PREAMBLE The Sustainable Development - PowerPoint Presentation

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PREAMBLE

The

Sustainable Development

Goals (otherwise known as the Global Goals)

are

a universal

call to action to end poverty, protect the planet and ensure that all people enjoy peace and

prosperity

They are

a collection

of 17 global goals set by the United Nations in

2015 and are

intended to guide global development until the year

2030

SDG

3: Ensure healthy lives and promote wellbeing for all at all

ages

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GOALS WITHIN A GOAL: HEALTH TARGETS FOR SDG 3

3.1 By 2030, reduce the global maternal mortality ratio to less than 70 per 100 000 live births.

3.2 By 2030, end preventable deaths of newborns and children under 5 years of age, with all countries aiming to reduce neonatal mortality to at least as low as 12 per 1000 live births and under-5 mortality to at least as low as 25 per 1000 live births.

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SOME STATISTICS

Each year about 7.9 million infants (6% of worldwide births) are born with serious birth defects

Each year about

3.3 million children under five years of age die from birth defects each year

Each year

about 3.2

million

of these children are disabled for life

Each year, approximately 300,000 babies are born with neural tube defects resulting in approx. 88,000deaths.

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In low income countries, NTDs may account for 29% of neonatal deaths due to observable birth defects

Birth defects produce severe impact in middle and low income countries where about 95% of these birth defects and 94% of serious birth defects occur

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HOLOPROSENCEPHALY WITH ASSOCIATED OMPHALOCELE AT 24 WEEKS 1 DAY

SEEN

AT THE

O & G DEPT.

KATH

.

COLLINS ANANE SAIH

Dip. Rad Tech

BSc Public Health

BSc

Sonography

OshAcademy

accredited trainer on Health and safety

Slide6

HOLOPROSENCEPHALY

During the third week of embryonic development, mesodermal cells migrates anteriorly to the notochord and are responsible for the induction and differentiation of the spinal cord, the brain and midline facial structures.

An error in this

migration produces a developmental field

defect.

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HOLOPROSENCEPHALY

Holoprosencephaly

describes a variety of

abnormalities of

the brain and face due to incomplete

cleavage and

rotation of the embryonic

forebrain,

the

prosencephalon

, resulting in

a single, centrally located ventricle and a

missing falx

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DORSAL VIEW OF A 23 DASY OLD EMBRYO

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AXIAL TRANSVENTRICULAR PLANE OF FETAL SKULL SHOWING NORMAL STRUCTURES

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TYPES OF HOLOPROSENCEPHALY

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Nomenclature for associated facial malformations

Cyclopia

Single

midline eye

Arrhinia

(absent nose)

Proboscis

may be

present

Ethmocephaly

Severe

hypotelorism

Arrhinia

Proboscis

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CebocephalyHypotelorism

Nose with single nostril

Face with median cleft lip

Cleft lip and palate

Hypotelorism

Flattened nose

Face with median

philtrum

premaxilla

anlage and flat nose

Bilateral cleft lip

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CAUSES OF HOLOPROSENCEPHALY

Poorly controlled maternal diabetes

Genetic causes (chromosomal abnormalities (trisomy 13 and 18 and polyploidy

)

Infections (TORCHS)

Teratogens

(such as alcohol, smoke, retinoic acid, aspirin, misoprostol, and cholesterol-lowering

agents

Idiopathic

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OMPHALOCELE

Congenital

midline abdominal wall defects at the base of the umbilical cord insertion with herniation of gut (or occasionally other content) out of the fetal

abdomen.

This

abnormality is only identified on the second-trimester scans a

s

the midline herniation is normal in a fetus until around gestational age 11-12

weeks

It is due

to the failure of the medial segments of the two lateral embryonic wall folds to fuse at approximately 3-4 weeks post conception.

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General pathogenesis includes Teratogenic

effects

tobacco, alcohol, and other narcotics

early

pregnancy use of

antithyroid

drugsChromosomal anomalies: Trisomy 13 (Edward Syndrome)Trisomy

18

(Edward Syndrome)

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ON ULTRASOUND…..

multiple

bowel loops (and

when the membrane is ruptured, liver, kidneys)

herniate into a membrane-covered defect

The

umbilical cord insertion is directly into the

omphalocoelemay also show evidence of polyhydramnios the abdominal circumference may be smaller as a result

an

allantoic

cyst is often present

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PATIENT’S HISTORY

A 25 year old G1P0 who was referred from a District Hospital

in Kumasi

on account of PPROM.

Patient is ANC attendant at the

District Hospital .

She noticed gradual loss of scanty clear fluid per

vaginum, which later increased in volume (one pad a day to four pads a day)She reported to the district hospital where she was asked to do an ultrasound scan. The findings for that scan was “Fluid filled in the fetal head: Hydrocephalus”. She was therefore referred to KATH for further management

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CONT’D

During patient’s clinical examination at KATH,

BP:

120/60 mmHg

, Hb

:

10.6g/dl,

HIV: -ve,

TT:

2 doses. No

hx

of

STI.

Does not

drink

n

or

smoke.

No history of

contraceptive use.

Pregnancy

was planned.

No

diabetes

or

hypertension

Previous hospital admission

was 3 weeks into the pregnancy on account of Lower abdominal pain

Patient has had an earlier

USG

at 10 weeks 4 days.

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PATIENT HISTORY CONT’D

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PATIENT HISTORY CONT’D

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OBSTETRIC SCAN AT 10 WEEKS 4 DAYS

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OBSTETRIC SCAN AT 34

WEEKS,

2 DAYS BEFORE PRESENTING AT KATH

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PATIENT HISTORY CONT’D

Patient was therefore admitted to A4 ward for further management.

An anomaly ultrasound scan was requested.

Laboratory investigations were also requested:

endocervical

swab R/E and C/S , FBC, BF, urine R/E and C/S.

She was also to screen for TORCH

She was also put on antibiotics treatment

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CLINICAL

NOTES

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CLINICAL

NOTES

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ULTRASOUND AND LABORATORY REQUESTS

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PROTOCOL FOR ANOMALY SCAN

HEAD: lateral Ventricles, Choroid Plexus,

Cavum

Septum

Pellucidum

, Cisterna Magnum, Cerebellum, Nuchal region,

Falx

, Thalami.

FACE: Orbits, Nose/Lips, Facial Profile.

THORAX: Lungs, Four chamber heart, LVOT, RVOT.

ABDOMEN: Stomach, Bladder, Kidneys, Chord Insertion, Bowel, Diaphragm

SPINE: C-spine, T-spine, L-spine, S-spine

EXTREMITIES:

Humerus

/Radius/Ulnar/Hands, Femur/Tibia/Fibula/Foot

.

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THE EXAMINATION

Patient was made to lie supine.

The machine used was Samsung

Sono

Ace R7.

Exam

preset was set to Gen

Obs

with patient’s details entered

The transducer used was curvilinear (2.5- 5MHZ)

Examination approach was

T

ransabdominal

Some knobs especially the gain, depth, TGC and M- mode were manipulated in the course of the exam in order to suit the examination.

Foetal

Biometry

was taken

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VARIATIONS FROM DEPARTMENTAL PROTOCOL

Color Doppler was used to try to analyze the cord insertion

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There is fusion of both lateral ventricles forming a mono ventricle and associated dilatation of the posterior fossa.There is disruption of falx in the mid portion and anterior aspect.The lungs are hypoplastic with moderate bilateral pleural effusion noted

.

There is an anterior abdominal wall defect of about 4.5cm with invagination of all abdominal content through

it together with umbilical cord

The tibia

, fibula and feet are not

visualise

ULTRASOUND FINDINGS

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All other parameters: Face, Femur, Upper limbs, 4-chamber heart, LVOT and RVOT, spine, are all normal.Estimated gestational age – 24W 1D

Presentation – Variable

Amniotic fluid index: Severe Oligohydramnios

CONCLUSION:

Single live intrauterine gestation at 24W 1D with features suggestive of

Omphalocele

with associated Holoprosencephaly .

ULTRASOUND FINDINGS

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USG REPORT

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AXIAL TRANSVENTRICULAR PLANE

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PROTRUDED FETAL BOWEL-Cauliflower-like appearance

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PROTRUDED ABDOMINAL CONTENTS

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PROTRUDED FETAL LIVER AND KIDNEYS

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PROFILE VIEW

Upper lip

Lower

lip

Opening to Oral cavity

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FETAL FOREHEAD, EYES, NOSE, MOUTH

EYE

EYE

FOREHEAD

NOSE

MOUTH

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Normal Fetal lungs

with homogeneous

mid-range

echotexture

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Sagital image of the thorax

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FEMUR

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Loss of liquor was could be due

to

the premature rupture of membrane. This could be caused by infection of

the uterus, cervix, or vagina

, or

cervical

incompetence

The

omphalocele

resulted from

the failure of

the lateral mesodermal

folds to

close during

the

third week of

gestation.

Mono

ventricle

is

as

a result of complete fusion of the lateral ventricles

.

RELATION OF ENCOUNTED PATHOLOGY TO PATIENT PRESENTATION AND INDICATION

Slide48

Failure of the anterior midline cerebral structures to form, and

fusion

of the more lateral dorsal structures in the midline could be as a result of congenital infections like toxoplasmosis or cytomegalovirus infection

The absence

of the

Cavum

Septum

Pelluicidum

resulted from agenesis of the corpus callosum

.

RELATION OF ENCOUNTED PATHOLOGY TO PATIENT PRESENTATION AND INDICATION

Slide49

Pathophysiologic correlation cont’d

Discrepancy

between

ultrasound-calculated, clinically calculated, and calculated

gestational

age using LMP

is as a result of the

fetal

abnormalities. Ultrasound used the femur length which turned out to be

deformed.

Malformed

lower limbs might be as a result

of chromosomal abnormality

The hypoplastic lungs might be as a result

of the

pleural

effusion

or

as a result of the severe oligohydramnios

Slide50

FOLLOW UP

Patient was monitored at the ward and induction of labor was started on her 4

th

day at the ward at 10:30am.

50µg misoprostol was administered sublingually and was repeated in the evening and on the following morning(a

total of 150µg administered).

Patient reached 2

nd

stage of labor at 12:10pm.

Fetus was in breech position and after 45 minutes, the trunk and the shoulders were delivered but the head was trapped.

Slide51

FOLLOW UP cont’d

An attempt to puncture the anterior fontanel with a forceps failed and so decision was taken to do cephalocentesis.

A 16 G cannula was used to puncture the fetal skull through the right parietal bone transabdominaly under

ultrasound

guidance.

Clear hydrocephalic fluid was drained until the head drastically reduced in size and the fetus and placenta delivered.

.

Slide52

POST OPERATIONAL FINDINGS

Fresh still baby with multiple congenital malformations:

Anterior abdominal wall has terminated viscera involving the intestines, the kidneys, the liver through a defective anterior abdominal wall with ruptured membrane cover.

Malformed lower limbs.

Collapsed hydrocephalic head

Undetermined sex

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OPERATIONAL NOTES

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OPERATIONAL NOTES

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OPERATIONAL NOTES

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DOCTOR’S LABOUR REPORT

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NURSE’S NOTES ON DELIVERY

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POST DELIVERY FINDINGS

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Patient was monitored at the labor ward for two days and was discharged on of November and was asked to return for reviewShe is currently doing well with no clinical symptoms through phone calls placed to her.

FOLLOW UP cont’d

Slide60

“Gastroschisis”: This findings could not be entirely correct since the abdominal defect is in the midline at the umbilicus. The defect was big enough to allow protrusion of almost all abdominal contents.

“Hydrocephalus”: Since the team did not have access to the internal brain structures, they could only use hydrocephalus

COMMENTS ON DELIVERY

FINDINGS

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DIFFERENTIAL DIAGNOSIS

Gastroschisis

Hydranencephaly

Patau

syndrome

:Trisomy

13 (

Patau

Syndrome): Holoprosencephaly, and other neural tube defects,

Ventriculomegaly

Slide67

REFERENCES

Callen

, P.W.,(2007). Ultrasonography in Obstetrics and

Gynaecology

(5th Ed) Philadelphia, PA.

Elsvier

Hagen-

Ansert

, S.L., (2012). Textbook of Diagnostic

Sonography

(7th Ed). Mosby St. Louis, Missouri

https

://

radiopaedia.org/article/holoprosencephaly

http://www.who.int/sdg/targets/en/

Rumack

,C. M ., Wilson , S.R.,

Charboneau

, J. W, Levine, D. (2011). Diagnostic ultrasound (4th Ed). Philadelphia, PA.

Mosby

World Health Organization. Global health estimates (GHE)–Cause-specific mortality. 2015.

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THANK YOU

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HYDRANENCEPHALYComplete or near complete absence of the cerebral cortexThe thalami, lower brain centers, and

cerebellum are

usually intact.

Slide74

TRISOMY 13 (PATAU SYNDROME):SONOGRAPHIC FINDINGSHoloprosencephalyNeural tube defects with skeletal abnormalities

Facial clefts