Vein of GalenClinical Case Presentation

Marissa Hampton SNNP, RN, BSN

University of Texas Medical Branch at GalvestonSchool of Nursing

NNP Concepts and Practicum IIGNRS 5632

Dr. Debra Armentrout PhD, RN, MSN, NNP-BCDr. Leigh Ann Cates PhD, APRN, NNP-BC,RRT-

NPS,CHSE

ObjectiveIdentify the high risk neonateIntegrate pertinent patient information in developing a plan of care.

Review the infants systems by hospital course.

Discuss current theory and research as related to the infants primary medical diagnosis

Maternal History Caucasian, 25 year old mother Gravida 2, Para 1

History of previous miscarriage at 8 weeks with same father of infant Estimated date of delivery: 5/1/2014 Adequate prenatal care

Known vein of Galen malformation seen on prenatal ultrasound Including follow up with pediatric cardiologist

O + blood type Negative maternal labs

Human Immunodeficiency Virus (HIV) Unknown maternal labs (awaiting arrival from office)

Group Beta Strep (GBS) Rapid plasma reagin (RPR) serology Rubella Hepatitis B status (HBsAg)

Denies tobacco, alcohol and illicit drug use Married

Maternal and Fetal Risks and Complications

Vein of Galen malformation Potential fetal complications Most complications arise after delivery due to altered blood flow and

vascular resistance Congestive Heart Failure Pulmonary Hypertension Myocardial ischemia Cerebral edema Renal failure Obstructive hydrocephalus

Related to size, position of malformation and compression on brain structures

(Hoang, Choudhri, Edwards & Guzman, 2009)(Gomella, Cunningham & Eyal, 2013)

(Geneva Foundation for Medical Education and Research, 2014)

Maternal and Fetal Risks and Complications

Unknown maternal labs Maternal risk

Low suspicion due to adequate prenatal care Possible untreated sexually transmitted disease, group b strep

infection or liver damage Fetal risk

At risk for Hepatitis B transmission At risk for GBS septicemia At risk for congenital syphilis At risk for congenital abnormalities due to rubella infection

Delivery and stabilization Elective Cesarean Section

Due to Congenital Anomalies Epidural Anesthesia Rupture of Membranes at delivery with clear fluid Vertex Presentation Apgars

8 at one minute of life 9 at 5 minutes of life

Delivery summary: Infant pink, crying, vigorous at delivery Drying and tactile stimulation provided Adequate respiratory effort, with initial HR >100 Infant remained stable with good tone, reflex, color and adequate

respiratory effort through out stabilization and during transfer to NICU

Admission Assessment and Diagnostics

Birth Gestation 38.4 weeks Gender: Male Birth weight: 3450 grams (51-75%tile) Head circumference: 35.5 cm (76-90%tile) Length: 52 cm (76-90%tile) Admission vital signs

Temperature 99.3 F Heart rate 172 bpm Respiratory rate 44, O2 saturation 91% on 4 liters per minute (LMP) Blood pressure: 57/34 (40)

Admission Physical Exam: General: Active and alert, responsive to exam. Head/EENT: Bilateral red reflexes noted. Normocephalic. AFSF. No cleft deformities.

Bruit present in anterior fontanels Lungs: Clear bilateral breath sounds with mild subcostal retractions. Heart: Regular rate and rhythm, without murmur, pulses 3+. Decreased perfusion. Abdomen: Soft, round, active bowel sounds. No organomegaly. 3 vessel cord. Genitalia: Normal external male genitalia with bilateral descended testicles. Patent

anus. Spine intact. Extremities: Full range of motion in extremities. Hips without click bilaterally. Skin: Pink and warm without pathologic lesions.

Admission Assessment and Diagnostics

Diagnostics: Chest x ray

Clear bilateral lung fields with adequate lung volumes, expansion to T 9 Enlarged cardiac silhouette

Head ultrasound Vein of Galen malformation 12 mm in diameter

Admission Blood Culture Results pending for 48 hours- Negative

Complete blood count (CBC) White blood cell count: 9.8 Hemoglobin/ Hematocrit: 12.3% / 36.9% No left shift

Blood glucose Initial: 37 mg/dl Repeat following bolus: 47 mg/dl Follow up blood sugar after second D10 W bolus and initiation of IVF: 75mg/dl

Arterial Blood gas Initial pH 7.28 pCO2 59.2 pO2 49 HCO3 27.6 BE 1 Mild respiratory acidosis

Primary Admission Diagnosis

38 week AGA male infantArteriovenous Malformation- Vein of GalanTransient HypoglycemiaRespiratory DistressSuspected sepsis

(Geneva Foundation for Medical Education and Research, 2014)

Etiology and Pathophysiology of primary admission diagnosis

Etiology: ~ 1 in 25,000 Vein of Galen

Arteriovenous (AV) malformation with shunting of blood Improperly named Persistent embryonic vein Congenital aneurysmal malformation of the vein of

Markowski Aneurysm forms in stages with development of cerebral

vascular system Stage I: Neural cells/tube receive nutrients from amniotic fluid Stage II: Neural matter/ vascular channels develop Stage III: Developing brain matures, cerebral veins develop and

drain into the vein of Markowski. Vein of Markowski should involute; persistence of great vein is now termed Vien of Galen.

(Hoang, Choudhri, Edwards & Guzman, 2009)(Li, Armstrong, terBrugge, 2011)

(Sheth, 2014)

Etiology: ~ 1 in 25,000 Vein of Galen

Arteriovenous (AV) malformation with shunting of blood Improperly named Persistent embryonic vein Congenital aneurysmal malformation of the vein of

Markowski Aneurysm forms in stages with development of cerebral

vascular system Stage I: Neural cells/tube receive nutrients from amniotic fluid Stage II: Neural matter/ vascular channels develop Stage III: Developing brain matures, cerebral veins develop and

drain into the vein of Markowski. Vein of Markowski should involute; persistence of great vein is now termed Vien of Galen.

Etiology and Pathophysiology of primary admission diagnosis

Vein of Galen is located under the cerebral hemispheres Drains the anterior and central regions of the brain into the

sinuses of the posterior cerebral fossa. Lesion is midline and receives bilateral blood supply from

collateral circulation Two types of AV malformation

Choriodal malformation Leads to severe vessel malformation and symptoms Extensive arterial network

Mural malformation Multiple high flow shunts that end within the vein of

Markowski Occurs 6th- 11th week of gestation

(Khullar, Andeejani, & Bulsara, 2010)

Etiology and Pathophysiology of primary admission diagnosis

Pathophysiology In utero

Low resistance rarely After birth

Changes in blood flow due to changing resistance Cerebral venous congestion causes:

Abnormal cerebral spinal fluid (CSF) flow High blood flow shunts to the vein of Galen and reduces flow to the cerebral

parenchyma Decreased blood flow to the brain results in cerebral edema May cause rapid loss of brain tissue Leads to impaired resorption of CFS resulting in hydrocephalus

Cardiac failure due to increased venous return Low resistance venous shunt to vein of Galen increases venous return to right

atrium Increased pulmonary blood flow causes pulmonary hypertension Congestive heart failure

(Hoang, Choudhri, Edwards & Guzman, 2009)

Initial Plan of Care Admit infant to NICU via radiant warmer. Cardiopulmonary monitoring, pulse oximetry monitoring. Admission weight, length, frontal occipital circumference (FOC). Admission vital signs every (q) 15 minutes x4, q 30 minutes x 4, q

hourly x 4, then q 3 hours. Admission 4 point blood pressures (bp). Strict intake and output. Infuse D12.5W + 1 meq Ca gluconate/100ml + 0.25 units of heparin/

ml via umbilical venous catheter(UVC) to run at 10 ml/hour. Infuse ½ Normal Saline(NS) + 0.25 units of heparin via umbilical

arterial catherter (UAC) at 1 ml/hr. Total IVF ~ 80 ml/kg/hr. Monitor bp via UAC. Notify if mean bp is <35 or >65. Administer 6.9 ml of D10 IV bolus x 1, repeat bedside blood sugar

level in 30 minutes. Initiate feeds of expressed breast milk or Similac Advance 20

kcal/oz 15ml q 3 hours by mouth (PO) or orogastric tube (OGT)

Initial Plan of care 4 liters per minute (LPM) high flow nasal cannula (HFNC) Titrate Fio2 to remain in target saturations (92-96%). Stat Chest /abdominal x-ray. Routine echocardiogram. Stat head ultrasound. AM magnetic resonance imaging (MRI) and MRA Obtain maternal labs. Begin Lasix 1mg/kg PO q 24 hours Obtain and follow admission lab work:

Blood culture, Complete blood count (CBC), C-reactive protein (CRP)

Q 6 hour bedside glucoses once blood sugars are stable In AM obtain:

Basic metabolic panel (BMP), Total/direct bilirubin level, CBC, CRP

Hospital course by systemNeurology Known vein of Galen malformation

Detected in utero via ultrasound Head ultrasound(HUS) performed on admission

12 mm in diameter Mild cardiomegaly in conjunction with malformation via echo Pulsating anterior fontanel with bruit

MRI/MRA performed on admission MRI revealed vein of Galen malformation with posterior feeding

vessels No hydrocephalus

Pediatric Neurosurgery/ Neuroradiology consulted Planned ablation in 2-6 months due to stable aneurysm

Follow up HUS at 3 weeks of age No hydrocephalus

Hospital course by systemPulmonology Initial respiratory distress after delivery

Possibly due to retained lung fluid from elective c section Required up to 4LPM due to desaturations (Fio2 titrated)

Development of pulmonary edema secondary to cardiac failure from vein of Galen malformation Low dose Lasix continued Improvement of capillary blood gases with decreased Fio2 needs

Infant tolerated daily flow wean on nasal cannula (NC) Inhaled nitric oxide initiated for probable persistent pulmonary hypertension (PPHN)

Confirmed by echocardiogram iNO initiated after a dysrhythmia event, increased flow and Fio2 requirements elevated brain

natriuretic peptide (BNP) and worsening echocardiogram results Trial recommended by cardiology, short term use

Gradual wean of iNO and NC Follow up echocardiograms obtained

Room air status Post operative O2 needs after G button replacement

Evidence of pulmonary edema and cardiomegaly Continued Lasix administration, NC, fluid restriction

Room Air status Sildenafil initiated

Hospital course by systemCardiology Known arteriovenous malformation

Vein of Galen diagnosed in utero via ultrasound Enlarged heart seen on x ray

Lasix administration initiated BNP monitored

Admission Echocardiogram Revealed moderate patent ductus arteriosus (PDA), mild PPHN and dilated right ventricle

Repeat Echocardiogram the next day Closing PDA with right to left shunting Suprasystemic PPHN

Initiated trial of iNO at 20 ppm Dilated right ventricle

Episode of Supraventricular tachycardia noted Resolved with vagal maneuver Cardiology consulted

Follow up echocardiogram Improving contractility and decreased pulmonary pressures BNP continues to improve

Final echocardiogram PDA is closed Some persistent PPHN with bidirectional shunting via the PFO/ASD Home regimen of sildenafil

Hospital course by systemsGastrointestinal system:

Initial hypoglycemia resolved with two D10 boluses D10 W IVF administration via UVC

Small bolus feeds initiated on DOL 1 Initially at 40 mg/kg/day then advanced daily to 160 mg/kg/day Hypoglycemia resolved

Hyponatremia acquired secondary to lasix administration Supplemental NaCl initiated in feeds

PO ad lib feeds prior to discharge Poor feeder Infant failed 2 day rooming in process by only completing 125 m/kg/day Insufficient volume and caloric intake

Family decides on G button placement Consulted with pediatric surgery G button placement for home management PO adlib feeds with remainder of volume given via g button

Hospital course by systemGenitourinary

UnremarkableMusculoskeletal

UnremarkableHematological

Unremarkable Adequate hct levels No transfusions during hospital stay

Ophthamology Unremarkable

Infectious disease Initial blood culture negative at 48 hour Once obtained all of mothers prenatal labs were negative Low risk for suspected infection

MedicationsOn admission:

Vitamin K intramuscular (IM) injection, 1 mg x 1 Erythromycin ophthalmic eye ointment OU x 1

Lasix Began on admission for congestive heart failure 1 mg/kg PO q 24 hours, then increased to 1 mg/kg PO q 12 hours

History of: Normal saline bolus IV at 10mg/kg at admission D10W IVF for initial hydration at admission Sodium chloride for hyponatriemia

2 mEq PO q 8 hours, then increased to 4 mEq PO q 8 hours Ancef prior to g button placement

100 mg IV q 8 hours Morphine for pain control post op

0.1 mg/kg IV Q 4 hrs PRN Versed for agitation prior to MRI

0.25 mg IV

Medications Hepatitis B vaccine 5mcg IM x 1Current medications

Sildenafil 0.5 mg/kg q 6 hours PO Tylenol 40 mg/kg PRN for pain related to circumcision

Pertinent theories and Evidence based practice

Vein of Galen Composes about 1% of all intracranial vascular lesions

About 30% of these lesions are discovered in neonates Historically, grave prognosis

First case described in 1949 As of 1982, infant with vein of Galen had a 100% intraoperative mortality

rate Development of endovascular techniques began in early 1980s By 1991 mortality rate for endovascular procedures decreased to 50% By 1993 mortality rate decreases dramatically

Due to advances in embolization technique Although mortality rates decreased dramatically, normal cognitive

development was not always preserved Currently neurological function with therapy may be abnormal in 50 % of

survivors.

(Zahka & Gruenstein, 2013)(Khullar, Andeejani & Bulsara, 2010)

(Heuer, Gabel, Beslow, Stiefel, Schwartz, Storm., … & Hurst, 2009)

Pertinent theories and Evidence based practice

Multiple studies emphasize that untreated infants have a poor prognosis Mortality rate of 75 %

Prognosis is related on age Infants who require endovascular treatment within the first month

of life have the worse prognosis Highest rates of mortality and neuro/developmental delays

Highest success rates if embolism is prolonged to 5-6 months of age Increased efficacy of embolization procedure Small risk to brain maturation

Reports of spontaneous resolution are rare

(Khullar, Andeejani, & Bulsara, 2010)(Geneva Foundation for Medical Education and Research, 2014

Pertinent theories and Evidence based practice

Improvements in endovascular therapy, antenatal diagnosis and neonatal critical care have improved outcomes

First consider medical management Treat congestive heart failure

Inotropes for renal stability Diuretics to decrease cardiac load Fluid restriction

Endovascular treatment Goal is to obliterate lesion by endovascular occlusion ~ 60% of neonates with endovascular treatment have had minimal

to no developmental delay at follow up Complete occlusion is not necessary Partial embolization may cause thrombosis

Surgical treatment is only used with embolization failure Primary treatment was open microsurgery Low success rate (Blount, Oakes, Tubbs & Humphreys, 2006)

(Moftakhar, Damielpour, Maya & Alexander, 2009)

Family interaction Mother and father of baby remained closely involved in

infants care. Family visited daily and participated in infants cares such as feeding, bathing and bonding. Parents participated in a two day room in prior to the first anticipated discharge date. Infant failed to complete feeds and only took about 120 ml/kg/day. Parents discussed at length infants feeding options with his primary neonatalogist and opted for a planned G button. Surgery for button placement was made for the end of the week. Infant’s rooming in was cancelled, and plan of care continued until surgery. Post operative care and problems were discussed with the family at length. Parents remain cooperative through out the infants stay and plan of care.

Discharge plan and follow up Discharge Plan

Infant is not currently ready for discharge, but upon discharge will: Continue PO adlib feeds, g button feed when infant does not finish

volume Circumcision care after surgery (g button placement) Hearing screen passed Discharge infant home in care of parents

Follow up appointments Pediatrician appointment Follow up as outpatient with surgery at one month for gbutton

placement Follow up with interventional neuroradiology at 2 months of life to

consider ablation Follow up with pediatric neurosurgery at 2 months of life to

consider ablation

Summary Early intervention is necessary to avoid a poor prognosis. Medical management should always be considered first. Avoidance of embolization until the 5th-6th month of life has

most desirable outcomes. Avoiding surgery can only be accomplished if infant remains

hemodynamically stable. Interdisciplinary team should be established to guide infants

care. Includes Neonatologist, Neuroradiology, Neurosurgery, and

nursing.

References Blount, J. P., Oakes, W. J., Tubbs, R. S., Humphreys, R. P. (2006). History of surgery for cerebrovascular disease in children. Part II. Vein of Galen malformation. Neurosurgery Focus (6), 1-6. Geneva Foundation for Medical Education and Research (2014). Vein of Galen

malformation. Retrieved from http://www.gfmer.ch/genetic_diseases_v2/gendis_detail_list.php?cat3=42

Gomella, T. L, Cunningham, M. D. & Eyal, F. G. (2013). Neonatology: Management, procedures, on-call problems, diseases and drugs (7th Ed.). New York: McGraw Hill Education

Heuer, G. G., Gabel, B., Beslow, L. A., Stiefel, M. F., Schwartz, E. S., Storm, B. P., Ichord, R. N., & Hurst, R. W. (2009). Diagnosis and treatment of vien of Galen malformations. Childs Nervous System. doi: 10.1007/s00381-009-1063-8

Hoang, S., Choudhri, O., Edwards, M., & Guzman, R. (2009). Vein of Galen malformation. Neurosurgery Focus, 27 (5), 1-6.

doi: 10.3171/2009.8FOCUS09168 Khullar, D., Andeejani, A., Bulsara, K. R. (2010). Evolution of treatment

options for vein of Galen malformations. Journal of Neurosurgery: Pediatrics 6, 444-451. doi: 10.317/2010.8.PEDS10231

Li, A., Armstrong, D., terBrugge, K. G. (2011). Endovascular treatment of vein of Galen aneurysmal malformation: management strategy and 21 year experience in Toronto. Journal of Neurosurgery: Pediatrics (7), 3- 10. doi: 10.3171/2010.9.PEDS0956

References Moftakhar, P., Danielpour, M., Maya, M., & Alexander, M. J. (2009).

Spontaneous thrombosis of neonatal vein of Galen malformation. Neurosurgery Focus, 27(5), 1-5. doi:10/3171/2009/.8.FOCUS09162)

Sheth, R. D. (2014). Vein of Galen malformation. Medscape Retrieved from http://emedicine.medscape.com/article/1179888-overview#showall

Zahka, K. G., & Gruenstein, D. H. (2011). Approach to the neonate with cardiovascular disease. In R. J. Martin, A. A. Fanaroff, & M. C.

Walsh (Eds.), Neonatal-Perinatal Medicine: Diseases of the fetus and infant (9th ed., pp. 1215-1222). Philadelphia, PA: Mosby Elsevier