin IVF cycle abandoned  Two embryos transferred with thin catheter through cervix into the uterus (via ultrasound)  No more then three embryos can be legally transferred  Number of embryos transferred depends on your age and chances of success  If successful able to take a pregnancy test in 2 weeks
    11. 11. IVF  IVF treatment takes 4-6 weeks to complete  Success rates vary  Advantages:  gives women with blocked, damaged, or missing fallopian tubes a chance to have a baby  Disadvantages:  increased chances for multiple births, increase risk for miscarriage and other complications, hormones not closely monitored lead to ovarian hyperstimulation syndrome, ectopic pregnancy
    12. 12. MULTIPLE PREGNANCY Multiply pregnancy occurswhen theuseof ovulation inducing medication triggersthereleaseof multipleeggs, which, when fertilized producemultipleembryosthat arethen implanted
    13. 13. Case Study  The couple live in a small town about half an hour from the city.  The medical clinic arranges for Jessica’s prenatal care and any tests and assessments she may need for the duration of her pregnancy and delivery.
    14. 14. Case Study  Jessica and Susan are so happy to finally be pregnant, they refuse most prenatal tests.  They’re just so happy to have a baby in their lives, they are not too concerned about genetics and anomalies with the baby.  They due, however, agree to have an ultrasound.
    15. 15. Non-invasivePrenatal Tests
    16. 16. Doppler Ultrasound Thisnoninvasivetest measures blood flow in different partsof your baby'sbody — such asthe umbilical cord, brain, liver, and heart — to help your caregiver assessyour baby'shealth. It can bedoneat thesametimeasan ultrasound and usesthesame equipment. Student presentation for more information…
    17. 17. Mrs. Pottsgoesinto spontaneouslabor at 3am at 39 weeks gestation. Her labor and delivery areunremarkableand Jessica and Susan welcomebaby Isabelleat 5:56am. Thecouplebecome concerned when thenursesstart whispering about thebaby. Jessicakeepsasking them what theproblem is, but getsno immediateresponse. Onenursefinally tellsSusan and Jessicathat thedoctor will bein soon to talk to them. Eventually, thedoctor deliversthenewsthat their preciouslittlebaby girl infact has Down Syndrome. Jessicaand Susan react with anger. They are dissappointed that they didn’t go through with theserum screening testshowever, they must moveforward now. Case Study
    18. 18. Down Syndrome
    19. 19. Down Syndrome…  Isthemost frequently occurring chromosomal disorder  Isuniversal acrossraceand gender  Iscaused by an error in cell division  Occursat conception  Why it occursisunknown
    20. 20. History  John Langdon Down (thefather of down syndrome)  1866: published an accuratedescription of aperson with down syndrome  JeromeLejeune  Identified down syndromeasachromosomal anomaly
    21. 21. Incidence  Incidenceincreaseswith age  In CanadaDown Syndromeoccursin approximately 1 in 800 livebirths  Increasesto 1 in 100 in second birth if your first child had Down Syndrome  In theUSmorethan 350 000 peoplehaveDown Syndrome
    22. 22. Incidenceof Down Syndrome Maternal Age Incidence of Down Syndrome 20 1 in 2000 25 1 in 1200 30 1 in 900 35 1 in 350 40 1 in 100 45 1 in 30 49 1 in 10
    23. 23. Trisomony 21  Also called Down’s Syndrome  Specific characteristics  Chromosome abnormality  Genetic testing
    24. 24. Down Syndrome  Thepresenceof 47 chromosomesinstead of 46  Morespecifically it isthepresenceof extra genetic material associated with the21st chromosome. (Trisomy)  Caused by an error in cell division
    25. 25. Mitosis  Theprocessof cell division involved in all cell growth, differentiation, and repair  Thechromosomesof each cell duplicate  Two daughter cellsareproduced  They arediploid (contain 46 chromosomesin 23 pairs)  Occursin all cellsexcept for theoocytesand sperm
    26. 26. Mitosis
    27. 27. Meiosis  Occursin reproductivecells  Thereisareduction in thenumber of chromosomes occurs(end up with 23 chromosomes)  Oocytesand sperm arereferred to asbeing haploid (contain asinglecopy of each chromosome)  Thepaired chromosomescometogether in preparation for cell division, portionscrossover and genetic material isexchanged  recombination createsgreater diversity in oocytesand sperm
    28. 28. Meiosis
    29. 29. Nondisjunction  Accountsfor approximately 95% of down syndromecases  A pair of chromosomesmay fail to separatecompletely creating asperm or oocytethat containseither 2 copiesor no copiesof aparticular chromosome.  Trisomy: when thereare2 copies  Monosomy: when thereareno copies  Prior to or at conception, apair of 21st chromosomesin either thesperm or theegg failsto separate. Astheembryo develops, theextrachromosomeis replicated in every cell of thebody
    30. 30. Trisomy  Down syndromeisaform of trisomy  thereisextragenetic material on the 21st chromosome  Trisomy can occur on any chromosomebut theonly formsthat arefrequently seen in livebirthsare on the13, 18, and 21 chromosome
    31. 31. Mosaicism  Mosaicism occurswhen nondisjunction of chromosome21 takesplacein oneof theinitial cell divisionsafter fertilization causing aperson to have46 chromosomesin someof their cellsand 47 in others  Thisistheleast common form of Down syndrome  accountsfor only 1 to 2 percent of all cases
    32. 32. Translocation  Occurswhen part of chromosome21 breaksoff during cell division and attachesto another chromosome, usually chromosome14. Whilethetotal number of chromosomesin thecellsremains46, thepresenceof an extra part of chromosome21 causesthecharacteristicsof Down syndrome  Maternal ageisnot linked to thechanceof having ababy with translocation. Most casesaresporadic, chanceevents, but in about onethird of translocation cases, oneparent isacarrier of atranslocated chromosome. For thisreason, thechanceof translocation in asecond pregnancy ishigher than that seen in nondisjunction.  Accountsfor 3 to 4 percent
    33. 33. Appearance  low nasal bridge  epicanthal folds(eyes)  protruding tongue  low set ears  poor muscletone(hypotonia)  short stature  singlecreaseacrosspalm of hand  slightly flattened facial profile
    34. 34. Characteristics & Conditions  3/4 fetusesarespontaneously aborted  20% die beforetheageof 10 r/t complications  haveand IQ ranging from 25-50  1-3 to1-2 have congenital heart defects  most common arean atrioventricular septal defect, persistant ductusarteriosus, and tetraology of fallot  decreased ability to fight respiratory infections  increased susceptibility to leukemia  usually develop Alzheimer symptomsby the ageof 40  increased risk for thyroid and vision problems  usually accompanied by somelevel of mental retardation  averagelife expectancy is55
    35. 35. Treatment  Down syndromeisnot treated  Thesymptomsaretreated  It isimportant to encourageindividualswith down syndrometo develop their giftsand talents  Early intervention programscan beinitiated  Many individualswith down syndromego to school (elementary, secondary, and post-secondary) and someadults arecapableof working in thecommunity.  With proper careindividualswith down syndromecan lead healthy lives
    36. 36. Diagnosis  Prenatal screening (usually diagnosed here, but not in Jessica’scase)  maternal serum screening  ultrasound (sonogram) screening  Diagnostic testing  chorionic villussampling (CVS)  amniocentesis  percutaneousumbilical blood sampling (PUBS)  Diagnosisismadeat thebirth of baby Isabelle
    37. 37. Along with the diagnosis of Down’s  The doctor informs Susan and Jessica that he would like to run some tests on baby Isabelle as it appears her head is abnormally large  Jessica and Susan are panicked at this point and the nurse tries to comfort the couples’ anxiety  The doctor refers to a neurologist who examines baby Isabelle
    38. 38.  The neurologist is a stoic man in his late 60’s and doesn’t believe in same-sex couples having children  He bluntly tells Susan and Jessica the his diagnosis of baby Isabelle and refers them to community services for follow up  Isabelle has…
    39. 39. What istheDiagnosis? Hydrocephalus
    40. 40. Structureof theBrain
    41. 41. Ventriclesof theBrain A Lateral View An Anterior View
    42. 42. Ventriclesof theBrain  Lateral Ventricles  Each cerebral hemispherecontainsalargelateral ventricle: Right and Left ventricle or First and Second ventricle  Theseptum pellucidum separatesthetwo lateral ventricles  Third Ventricle  Located in thediencephalons  Two lateral ventriclesarenot directly connected to each other  communicateswith thethird ventriclethrough an interventricularforamen(foramen of Monro)  Third and Fourth ventricleareconnected through aslender canal known asthe mesencephalic aqueduct (the aqueduct of Sylvius) located in themesencephalon  Fo urth Ventricle  Superior portion liesbetween theposterior surfaceof theponsand theanterior surfaceof thecerebellum  Extendsinto thesuperior portion of themedullaoblongata  Then narrowsand becomescontinuouswith thecentral canal of thespinal cord
    43. 43. Ependymal Cells
    44. 44. Ependymal Cells  Central canal: narrow passageway in thespinal cord  In thebrain, thepassageway formstheventricles  Thecentral canal and ventriclesarelined by acellular layer of epithelial cellscalled theependymaand arefilled with cerebrospinal fluid (CSF)  During embroyonic development and early childhood, thefree surfaceof ependymal cellsarecovered with cilia  Theciliapersistsin adultsonly within theventriclesof thebrain, where they assist in thecirculation of CSF  In other areas, theependymal cellstypically havescattered microvilli  Function:  participatein thesecretion of theCSF  sensory functions, such asmonitoring thecomposition of theCSF
    45. 45. TheCranial Meninges Layers:  Cranial duramater:  Consistsof outer (Endosteal) and inner (Meningeal) fibrouslayers  Layersaretypically separated by aslender gab that containstissuesfluidsand blood vessels, including several largevenoussinuses(Dural sinus).  Theveinsof thebrain open into thesesinuses, which deliver thevenousblood to the internal jugular veinsin theneck.  Arachnoid:  Consistsof thearachnoid membrane, an epithelial layer, and thecellsand fibersof the arachnoid trabeculaethat crossthesubarachnoid spaceto thepiamater.  Arachnoid membranecoversthebrain, providing asmooth surfacethat doesnot follow the brain’sunderlying folds.  Piamater:  Sticksto thesurfaceof thebrain  It extendsinto every fold, and accompaniesthebranchesof cerebral blood vesselsasthey penetratethesurfaceof thebrain to reach internal structures.
    46. 46. Cranial Meninges
    47. 47. TheCranial Meninges Functio n:  To protect thebrain  Dural foldsprovideadditional stabilization and support to thebrain.  Dural sinusesarelargecollecting veins  Threelayersof dural folds:  Falx cerebri:  Superior sagittal sinusand theinferior sagittal sinus(venoussinuses) liewithin thisdural fold.  Tentorium cerebelli:  Transversesinuslieswithin thetentorium cerebelli.  Falx cerebelli
    48. 48. Dural Folds
    49. 49. Cerebrospinal Fluid Functio n:  Completely surroundsand bathestheexposed surfacesof the CNSand hasseveral important functions:  Cushioning DelicateNeural Structures  Supporting theBrain:  Thebrain issuspended insidethecranium and floatsin theCSF.  A human brain weighsabout 1400 g in theair, but only about 50 g when supported by theCSF.  Transporting Nutrients, Chemical Messengers, and WasteProducts:  Ependymal lining isfreely permeable(exception: choroidsplexus)  CSF isin constant chemical communication with theinterstitial fluid of the CNS
    50. 50. CSF continued Fo rmatio n o f CSF:  Choroid plexus: consistsof acombination of specialized ependymal cells and permeablecapillariesfor theproduction of cerebrospinal fluid.  Location:  Two extensivefoldsof thechoroid plexusoriginatein theroof of thethird ventricleand extend through theinterventricular foramina. Thesefoldscover thefloorsof thelateral ventricles.  In theinferior brain stem, aregion of thechoroid plexusin theroof of thefourth ventricleprojectsbetween thecerebrellum and thepons.  Specialized ependymal cells, interconnected by tight junctions, surround the capillariesof thechoroid plexus.  Theependymal cellssecreteCSF into theventricles  Also remove waist productsfrom theCSF and adjust itscomposition over time.
    51. 51. Circulation of Cerebral Spinal Fluid
    52. 52. CSF continued Circulating CSF  Choroid plexusproducesCSF at arateof about 500 ml/day  Total volumeof CSF at any momentsisapproximately 150 ml/day  entirevolumeof CSF isreplaced every eight hours  CSF circulatesfrom thechoroid plexusthrough theventriclesand thecentral canal of thespinal cord  AstheCSF circulates, diffusion between it and theinterstitial fluids(theextracellular fluidsin most tissuesiscalled interstitial fluid) of theCNSisunrestricted between and acrossthe ependymal cells.  TheCSF reachesthesubarachnoid spacethrough thetwo lateral aperturesand thesinglemedian aperture, opening in theroof of thefourth ventricle.  CSF then flowsthrough thesubarachnoid spacesurrounding thebrain, spinal cord, and cauda equine.  Fingerlikeextensionsof thearachnoid membrane, called arachnoid villi, penetratethe meningeal layer of theduramater and extend into thesuperior sagittal sinus.  In adults, clustersof villi form largearachnoid granulations.  CSF isabsorbed into thevenouscirculation at thearachnoid granulationswhereit isfiltered and discarded by thebody.
    53. 53. TheBlood Brain Barrier  Neural tissuein the CNSisisolated from thegeneral circulation by theblood-brain barrier  Thisbarrier existsbecausetheendothelial cellsthat linethecapillariesof theCNS areextensively interconnected by tight junctions  Thesejunctionsprevent thediffusion of materialsbetween adjacent endothelial cells  Only lipid-solublecompoundscan diffuseacrosstheendothelial cellsmembranes into theinterstitial fluid of thebrain and spinal cord Astro cyte Cells:  Restricted permeability characteristicsof theendothelial lining of brain capillaries aredepended on chemicalssecreted by theastrocytes  cellsthat arein closecontact with CNScapillaries  Outer surfacesof theendotheliacellsarecovered by theprocessesof astrocytes  Releasechemicalsthat control thepermeability of theendothelium to various substances  If damaged or stop stimulating theendothelial cells, theblood-brain barrier disappears.
    54. 54. AstrocyteCells
    55. 55. Blood-CSFBarrier  Choroidsplexus:  Not part of theneural tissueof thebrain thereforeno astrocytesarein contact with theendothelial cells.  Asaresult, capillariesin thechoroidsplexusarehighly permeable  Blood-CSF barrier:  Substancesdo not havefreeaccessto theCNS  Specialized ependymal cellsinterconnected by tight junctions, surround the capillariesof thechoroidsplexus  Transport acrosstheblood-brain and blood-CSF barriersisselectiveand directional  Even the passage of small ions(sodium, hydrogen, potassium, or chloride) is controlled  Someorganic compoundsarereadily transported, and otherscrossonly in minuteamounts.
    56. 56.  Susan and Jessica and angry and bitter  They want to know why this diagnosis of Hydrocephalus wasn’t caught sooner? Why were they missed?  An ultrasound will normally show that the fetus’ fontanelle is bulging, and that the head circumference is larger than normal for the gestational age
    57. 57. What isHydrocephalus?  Theterm hydrocephalusisderived from two words:  "hydro " meaning water  "cephalus" referring to thehead  A condition in which excessCSF buildsup within theventricles of thebrain or in thesubarachnoid spaceand may increase pressurewithin thehead  Can occur at any age  most common in infantsand adultsage60 and older  In most instances, hydrocephalusisalifelong condition in that thepatient istreated rather than "cured"  If left untreated in theinfant, they can suffer from somedegree of mental retardation and/or motor dysfunction.
    58. 58. Epidemiology  In theUnited States, alittleover 1 in 1000 birthsareaffected by hydrocephalus.  Ashigh as1 in 500 births.  Hydrocephalusisoneof themost common "birth defects" and afflictsin excessof 10,000 babieseach year.  Studiesby theWorld Health Organization show that onebirth in every 2,000 result in hydrocephalus.  Thereare70,000 dischargesayear from hospitalsin theUnited Stateswith a diagnosisof hydrocephalus.  Morethan 50% of hydrocephaluscasesarecongenital.  Asmany as75% of children with hydrocephaluswill havesomeform of motor disability.  Over thepast 25 years, death ratesassociated with hydrocephalushavedecreased from 54% to 5%; intellectual disabilitity hasdecreased from 62% to 30%.  Ocular gazeand movement disordersarefound in approximately 25 to 33% of children with hydrocephalus.  About 80% of hydrocephaluspatientsareborn with other defects.
    59. 59. Typesof Hydrocephalus Congenital: when the condition exists at birth Acquired: when it occurs as the result of a trauma to the brain afterbirth.
    60. 60. Pathophysiology Impaired absorption of CSF from thesubarachnoid spaceoccurswhen an obstructive processdisruptstheflow of CSF through thesubarachnoid space. Thefluid doesnot reach theconvex portion of thecerebrum, wherethearachnoid granulationsarelocated. With acutehydrocephalus, thereisincreased ICPthat hasarapid onset. Thepatient can deterioraterapidly into a deep comaif it isnot treated promptly. ICPrisesif production of CSF exceedsabsorption. Thisoccursif CSF isoverproduced, resistanceto CSF flow isincreased, or venoussinuspressureisincreased.
    61. 61. CSF production fallsasICPrises. Compensation may occur through transventricular absorption of CSF and also by absorption along nerve root sleeves. Temporal and frontal hornsdilatefirst, often asymmetrically. Thismay result in elevation of thecorpuscallosum, stretching or perforation of theseptum pellucidum, thinning of thecerebral mantle, or enlargement of thethird ventricledownward into thepituitary fossa (which may causepituitary dysfunction).
    62. 62. Causesof Hydrocephalus Thisisgrouped into 3 main causes: 1.  Excessivesecretion of CSF by thechoroid plexusasin casesof choroid plexuspapilloma(rare, bening tumour) or carcinoma. This isararecause. Choroid PlexusPapilloma
    63. 63. 2.  Blockageto CSF circulation. Thiscould beat any level of theCSF circulation. •It could beat thelevel of theforamen of Monro wherewethereisunilateral or bilateral coverageof theforamen of Monro giving dilatation of oneor both lateral ventricles. •Thisiscommonly seen in thecolloid cyst and tumoursof thethird ventricle. • Suprasellar lesion assuprasellar arachnoid cyst or hypothalamic tumours (craniopharyngioma; congenital pituitary tumour). • Posterior fossatumoursareacommon causeof obstructivehydrocephalus dueto blockageof the4th ventricle. • Medulloblastoma, cystic astrocytomaand ependymomacan all lead to obstructivehydrocephalus. 3. Poor secretion of CSF into thevenoussinusescaused by scarring of the arachnoid villi and iscommonly seen after meningitisor hemorrhage.  
    64. 64. Forms of Hydrocephalus Description Communicating Hydrocephalus (non-obstructive hydrocephalus) caused by inadequate absorption of CSF when the ventricular pathways are not obstructed. Noncommunicating Hydrocephalus (obstructive hydrocephalus) caused by blockage in the ventricular pathways through which CSF flows. Obstructive hydrocephalus Results from obstruction of the flow of CSF (intraventricular or extraventricular). Most hydrocephalus is obstructive, and the term is used to contrast the hydrocephalus caused by overproduction of CSF. Arrested hydrocephalus Stabilization of known ventricular enlargement, probably secondary to compensatory mechanisms. These patients may decompensate, especially following minor head injuries. Forms of Hydrocephalus
    65. 65. Causes of Hydrocephalus
    66. 66. Congenital Causesin Infantsand Children  Characterized by an increased volumeof CSF May becaused by:  A blockagewithin theventricular system in which theCSF flows An imbalancein production of theCSF Reduced reabsorption of theCSF that resultsin enlargement of the ventricles, and increased ICP  This pressurewithin theventricular system pushesand compressesthe brain against theskull cavity.  Beforethecranial suturesfuse, theskull can increaseto accommodate theadditional space-occupying volumeto preserveneuronal function.
    67. 67. Stenosis of the Aqueduct of Sylvius Due to malformation: This is responsible for10% of all cases of hydrocephalus in newborns, and is the most common cause. Bickers-Adam Malformation This is an X-linked hydrocephalus. It is characterized by stenosis of the aqueduct of Sylvius, severe mental retardation, and in 50% by an adduction-flexion deformity of the thumb.
    68. 68. Dandy-WalkerMalformation • Thisaffects2-4% of newbornswith hydrocephalus. •Dandy-Walker Malformation isararemalformation of thebrain that ispresent at birth (congenital). •Dandy-Walker Malformation isaform of "Obstructive" or "Internal Noncommunicating Hydrocephalus," meaning that thenormal flow of cerebrospinal fluid isblocked resulting in thewidening of theventricles. •It ischaracterized by an abnormally enlarged spaceat theback of thebrain (cystic 4th ventricle) that interfereswith thenormal flow of cerebrospinal fluid through theopeningsbetween theventricleand other partsof thebrain (foraminaof Magendiaand Luschka). •Excessiveamountsof fluid accumulatearound thebrain and causeabnormally high pressurewithin theskull, swelling of thehead (congenital hydrocephalus), and neurological impairment. Motor delaysand learning problemsmay also occur.
    69. 69. Arnold-Chiari Malformation • Chiari malformations(CMs) arestructural defectsin the cerebellum, thepart of thebrain that controlsbalance. • Thecerebellum and brainstem can bepushed downward. • Theresulting pressureon thecerebellum can block theflow of cerebrospinal fluid and can causearangeof symptomsincluding dizziness, muscleweakness, numbness, vision problems, headache, and problemswith balanceand coordination.
    70. 70. • Isaccompanied by amyelomeningocele-aform of spinabifida that occurswhen thespinal canal and backbonedo not closebefore birth, causing thespinal cord to protrudethrough an opening in the back. •Thiscan causepartial or completeparalysisbelow thespinal opening, and hydrocephalus.
    71. 71. Agenesis of the Foramen of Monro • Narrowing of theforamen of Monroe. • Sincetheforamen narrows, thisleadsto increased pressureto push theCSF through theforamen of Monroe. • AKA Interventricular foramen.
    72. 72. Congenital Toxoplasmosis • Group of symptomsand characteristicscaused by infection of thefetuswith the organism To xo plasma go ndii. • Fetal infection resultswhen anonimmunepregnant woman isinitially infected with toxoplasmosis(from certain foods, cat feces, or if shehasahistory of toxoplasmosisduring previouspregnancies). • Congenital toxoplasmosisischaracterized by damageto theeyes, nervous system, skin, and ears. • Can occur asaresult of ingestion of raw or inadequately cooked infected meat, ingestion of oocysts, an environmentally resistant form of theorganism that cats passin their feces, with exposureof humansoccurring through exposureto cat litter or soil (e.g., from gardening or unwashed fruitsor vegetables), and anewly infected pregnant woman passing theinfection to her unborn fetus.
    73. 73. Arnold-Chiari Malformation Dandy-Walker Malformation MRI’s: Congenital Causes of Hydrocephalus
    74. 74. Acquired Causesin Infantsand Children Masslesionsaccount for 20% of all casesof hydrocephalusin children. Theseareusually tumors(eg, medulloblastoma, astrocytoma), but cysts, abscesses, or hematomaalso can bethe cause. Intraventricular hemorrhagecan berelated to prematurity, head injury, or ruptureof avascular malformation. Infections: Meningitis(especially bacterial) and, in some geographic areas, cysticercosiscan causehydrocephalus. •Increased venoussinuspressure: Thiscan berelated to achondroplasia, somecraniostenoses, or venousthrombosis. Iatrogenic (result of medical interventions): HypervitaminosisA, by increasing secretion of CSF or by increasing permeability of theblood-brain barrier, can lead to hydrocephalus. Idiopathic
    75. 75. Signs and Symptoms of Hydrocephalus Clinical featuresof hydrocephalus areinfluenced by thefollowing:  Patient'sage  Cause  Location of obstruction  Duration Rapidity of onset
    76. 76.  Poor feeding  Irritability  Reduced activity  Vomiting  Seizures  Bulging fontanelle Thin, shiny skin over fontanelles Papilledema(swelling of theeye’snerves) and later optic atrophy Symptomsin Infants
    77. 77. Symptomsin Children  Slowing of mental capacity  Headaches(initially in themorning) that aremoresignificant than in infantsbecauseof skull rigidity  Neck pain suggesting tonsillar herniation  Vomiting, moresignificant in themorning  Blurred vision - Consequenceof papilledema(swelling of theeye’s nerves) and later of optic atrophy  Doublevision - Related to unilateral or bilateral sixth nervepalsy (affectsabducenscranial nerve, and eyescannot turn outward beyond midline, doublevision also occurs, but disappearswhen oneeyeisclosed)
    78. 78.  Stunted growth and sexual maturation from third ventricle dilatation: Thiscan lead to obesity and to precociousor delayed onset of puberty.  Difficulty in walking secondary to spasticity: Thisaffectsthe lower limbspreferentially becausetheperiventricular pyramidal tract isstretched by thehydrocephalus. Drowsiness
    79. 79. Physical Assessment of A Neonate *Similar to adult head-to -to e assessment, with the fo llo wing exceptio ns: *
    80. 80. • Vital signs • Skin and hair – Lanugo, vernix caseosa(thick, cheezy protectiveintegumentary deposit that consistsof sebum, and shed epithelial cells). Stork bites(back of neck, lower occiput, upper eyelids, and upper lip). • Head, Faceand Eyes– Infantshaveanterior or posterior fontanelles, and they should not bulgeor sink.
    81. 81. • Newbornsdon’t producetearsuntil 2-3 months. • TheEustachian tubeismorehorizontal, wider, and shorter, thuscan increaselikelihood of middleear infections. • Thorax and Lungs: up to 3-4 monthsabdominal breathing. Measurechest circumference. • Cardiovascular: Infantshaveahigher circulating blood volume • Abdomen: Liver isproportionately larger. • Musculoskeletal: Bonegrowth endsat 20 (when epiphysiscloses).
    82. 82. • Neurological: ApgarScores – Method to reassessneed for newborn resuscitation in thedelivery room. • Given at 1 and 5 minutesfollowing birth. Scoreof 8-10 Newborn in good condition, 4-7 Moderately depressed newborn, 0-3 indicatesseveredepression, and needs immediateresuscitation (Seeoverhead) • Reflexes: Rooting, sucking, palmar grasp, tonic neck, stepping, plantar grasp, Babinski’s, and Moro.
    83. 83. • Genitourinary System: During infancy, thebladder islocated in between thesymphysispubisand theumbilicus. Monitor I & O. • Gastrointestinal: Meconium stools, then after 3 days, yellow coloured. Important to monitor bowel function and I & O to ensurethat infant doesnot becomedehydrated. • Inspection of genitalia
    84. 84. Clinical Manifestations Upon Physical Assessment
    85. 85. Infants  Head enlargement: Head circumferenceisin the98th percentilefor theageor greater.  Dysjunction of sutures: Thiscan beseen or palpated.  Dilated scalp veins: Thescalp isthin and shiny with easily visibleveins.  Tensefontanelle: Theanterior fontanellein infantswho are held erect and arenot crying may beexcessively tense.
    86. 86. Setting-sun sign: In infantsit ischaracteristic of increased ICP. Both ocular globesaredeviated downward, theupper lidsareretracted, and thewhite scleraemay bevisibleabovetheiris. Increased limb tone: Spasticity affectsthelower limbs. Thecauseisstretching of theperiventricular pyramidal tract fibersby hydrocephalus.
    87. 87. Children  Papilledema: if theraised ICPisnot treated, thiscan lead to optic atrophy and vision loss.  Failureof upward gaze: Thisisdueto pressureon the tectal platethrough thesuprapineal recess.  Macewen sign: A "cracked pot" sound isnoted on percussion of thehead.
    88. 88.  Unsteady gait: Thisisrelated to spasticity in thelower extremities. Largehead: Suturesareclosed, but chronic increased ICPwill lead to progressiveabnormal head growth.  Unilateral or bilateral sixth nervepalsy (affectsabducens cranial nerve, and eyescannot turn outward beyond midline, doublevision also occurs, but disappearswhen oneeyeis closed) issecondary to increased ICP.
    89. 89. Getting theDiagnosis  With newborns, hydrocephalusisdetected almost immediately asthechild'shead may belarger than normal (macrocephaly). However, with older children or adults, hydrocephalususually startsto reveal itself with avariety of signsand symptomsweeksor months beforeit isdetected.  It may bedetected by signsand symptomsof increased cranial pressure.
    90. 90. CT and MRI  X-Raysdo not provideenough contrast to seethetissuesof thebrain.  CT  Clearer picturesof thebodiesorgans, tissuesand bones. Approx. 2-5 minutes.  MRI  Internal structurescan beseen. Approx. an hour in length.  Provideaclearer view of gray and whitematter of thebrain, aswell as thevascular system. Primary usefor neurosurgeons.  CT and MRI scanstakepicturesof thecompletecranial and intracranial anatomy, including thesubarachnoid spacesand thestructuresof the posterior fossa.  Taken laterally and sagitally (front-back)
    91. 91. Diagnosisof Hydrocephalus  Abnormal Head Growth (Macrocephaly)  Infantsand small children primary indicator. Kidssutureshavenot fused together yet.  Continueto monitor thegrowth of thechild’shead until thechild reachestheage of 6 or 7.  Signsand Symptoms:  Irritable  High pitched cry/scream  Split suturesof theskull  Distended veinsin thescalp-bulging or widening of thefontanels  Absenceof up ward'sgaze, known as“sun setting”  usually in acutenon- communicating hydrocephalus.  Impaired lateral gaze(Sun setting oneor both eyes)  Lossof vision-weaknessor spasticity of limbs.
    92. 92. Initial Diagnosis  Initially, when oneor moresymptomsbecomeevident.  Infant  Child’shead isbulging or larger than normal  Child  Painful headaches, gait disorder or vision problems  Should bereferred to aneurosurgeon  Neurological Examination  History of milestones, as well asaphysical examination for neurological deficits.
    93. 93. Full-Term Infant  1 year or older  Examinetheinfant to seeif they arereaching mental and physical developmental milestones.  Mental Milestones:  Isyour infant communicating verbally?  Isyour infant performing well in school?  Hasyour infant fallen behind hispeersin recent months?  Isyour child having ahard timeremembering things?  Haveyou noticed any changesin personality in thelast few weeks/months?
    94. 94. Continued…  Physical Milestones:  Hasyour child started to show signsof walking by thetimethey were1?  Isyour infantsgait steady or unbalanced?  Doesyour child drift to thesidewhilethey walk?  Get thechild to balanceon onefoot, with their eyesclosed. Placeboth feet together sideby sideto maintain balance.  Placetheindex finger in front of thefaceand ask to follow movement. (testing for paralysisof theabducens- 6th cranial nerve). Controlssideto side(lateral) movement.  Walk heal-to-heal. If child hasdifficulty could bean indicator of pressure on thecerebellum.  Check plantar, Babinski reflex.  If thebig toemovesupward, resultsan extensor responseor Babinski reflex. Babinski reflex isaclear indication of someform of brain or spinal cord disease. Usually skip thistill theinfant isat least 1 year old becauseit isusually positivewhether theinfant hasit or not.  Pronator drift: Closeeyeswhilestanding, extend both armsin front with palmsup. Seeif onearm waversor drifts. Indication of injury to themotor areasof thebrain.
    95. 95. Effectson Family Dynamics  Emotionscan rangefrom worry to fear, aswell asresentment and jealousy.  Children also haveactiveimaginations.  Usually their emotionsareworsethan their reality perceives.  Talk through their fears.  Siblingsmay feel completely overwhelmed.  Resentment and jealousy arecommon feelings experienced by siblings.  Let them know they areloved and valued.
    96. 96. Preventing Childhood Hydrocephalus  Protecting thehead of theinfant or child from injury by handling thechild carefully may help prevent thedevelopment of injury induced hydrocephalus.  Prompt treatment of infectionssuch asmeningitisand othersassociated with hydrocephalusmay reducetherisk of developing thedisease.  Women who takecytomegalovirusor toxoplasmosisacquired by amother during pregnancy may cause hydrocephalus. May reducetherisk of being infected by toxoplasmosisby:  Cooking meet and veggiescarefully.  Cleaning contaminated knivesand cutting servicesproperly.  Avoid handling cat litter, or wearing gloveswhen cleaning thelitter box.  Lymphocytic choriomengitisvirus(LCV) which pet rodants(mice) often carry can lead to hydrocephalusin pregnancy.  Infection with chickenpox or mumpsduring or right after pregnancy may also lead to hydrocephalusin thebaby.
    97. 97. Roleof Nurses  Bedsidenurseisin auniqueposition to havean impact on patients’ and families’ lives.  Nurseneedsto empower and educatethefamily of theimportanceof aseptic techniquewhen taking careof thechild’ssurgical site.  Stresstheimportanceto thefamily that their child should maintain optimal health with proper nutrition and exercise.  Needsto supply thefamilieswith life-saving information of thesignsand symptomsof ashunt malfunction and or infection.
    98. 98. Multidisciplinary Workers  Nutritional Support  Physical Therapy  Occupational Therapy  Neurosurgeon  Pediatrician  Nurses  Ophthalmologist
    99. 99. Isabelle’sscalp, over theanterior fontanelle, is shiny and thin and thetiny veinsareprominent. Isabelleisthen sent to theNICU to beclosely monitored for complicationsassociated with increased intracranial pressure. Exactly 2 weeks after Isabellewasborn, sheundergoessurgery to insert aVPshunt. Thesurgery went very well with no complications.
    100. 100. Ventriculo-Peritoneal Shunts In Infants
    101. 101. HydrocephalusShunt Statistics  Thereare25,000 shunt operationsperformed each year in theUnited States. Of those, some18,000 areinitial shunt placements.  Some85% of peoplewith shuntshavehad at least two shunt operations.  Studiesshow that therisk of shunt failurein an infant'sfirst year is30%.  Shuntsarerevised about 2 timesin thefirst ten yearsof useper patient.  95% of shunt infectionsoccur within 3 to 5 daysof surgery.  Thereported frequency of shunt infection variesfrom 1.5 to 39% with an averageof 10 to 15%.  Morethan 50% of staphylococcal infectionsoccur with in 2 weeksof the operation, and 70% of infectionsoccur within 2 months.  Theoverall complication rateof CSF shuntsremainsquitehigh: 25 to 60%.  Shunt malfunctionsoccur in about two to 40% of cases.
    102. 102. What isaVentriculo-Peritoneal Shunt?  Primary Goal of aVPshunt: To ensureon aregular basisthat theshunt continuesto function!  A VPisalong, plastic tubethat allowsfluid to drain from thebrain to another part of thebody (Peritoneal Cavity). Thisdrainagepreventsincreased pressureon thebrain.  VPShunt hasat least threeparts:  1) Ventricular Catheter: Goesin thebrain  2) Valve: It controlsthepressurewithin thebrain.  3) Distal Catheter: Isunderneath theskin and connectstheother partsof theVP shunt to aspacewithin thebody, usually theabdominal cavity (peritoneal cavity). Thismay also beplaced behind theinfant’sear.  Thefluid flowsthrough thistubefrom thebrain into theabdominal cavity. In thisarea, the body absorbsthefluid. It doesnot go into thestomach.
    103. 103. Advantagesof aVPShunt  Advantages of Peritoneal Shunting. 1. If an infection develops, it isnot aspotentially life threatening, aswith shuntsin thevenoussystem. 2. A largeamount of tubing can beplaceintra- peritoneal to minimizetheneed for elective lengthening. 3. Theoverall easein placing peritoneal shuntsin a relatively short operation.
    104. 104. Problemsthat may Arisewith VPShunts  Risks that may Arise: 1. Abdomen= Bowel twisting and excessfluid overload. 2. Blockageof theShunt 3. Brain Injury= Clots, Lossof Sensation, Memory Loss, Paralysis, Seizures, Speech Problems, Headachescaused by overdraining, and Mechanical Failure 4. Bleeding, Problemswith anesthesia 5. Body may react negativebecauseof foreign material 6. Approximately 10% of shuntsfail within 10 yearsof placement. 7. May requireasmany as5 surgeries
    105. 105. How ShuntsWork  Beforeshunt placement aCT imageof thebrain will show abuild up of CSF in theventricles. Figure1. Dark areain themiddleisthebuild up of CSF.
    106. 106. How ShuntsWork  Shunt implantation  Goal isfor theshunt system to mimic what would occur in the body naturally. CSF will bechained by theshunt, and theflow will beregulated so that aconstant ICPismaintained.  After shunt placement Post-op CT scan image  Ventricleshavebeen drained and haveresumed normal size. Whitespot in the middleistheshunt.
    107. 107.  CSF enterstheshunt system through small holesor slitsnear thetip of theproximal catheter.  AsCSF isproduced by thechoroid plexus, theshunt valve will regulatetheamount of ICPby draining fluid from the ventricles.  From theproximal catheter CSF flowsthrough thevalve system and into thedistal catheter, drainstheCSF into another areawhereit isreabsorbed either directly or indirectly by thebloodstream. Ex. Peritoneal cavity with a VPshunt.  No harm becauseCSF isnormal. Reabsorbed by thesuperior sagittal sinus, alargevenousstructurethat carriestheblood flow away from thebrain.
    108. 108. VPShunt Insertion Reservoir Valve Stastic Tubing Ventricular Catheter Ventriculoarterial Shunt Ventriculoperitoneal Shunt
    109. 109. ValvePressureRatings  ValvePressureSettings:  Most shunt valvesareknown asdifferential pressurevalves.  A valveisself-regulating . They arecapable of gauging theamount of ICPand can adjust to different pressuresbetween theventriclesand thedistal cavity that theshunt drainsinto.  Most common pressureratingsfor differential pressurevalvesare:  Extra-low pressure: 0-10mmH2O  Low 10-50mmH2O  Medium 51-100mmH2O  High 101-200mmH2O  Amount of fluid that isallowed to flow through theshunt valvedependson thespecific design characteristicsof thevalve, aswell asthelevel rating by themanufacturer.  Normal ICPrangefrom 50mmH2O-200mmH2O.  Infantsnormal ICPusually lessthan 60 and lessthan 40 for premature infants.
    110. 110. VPShunt Vs. VA Shunt
    111. 111. Ventriculo-Atrial Shunt (VA)  Shunt tubing ispassed from thevalveto theneck whereit isinserted into a vein. It isthen passed through thevein until thetip of thecatheter (shunt) is in theatrium (achamber) of theheart.  In theheart, theCSF passesinto theblood stream and isfiltered along with other body fluids.  Vascular shuntsfunctioned very well, but they wereproneto multiple problemsincluding early and lateinfection, aswell asrare, potentially fatal heart failuredueto blockageof blood vesselswithin thelungsby particlesof blood clot flaking off theshunt'scatheter tip.  Theuseof theheart hasbeen largely abandoned asan initial choicebecause of theseproblems, but it remainsaviablesecond option when infection or surgery hasrendered theabdominal cavity unaccommodating of thedistal shunt catheter.
    112. 112. Ventriculopleural Shunt  Thechest cavity isanother cavity which can beused asa backup to theabdominal cavity (ventriculopleural shunt).  Occasionally, thiscavity cannot resorb theCSF rapidly and thelung becomescompressed by theexcessCSF resulting in difficulty in breathing. Thecatheter must bemoved to a different cavity issuch cases.
    113. 113. Non-Surgical Treatments  Pharmacological  Acetazolamide(Diamox) and Furosemide(Lasix) Diuretics. Given to control ICPand fluid retention. Temporary relief of increased ICP, but areusually not helpful.  Used to decreasetheproduction of CSF by thechoroid plexusand serial lumbar puncturesof thespineto drain CSF.  Serial lumbar puncturesarepredominantly used on prematurebaby’swho had an intraventricular hemorrhage.  Drain excessCSF within theventriclesof an intraventricular hemorrhage will block CSF flow within theventriclesor in thebasal cistein, causing non-communicating hydrocephalusmaking serial lumbar puncture ineffective.  Non-operational proceduresprovidemoderatesuccessuntil theclient is shunted.
    114. 114. Patient and Family Education  Parents, older children, friendsand roommatesmust betaught thesignsand symptomsof shunt failure.  Persistent headache, emesis, lethargy, changein theneurological exam, visual changessuch asdiplopiaor lossof conjugategaze, or swelling or rednessalong theshunt valveor tubing aresignsthat your child needsmedical attention.  Children arecounseled to avoid contact sportsthat may causeinjury to the shunt valveor head trauma.  Discouragepatientsfrom wearing purses, shoulder bags, or backpackson the sidewhere theshunt tubing passesdown theneck. Continuouspressureon the tubing can causeabreak or kink in thetubing.  Constipation may beafactor in thedevelopment of ashunt malfunction dueto increased abdominal pressure, d/t decreased CSF drainage.  Medical Alert Bracelet.
    115. 115.  Despite the many complications with Susan, Jessica, and baby Isabelle, the family does well  Isabelle continues to grow and learn  Jessica and Susan become even closer in their marriage and say that Isabelle has brought them so much joy and happiness and she has taught them the importance of life  Every day they feel blessed to have her in their lives
    116. 116. Any Questions??
    117. 117. The End