Hydrocephalus with fetal hydrops

CLINICAL

SEMINAR CASE

PRESENTATION

4/26/2014 ADU-POKU PATRICK

Clinical Case Case Of A Hydrocephalus And

Associate Hydrops Fetalis In A

Single Viable Intrauterine

Gestation Of About 33w2d, In A

26-year Old Woman.


Patient Clinical History

Current History :

• A 26-year-old pregnant woman presented with

chronic lower abdominal pains, pruritus, burning

sensations and swelling.

• First pregnancy and with no history of abortion.

• Abdomen was large for GA

Previous Medical History:

• No history of heart /kidney disease, chronic

hypertension or diabetes mellitus

• Rhesus factor : D(+). Blood Group: O(RH)

• Sickling: (-)


REFERRING PHYSICIAN

REQUEST:

• ?? Hydrocephalus ?? Severity.

• Do obstetric scan.

• USG REQUEST ST, PATRICKS HOPITAL

OFINSO.pdf


USG REQUEST ST, PATRICKS HOPITAL OFINSO.pdf

USG REQUEST ST, PATRICKS HOPITAL OFINSO.pdf

Ultrasound Findings From Her

Previous Examination

• First trimester USG Date: 15/5/2013

• GA: 11w2d EDD: 2/12/2013 CRL-4.4cm

• Comment : normal early OBS USG.

SONOGRAM

2nd USG Examination(3rd tri) Date:20/11/2013

GA: 37w4d(HC/BPD) FH-138BPM

EDD: 07/12/2013 Cephalic presentation

Hydrocephalus noted. SONOGRAM


1ST TRIM USG SONOGRAM ST.PAT H.pdf

2nd SONOGRAM ST PAT H.pdf

Department Protocol for Second

and Third Trimester OB USG

• Survey uterus and determine fetal number.

• Observe fetal cardiac activity.

• Determine fetal position(s) and placental

location(s).

• Check cervix and lower uterine segment.

• Survey for uterine or adnexal masses.

• Assess amniotic fluid.

• Perform anatomy survey of each fetus.

• Perform biometric measurements of each fetus.

If anomaly is detected protocol is varied.


The Examination

Patient in supine position

Machine Exam preset: Gen. OB

Transducer: curvilinear (2- 5MHZ) Fetal

Trans-abdominal approach

Biometry taken (i.e. HC, BPD, AC, FL and

EGA)

For sex determination patients are asked to

vary their position (in terms of difficulty)

There is variation of protocol and examination

in terms of twin gestation.


Ultrasound findings: GENDER


Ultrasound findings: FL


Ultrasound findings


GA from Fetal Biometry

• The fetal biometry showed significant

growth inconsistencies

• BPD-More than 41weeks

• HC -39weeks

• AC- 35weeks

• FL- 33weeks 2days

TABLE


GA TABLE i.pdf

Ultrasound findings: FETAL

HEAD

• Intact skull

• Altered falx

• Extensive Intra cranial fluid collection with

little remnant of cortical mantle bilaterally

(residual brain tissue≥16mm)

• Fetus lie in cephalic presentation with fetal

head close to the maternal cervix.


RESIDUAL BRAIN TISSUE


Fetal Presentation


Ultrasound findings: ABDOMEN

• Diffuse skin thickening (Generalized body

Edema)

• Fetal Ascites

• Intact diaphragm

• Normal cord insertion

• Intact stomach

• Hepatomegaly

• Urinary bladder intact


Ultrasound findings


ASCITES


INTACT DIAPHRAGM


NORMAL CORD INSERTION


INTACT STOMACH


URINARY BLADDER


OTHER FINDINGS

• Heart occupying about 3/4 of chest cavity

(CARDIOMEGALY) how ever no visible

defect and effusion noted.

• Normal Sonographic appearance of :

Nose and lips

Limbs and digits

Spine was also intact

Adequate AFI (16.4cm)


Other findings


ADEQUATE AFI


Posterior high thick placenta


CRITIQUE OF

IMAGES/INADEQUACIES OF

STUDY

• Color Doppler was not demonstrated through

out the study.

• Though spine was assessed but images on them

was not shown.


RELATION OF ENCOUNTERED PATHOLOGY IN

RELATION TO PATIENT PRESENTATION AND

INDICATIONS

• Extensive intra cranial pressure 2⁰ extensive fluid collection with altered falx and bilateral little remnant of brain cortical tissue (BPD/HC >>AC/FL) detected on sonogram is an evidence of severe hydrocephalus as suspected by the referring clinician.

• Diffuse skin thickening (Generalized body Edema),Fetal ascites, Hepatomegaly, Cardiomegaly and edematous placenta noted on sonogram as an incidental findings, per patients history is consistence with Non- immune fetal hydrops.




INDICATIONS

• Normal umbilical cord insertion

• Intact spine, stomach, diaphragm, nose,

lips, limbs, urinary bladder and adequate

AFI, Cephalic presentation.

• No visible atrial-septal defect noted on

sonogram is a suggestive feature of

systemic malfunctioning of the

underlying condition under discussion

rather than chromosomal anomaly.




INDICATIONS

• Large fetus(macrosomy) with

much pressure exerting on mothers

iliac vessels as well as the

lymphatic drainage channels is an

indicative feature of mothers

manifestation of lower abdominal

pains and swellings of the feet as a

result of poor venous returns and

lymphatic drainage.


CONCLUSIONS DRAWN

• After discussing case with my

supervisor we came to a

conclusion that the findings

were consistent with

hydrocephalus and suggestive

fetal hydrops in a single live

intrauterine gestation of about

33w2d.


Ultrasound Exams Final Report

Ultrasound Report

Differentials

Hydranencephaly

Holoprosencephaly

DIAGRAM


USG REPORT.docx

Presentation DIAGRAM.pdf

Follow-up • Follow up was done through phone calls.

• 21/11/2013- patient seen @ KATH

• 25/11/2013- Patient was reviewed at St.

Patrick's Hospital Offinso, on interaction with

her Physician clinician he said patient is under

going counseling for termination of pregnancy

by cesarean section. If she agrees pregnancy

will be terminated and folic acids will be given

on her next and subsequent pregnancies.

• Patient shown consent upon the counseling and

opted for the termination by C/S


CONTINUAT

ION

• 26/11/2014-Patient was admitted, on

examination by an attendance nurse

fetal heart was absent on fetoscope.

• 27/11/2014- An Emergency C/S was

done on patient with intrauterine fetal

demise at 34w1d with macrocephaly

and anasarca, baby(female) weighing

more than 4kg

• Patient doing well and discharged from

the hospital after few days.


Presumption made from the entire

study up to follow up

• Extensive brain damaged 2⁰ intracranial pressure from extensive hydrocephalus> congestive heart failure > malfunctioning system > fetal hydrops > intrauterine fetal demise.

• NB: Death may set in as a results of tonsillar herniation 2⁰ raised intracranial pressure with compression of brain stem and subsequent respiratory and cardiac arrest.


DISCU

SSION

• Hydrocephalus is defined as excess cerebrospinal fluid

(CSF) accumulation in the head caused by disturbance of

formation, flow, or absorption. The term stems from the

Greek hydro (water) and cephali (head). Davis et al,

• The incidence of hydrocephalus is 0.3 to 1.5 per 1000 live

births. This obstruction may be caused by a ventricular

defect, such as aqueductal stenosis, and is referred to

as noncommunicating hydrocephalus. The obstruction

may be noted outside of the ventricular system, such as

with an arachnoid cyst, and is referred to as

communicating hydrocephalus(Sandra Hagen page 1307)

• Generally, incidence is equal in males and females. The

exception is Bickers-Adams syndrome, an X-linked

hydrocephalus transmitted by females and manifested in

males. NPH has a slight male preponderance.(Vinchon et.

al)


• Ventriculomegaly refers to dilatation of the ventricles within

the brain measuring (10-15mm). Hydrocephalus occurs when

ventriculomegaly (15mm>) is coupled with enlargement of the

fetal head.(Sandra hagen)

• Normal CSF production is 0.20-0.35 mL/min; most CSF is

produced by the choroid plexus, which is located within the

ventricular system, mainly the lateral and fourth ventricles. The

capacity of the lateral and third ventricles in a healthy person is

20 mL. Total volume of CSF in an adult is 120 mL.

• Normal route of CSF from production to clearance is as follows:

From choroid plexus>CSF>lateral ventricle, > interventricular

foramen of Monro>third ventricle>cerebral aqueduct of

Sylvius>fourth ventricle>2 lateral foramina of Luschka and 1

medial foramen of Magendie, the subarachnoid space, the

arachnoid granulations, the dural sinus, and finally into the

venous drainage.


• ICP rises if production of CSF exceeds absorption. This occurs if CSF is

overproduced, resistance to CSF flow is increased, or venous sinus pressure is

increased. CSF production falls as ICP rises. Compensation may occur through

transventricular absorption of CSF and also by absorption along nerve root

sleeves. Temporal and frontal horns dilate first, often asymmetrically. This may

result in elevation of the corpus callosum, stretching or perforation of the septum

pellucidum, thinning of the cerebral mantle, or enlargement of the third ventricle

downward into the pituitary fossa (which may cause pituitary dysfunction).

• Associated CNS and somatic malformations are common and seen in about

70% to 83% of cases. Chromosomal abnormalities are more common with

nonisolated (25%-36%) than isolated (3%-6%) VM and include the

trisomiesand X-linked hydrocephalus in males. D’Addario et al. 37 reports that

the main associations with VM are aqueductal stenosis (30%-40%), Chiari II

malformationwith spina bifida (25%-30%), Dandy-Walker complex (7%-10%),

agenesis of the corpus callosum, and other, less common conditions.


• Fetal hydrops refers to the pathologic accumulation of fluid in body

cavities and tissues. US demonstrates ascites, pleural and pericardial

effusions, and subcutaneous edema. Immune hydrops is caused by

blood group incompatibility between mother and fetus. Current

treatment, including fetal transfusion, is highly successful.

Nonimmune hydrops is caused by a host of conditions including

cardiac disorders, infections, chromosomal anomalies, twin

pregnancy, urinary obstruction, and umbilical cord complications.

The cause of many cases is not identified. The prognosis for

nonimmune hydrops remains poor.(Goldstein et al)

• Preterm birth at less than 34 weeks' gestation and a serum albumin

concentration level of less than 2 g/dL are poor prognostic factors for

survival. A more recent study found that there is strong association

between gestational age, the presence of 2 or more serous cavity

effusions, and poor outcome in infants with hydrops fetalis (Takci et

al)


Role of ultrasound in detecting hyrocephalus/fetal hydrops

• Currently, most fetuses undergo in utero ultrasonographic scanning.

Ultrasonography allows for good identification of any ventricular dilatation that

indicates active hydrocephalus. In such cases, or in patients who present with

progressive head enlargement to pediatricians or family doctors, ultrasonography is

usually performed first because it is widely available and does not expose the Fetus

or child to ionizing radiation.

• In babies with open fontanelles and large heads, ultrasonography reveals the

enlarged ventricular system and any mass lesions or hemorrhage. However, the

anatomic detail produced by ultrasonography remains reduced and serves only as a

guide to further investigations.

• Fetal Hydrops. Image through the fetal thorax at the level of a four-chamber view

of the heart will demonstrates large bilateral pleural effusions.US of the fetal

abdomen will also shows marked skin thickening. Fluid collection in at least two

body cavities to qualify hydrops, other findings in hydrops can include

subcutaneous edema, polyhydramnios,and placentomegaly in which US is very

sensitive in picking.

• (T).(BOOK SOURCE FOUNDAMENTAL OF DIAGNOSTIC RADIOLOGY 4TH

ED BY WILLIAM E. BRANT AND CLYDE A. HELMS.)


Reference

s

Davis BE, Daley CM, Shurtleff DB, Duguay S, Seidel K, Loeser

JD, et al. Long-term survival of individuals with

myelomeningocele. Pediatr Neurosurg. Jul-Aug 2005;41(4):186-

91. [Medline].

Hagen-Ansert, S. L. (2012). Textbook of diagnostic sonography 7th

edition.

Vinchon M, Rekate HL, Kulkarni AV. Pediatric hydrocephalus

outcomes: a review. Fluids Barriers CNS. Aug 27 2012;9(1):18.

[Medline].

Rumack. (2011). Diagnostic ultrasound 4th edition.

Bullen PJ, Rankin JM, Robson SC: Investigation of the

epidemiology and prenatal diagnosis of holoprosencephaly in the

North of England, Am J Obstet Gynecol 184:1256-1262, 2001

Goldstein R. A practical approach to fetal chest masses.

Ultrasound Q 2006;22:177–19426.

Takci S, Gharibzadeh M, Yurdakok M, Ozyuncu O, Korkmaz A,

Akcoren Z, et al. Etiology and Outcome of Hydrops Fetalis: Report

of 62 Cases. Pediatr Neonatol. Oct 1 2013;[Medline].





Health & Medicine

Hydrocephalus with fetal hydrops