INTUSSUSCEPTION

Failure to Thrive

17
01/23/23 Failure to Thrive 18
Shannon Pittman, M.D.
01/23/23 Failure to Thrive 19
Shannon Pittman, M.D.
http://www.peacecorpsonline.org/messages/messages/2629/1008996.html

01/23/23 Failure to Thrive 20

Shannon Pittman, M.D.
 http://bluegoldfish.blogs.com/surface/2004/05/present_from_pr.html

01/23/23 Failure to Thrive 21

Shannon Pittman, M.D.
01/23/23 22
 Recap - Classification
Failure to Thrive

Organic Nonorganic

Prenatal Postnatal Prenatal Postnatal

Malnourished
Toxic Exposure Inborn errors
mother
Abuse/Neglect

01/23/23 23
 FTT - Workup
• +/- Basic screening labs
– CBC, Chemistry, & UA
• Specific test directed by history
– HIV, ESR, TSH, Sweat chloride test, serum
IGF-I, serum IgA/IgG antigliadin antibiodies
• X-rays for bone age

01/23/23 24
 FTT – Treatment
• High calorie diet for catch up growth
– 150% of recommended daily caloric intake
based on expected wgt
• +/- Feeding behavior modification
• Psychosocial involvement/ intervention
• Close follow up
– Physical and cognitive delays
• Hospitalization when necessary

01/23/23 25
 References
• Listernick, R. (2004). Accurate feeding history key to failure to thrive.
Pediatr Ann, 33:3, 161-9.
• Burgos, R., Jutte, D. (2000). Resident’s column: “doctor, is my child
growing ok?”. Pediatr Ann, 29:9, 585-7.
• Krugman, S., Dubowitz,H. (2003). Failure to thrive. American Fam
Phy, 68:5, 879-84.
• Schwartz, R., Abegglen, J. (1996). Failure to thrive: an ambulatory
approach. Nurse Pract, 21:5, 19-31.
• Careaga, M., Kernder, J. (200). A gastroenterologist’s approach to
failure to thrive. Pediatr Ann. 29:9, 558-67.
• Bassali, R., Benjamin, J. (2004, August 11). Failure to Thrive.
eMedicine. Retrieved September 17, 2005, from
http:///www.emedicine.com/ped/topic738.htm.

01/23/23 26
 Sudden Infant Death
Syndrome
SIDS
SIDS is the largest known cause of death in
children under the age of one. To date there
is no known cure, or reason for that matter,is
to why some children are affected while
others aren't.
Over 2,500 babies die in the US
each year from SIDS
Although the number of deaths have been lowered by
around 40% by taking several preventative approaches
and incorporating baby sleep safety methods to safe guard
children from SIDS, there is no known way to fully avoid
the occurrence of SIDS.
 SIDS FACTS

• 4500 infants die of no obvious cause in the U.S. annually

• Of these, 50% are due to SIDS
• SIDS is the leading cause of death for infants between 1 and 12 months of age
• SIDS rates have declined by more than 53% since 1990
• Deaths in child care* in US accounted for 20% in 1996 and 16.5% in 2001
(Moon, et.al., Pediatrics, 2000 and 2005)
• The exact cause of SIDS remains unknown
• Experts cannot predict which babies will die from SIDS

* child cared for by a non-parental caregiver.

Massachusetts SIDS Center

29
 SIDS RISK FACTORS
• Babies who sleep prone (lying
face downward)
• Babies who sleep on their sides
• Unaccustomed tummy sleepers
• Smoking during pregnancy
• Exposure to 2nd hand smoke
• American Indian and African
American babies
• Multiple Births
• Males slightly more than
females
• Young Maternal Age (under 20)
• Mothers with late or no prenatal
care
• Preterm (before 37 weeks) and
birth weight under 5.5 lbs.
• Bed sharing
• Mild upper respiratory
infections
• Soft sleep surfaces
• Cluttered sleep area
• Overheating: temperature range
should be 68°-72°
• Substance abuse during
pregnancy
Massachusetts 30
SIDS Center
 BED SHARING

What is Bed Sharing? What is Co-Sleeping?

Bed Sharing refers to a sleeping Co-Sleeping refers to a sleeping
environment in which the baby shares environment in which the baby shares the
the same sleeping surface with another same room with a parent/caregiver.
person.

Infants who share a bed with another person, adult or child, are at an increased risk for SIDS.

31
TRIPLE RISK MODEL

Critical
development period

SIDS
Vulnerable External stressors
infant (Sleep position and
sleep environment)

32
 SIDS AND CHILD
CARE
Infants in Child Care
• Two thirds of US infants younger than
1 year are in non-parental child care.
• Infants of employed mothers spend an average of 22 hours
per week in child care.
• Statistically, we would expect less than 9% of SIDS deaths
to occur in child care.
Ehrle et al, 2001

Massachusetts
33
SIDS Center
 SIDS IN CHILD CARE
Although we would expect less than 9% of SIDS
deaths to occur in child care settings;
• In the United States, 16.5% of SIDS deaths
occurred while the infant was in the care of a non-
parental caregiver.
– 36.7% in family child care
– 17.7% in child care centers
– 21.3% in relative care
– 17.7% with nanny/babysitter at home
» Moon & al. 2005

34
 Number of SIDS Deaths in Massachusetts
1990 - 2001
100

90

80
Total
70
number of
60 SIDS
50 Deaths
SIDS
40
Deaths in
30
child care
20

10

0
Chart: Massachusetts
90 91 92 93 94 95 96 97 98 99 '00 '01
SIDS Center
35
 REDUCING THE RISK OF
SIDS
• Place baby on back to sleep • Do not smoke around infants
• Use firm surface • Consider a pacifier at nap
• Keep soft objects and loose and bedtime during 1st year
bedding out of sleep area/crib (with parental approval)
• Avoid overheating
• Continue to educate others
about SIDS and safe sleep
• Supervise infants during sleep practices
• Place one infant at a time in
each crib / playpen / bassinet
for sleep.

Massachusetts SIDS Center

36
SIDS RISK REDUCTION

1. Back to Sleep
2. Avoid Overheating
3. Safe sleep environment and
supervision
4. “Tummy Time” when infant is awake
and supervised

37
SIDS RISK REDUCTION:
BACK TO SLEEP

38
EXAMINING COMMON BELIEFS

Why don’t people

put babies to
sleep on their
backs?

39
 Why not
Back to Sleep?

• The baby may

spit up /
choke
 Why not
Back to Sleep?
• The baby will get a bald spot
 Why not
Back to Sleep?

• The baby will get a flat head

Before After
 Why not
Back to Sleep?

• The baby won’t sleep as soundly

 SIDS RISK REDUCTION
AVOID OVERHEATING
– Check baby upon arrival at program.
If baby is in car seat, remove and
place in crib
– Never cover baby’s head with a
blanket
– Room temperature should be
between 68°-72°
– Do not overdress baby or leave in
winter clothes while inside or during
extended rides in a car seat
 SIDS RISK REDUCTION
A SAFE SLEEP ENVIRONMENT AND
SUPERVISION

• Safe crib
• No blankets
• No pillows
• No toys
• No wedges
• No smoke

45
Safe sleep environment while
transporting?

46
SIDS RISK REDUCTION
TUMMY TIME

47
SIDS RISK REDUCTION
Communicate with Parents

48
 HANDLING A MEDICAL
EMERGENCY
• Have a plan in place
• Review the plan with all staff periodically
• Practice emergency response
• Be trained in infant first aid and CPR

49
 FIRST AID:
UNRESPONSIVE INFANT
• Initiate one sequence of CPR
• Call 911
• Return to CPR
• Call emergency child care backup person
• Send/bring infant’s medical records to hospital
• Accompany infant to hospital, if possible
• Notify parents
• Notify supervisor if you have one or child care system
• Notify EEC

50
Imperforate Anus
Imperforate Anus
• Incidence: 1 in 5000 births
male preponderance

• noticed initially as failure to pass meconium

• Persistence of the cloacal membrane results

in an imperforate anus

• can be associated with congenital anomalies such as

Vertebral anomalies-hemi-vertebra, hypoplastic vertebra

Anal defects
Cardiac defects-atrial septal defect, ventricular septal defect, tetralogy of fallot
Tracheo-Esophageal, esophageal atresia
Renal defects
Limb defects-hypoplastic thumb, polydactyl, syndactyl, radial aplasia.
Imperforate Anus

High lesions
• rectum ends above the levator ani muscle
• long term fecal incontinence
• communication with urethra in males and
vagina in females
• surgery-colostomy preceding repair

Low lesions
• rectum ends below the levator ani muscle
•communication with skin in the perineum,
median raphe of scrotum, or into the
vaginal vestibule
• perineal anoplasty with closure of fistula, creation of anal opening, repositioning the
rectal pouch into the anal opening.
Imperforate Anus-preoperative assessment
•Assess for other anomalies with

 Ultrasound of renal system

 Chest X-ray

 ECG

 Echocardiogram

 X rays of lumbar and sacral spine

• Presentations

 abdominal distension
 impairment of ventilation, apnea
 Bowel ischemia
Imperforate Anus-Postoperative Care

1. Patients are usually extubated.

2. Maintenance fluids are required until feeding is established.

3. Analgesia should be adequate—paracetamol 15 mg/kg oral 6-hourly (maximum 60

mg/kg/day) and a weak opioid such as codeine phosphate 0.5 mg/kg oral 4-hourly.

4. After formation of a colostomy the options are an IV infusion of morphine sulphate

or an epidural infusion of LA usually for 24 hours.
HIRCHSPRUNG’S DISEASE
 Introduction
• A.K.A Congenital aganglionic megacolon
• Discovered by Harald Hirchsprung (1886)
• Is a condition that affects GIT and usually causes problems with passing stool.
• Present when a baby is born (congenital) and results from missing nerve cells
in the muscles of part or all of the baby’s colon.
• most common cause of lower intestinal obstruction in neonates.
• Familial tendency
• Risk factors;
- + family hx,
- being male,
- having other inherited conditions (heart problems, Down sydrome)
 Incidence
• 1\5000 live birth newborn

• 70-80% is boys.

• (M / F. 4: 1 )

• Total colonic aganglionosis, 35% girls

• 3-5% have Down’s sydrome

• Less common in blacks.

• >95% cases are full term babies

• Prematurity is reported in as many as 10% of those children with HD

• Familial tendency (Dominant pattern of inheritance)

 EMBRIYOLOGY
During normal fetal development cells from
neural crest migrate into the large intestine to
form the network of nerves called Auerbach’s
plexus (Muscularis externa) and Meissner’s
plexus ( submucosa)
-Occurs in the end of first trimester
-Lack of these nerves causes failure of relaxation
of the involved part of the colon.
-Also supplied by sympathetic nerves, and
intrinsic component (enteric nervous system)
 Types
1. Congenital :
Most common
2. Acquired :
Degeneration of the ganglions may occur due to:
-Vascular causes like after pull through procedure due to
ischemia & tension.
- Non vascular causes like
Vit B1 def.
Chronic infection ( TB.).
 ASSOCIATED ANOMALIES

HD is usually a solitary anomaly in a full term, otherwise healthy infant

Associated anomalies do occur in nearly 20% of cases
• urogenital system (11%)
• cardiovascular system (6%)
• gastrointestinal system (6%),
• with 8% having various other malformations

Associated syndromes:
Waardenburg-Shah sydrome
Trisomy 21 occurs in approximately 5% of cases
Mowat-Wilson sydrome,
Goldberg-Shpritez megacolon sydrome, and
Congenital central hypoventilation sydrome.
MEN2 (Multiple endocrine neoplasia)
 Waardeberg syndrome
An inherited auotosomal dominant disorder
-hearing loss
-Pigmented anomalies affecting the eyes, hair,
skin and various defects of neural crest
derived tissues
Signs and Symptoms
In newborns
-Failure to pass meconium
-Abdominal distension
-Vomiting (bilous)
-Constipation or gas
-Diarrhea

In older children
-Chronic constipation
-abdominal distension
-failure to gain weight (growth retardation)
 COMPLICATIONS
Neonatal Intestinal Obstruction
-bilous vomiting,
-abdominal distention
-failure to pass meconium

Recurrent Enterocolitis
mainly in the 1st three months of life.
-fever,
-lethargy,
-anorexia,
-vomiting,
-abdominal distention and
-diarrhea
Tx: antibiotics, antipyretics, fluids

Spontanous perforation occurs in 3%, specially if long segment aganglionosis.

Chronic constipation
Growth retardation
Volvulus.
 Diagnosis
History
Failure to pass meconium,
Painless abdominal distention
Constipation

Physical Examination
Distended abdomen
Multiple fecal masses on abdominal examination

On DRE:
• Anal sphincter is hypertonic
• Rectal ampulla is typically empty.
• Hard fecal mass.
• Gush of air upon withdrawal of finger
 Investigations

Radiology
1. Plain x-rays of the abdomen :Erect & supine
2. Contrast enema
– contrast enema should be done without preparation of
bowel
– Shows narrow distal segment,
– funnel-shaped dilatation at level of transition zone with
marked dilatation of the proximal colon.
24-hrs delayed films

(child with psychogenic stool holding)

Electromanometry .
– The classic finding is the absence of the recto anal inhibitory reflex
when the rectum is distended.
(Lack of internal anal sphincter relaxation in response to rectal stretch),
ballooning
– not useful in neonate
– excellent screening tool in infant & children
Rectal biopsy :
– Definitive diagnostic test
– demonstrates absence of ganglion cells,
– nerve hypertrophy and
– stains indicating increased acetylcholinesterase activity.

Suction mucosal biopsy (at different levels ), can be done without anesthesia
Full thickness biopsy is done under general anesthesia.

Ultrasonography: for associated anomalies.

 Management :
• Acute Stage
– NGT ,
– NPO
– IVF ,
– Antibiotics ,
– Rectal tube irrigations .
– The initial treatment requires performing a colostomy.
( multiple seromuscular biopsies)

Note: The colostomy is placed above the transition zone.

– Placement in an area of aganglionosis will lead to persistent obstruction
– Definitive treatment will be planned.

• Chronic constipation :
– Laxative
– Saline enema.
– Work up to establish the diagnosis
– Definitive treatment will be planned
 Definitive Procedures
By the age of 6-12 months; 9kg or more), a formal pull-through procedure is done

1. Open surgery :
There are many surgical options for Pull-through operation.
All aiming at resection of aganglionic segment
They give excellent result in 90%.
a. Swenson.
b. Soave.
c. Rehbein.
d. Duhamel.
e. Boley's.
Swenson Procedure
Sharp extrarectal dissection down to 2
cm above the anal canal

Aganglionic colonic segment resected

End-to-end anastamosis of normal

proximal colon to anal canal

Completely removes defective

aganglionic colon
Duhamel Procedure
Posterior portion of defective colon
segment resected

Side to side anastamosis to left over

portion of rectum

Constipation a major problem d/t

remaining aganglionic tissue

Simpler operation, less dissection

Soave Procedure
Circumferential cut through muscular
coat of colon at peritoneal reflection

Mucosa separated from the muscular

coat down to the anal canal

Proximal normal colon is pulled

through retained muscular sleeve

Telescoping anastamosis of normal

colon to anal canal

Advantage: rectal intramural dissection

ensures no damage to pelvic neural
structures

Higher rate enterocolitis, diarrhoea, often

requires repeated dilations
2. LAPAROSCOPY .
Trans-anal Endorectal Pull-through
-Excised aganglionic tissues removed through anal
canal
-no abdominal incision
-Better results in terms of pain, return of bowel
function, shortens hospital stay
-Similar incidence of leaks, pelvic abscesses,
enterocolitis.
 One vs Two Stage Procedure

Historically,
Two stage procedure performed: preliminary
colostomy, then completion pull through
-Delicate muscular sphincters of newborn may be
injured

1980s, 1 stage procedures became more popular

 COMPLICATIONS

1. anastomotic leak.
2. stricture.
3. retraction of the colon.
4. fecal incontinence
5. persistent constipation.

NOTE:
-Afebrile Diarrhea soon after pull-through is
expected
-Fluid and observation, Avoid anti-emetics
 Distinguishing features between childhood functional
constipation and Hirschsprung’s disease
Feature Functional Constipation Hirschsprung’s Disease
Onset years 2-3 At birth
Delayed passage of meconium Rare Common
Obstructive symptoms Rare Common
Withholding behavior Common Rare
Fear of defecation Common Rare
Fear of incontinence Common Rare
Stool size Very large Small, ribbon-like
Poor growth Rare Common
Enterocolitis Never Possible
Rectal ampulla Enlarged Narrowed
Stool in ampulla Common Rare
Barium enema Lg amount of stools, Transitional zone, delayed
no transitional zone emptying

Anorectal manometry Normal Absent rectosphincteric refl ex

Rectal biopsy Normal No ganglion cells, nerve hypertrophy
and increase acetylcholinesterase
activity
 Hydrocephalus in Children

www.SpringfieldClinic.com
What is Hydrocephalus
• “Water on the Brain”
• CSF constantly produced and absorbed
• Caused by lack of absorption
• Results in increased fluid pressure in
brain
• Can be present at birth or later in life
Function of CSF
• Maintenance of a constant external
environment for neurons and glia
• Mechanical cushion to protect the brain
and provide buoyancy to the heavy
brain (1400 g)
• Serves as a lymphatic system and a
conduit for neuropeptides
• pH of CSF regulates pulmonary
ventilation and CBF
Causes
• Congenital
• Acquired
– Prematurity
– Infection
– Tumor
– Bleeding in brain
– Trauma
Signs and Symptoms
• In Young Children
– Abnormal increase in head size
– Irritability
– Sleepiness
– Vomiting
– “Sunset” eyes
Signs and Symptoms
• In older Children
– Headache
– Poor school performance
– Loss of coordination and difficulty walking
– Sleepiness
– Vomiting
– Loss of bladder control
Treatment Goals

• To restore normal pressure in head

– 1. restoring normal CSF flow
– 2. divert CSF to another part of body
Causes of OBSTRUCTIVE
Hydrocephalus

• Congenital Malformations
– Aqueductal Stenosis
– Arachnoid Cysts
• Acute Post-hemorrhagic
• Mass lesion
Causes of COMMUNICATING
Hydrocephalus

•Defective absorption of CSF

– Chronic Post-hemorrhagic
– Chronic Post-Infectious
•Venous drainage insufficiency
•Overproduction of CSF (RARE)
Normal Head CT
Hydrocephalus
Aqueductal Stenosis

• Obstructive hydrocephalus
• Most common cause of congenital HCP
(43%)
• Asymptomatic at early age
• OFC increase
• May present later with headaches
 Ventriculoperitoneal (VP)
Shunt

Journal of NeurosurgeryPediatrics
Mayo Foundation for Medical Education and Research
Signs & Symptoms of
Shunt Malfunction
• Same as hydrocephalus
• But also signs of infection
– Fever
– Swelling
– Redness
– Drainage
Preventative Medicine

• Education
• Routine Clinic Follow-up
• Surveillance Imaging
– Ultrasound
– CT scan
– MRI
Hydrocephalus in
Premature Infants

• VP Shunting is poor option due to:

– Small size
– Abdomen is poor terminus
– Blood in ventricles causes shunt malfunction
ARACHNOID CYST
• 14 month-old
• Inappropriate head size increase
• Unable to walk
• Abnormal reflexes
• No irritability
• No vomiting
• No excessive somnolence
PRE-OP 6 MONTHS POST-OP
Prognosis
• 6 in 10 will die if untreated
– Survivors left with neurologic deficits
• Prompt treatment
• Prognosis dependent on cause of
Hydrocephalus:
– Infection/ Trauma / Tumors
– Aqueductal Stenosis/ Arachnoid cyst
Febrile seizures
A febrile seizure
Is defined as:
an event in infancy or childhood, usually
occurring between 3 months and 5 years of age,
associated with fever but without evidence of
intracranial infection or defined cause

Engel J, Jr. Report of the ILAE classification core group. Epilepsia. 2006;47:1558-1568

105
Pediatric febrile seizures

Represent the most common childhood

seizure disorder, exist only in association
with an elevated temperature
http://emedicine.medscape.com/article/1176205-overview

106
Febrile seizures groups
Epidemiologically; febrile seizures are
grouped into 3 groups, as follows:

1.Simple febrile seizures

2.Complex febrile seizures

3.Symptomatic febrile seizures

107
Simple febrile seizures

The setting is fever in a child aged 6 months to

5 years

108
Simple febrile seizures (cont.)

The single seizure is generalized and lasts

less than 15 minutes

109
Simple febrile seizures (cont.)

The child is neurologically healthy and

without neurologic abnormality by
examination or by developmental history

110
Simple febrile seizures (cont.)

Fever (and seizure) is not caused by

meningitis, encephalitis, or any other
illness affecting the brain

111
Simple febrile seizures (cont.)

The seizure is either:

• Generalized clonic
or
• Generalized tonic-clonic seizure

112
Complex febrile seizures

Age, neurologic status before the illness,

and fever are the same as for simple febrile
seizure

113
Complex febrile seizures (cont.)

This seizure is either:

• Focal or prolonged (ie, >15 min)
or
• Multiple seizures occur in close
succession

114
Symptomatic febrile seizures

Age and fever are the same as for simple

febrile seizure

115
Symptomatic febrile seizures (cont.)

The child has a preexisting neurologic

abnormality or acute illness

116
Physical examination

Neurologically and developmentally

healthy child

No signs of meningitis or encephalitis

117
Diagnosis

No specific laboratory studies are

indicated for a simple febrile seizure.

Other laboratory tests may be indicated by

the nature of the underlying febrile illness.

118
Diagnosis (Cont.)

Concerning lumbar puncture :

Strongly considered in children younger

than 12 months
( because the signs and symptoms of
bacterial meningitis may be minimal or
absent in this age group)

119
Diagnosis (Cont.)

Concerning lumbar puncture (Cont.) :

Should be considered in children aged 12-

18 months
( because clinical signs and symptoms of
bacterial meningitis may be subtle in this
age group)

120
Diagnosis (Cont.)

Concerning lumbar puncture (Cont.) :

In children older than 18 months, the

decision to perform lumbar puncture rests
on the clinical suspicion of meningitis

121
Diagnosis (Cont.)

Neither computed tomography (CT) nor

magnetic resonance imaging (MRI) is
indicated in patients with simple febrile
seizures

122
Diagnosis (Cont.)

EEG is not indicated in children with

simple febrile seizures

Vast majority of these children have a

normal EEG

123
Treatment

Neither long-term nor intermittent

anticonvulsant therapy is indicated for
children who have experienced 1 or more
simple febrile seizures.

124
Treatment (Cont.)

Any therapy administered after a first

simple seizure will not reduce the risk of :
– Subsequent afebrile seizure
– Recurrent afebrile seizures
(ie, epilepsy).

125
Treatment (Cont.)

Rectal diazepam or intravenous, 0.2-0.5

mg/kg for patients with febrile seizures,
particularly those with febrile seizures
lasting more than 5 minutes

126
Treatment (Cont.)

Midazolam, administered intranasally,

0.2 mg/kg is as safe and effective as
intravenous or rectal diazepam in the
treatment of acute pediatric seizure
emergencies.
Brooks M. Intranasal Midazolam Works for Seizure Emergencies in Kids. Medscape Medical News. Nov 5 2013 .

127
Prognosis

Prognosis for normal neurologic function

is excellent

128
Prognosis (Cont.)

About one third of children who

experience a single simple febrile seizure
will have another

129
Conclusion

Children aged 3 months to 6 years may

have tonic-clonic seizures when they have
a high fever.

http://www.epilepsy.com/learn/types-seizures/febrile-seizures

130
 Conclusion (cont.)

More likely to occur if there is a family

history of febrile seizures.

http://www.epilepsy.com/learn/types-seizures/febrile-seizures

131
Conclusions (cont.)

Most children do not require daily

treatment with medication.

http://www.epilepsy.com/learn/types-seizures/febrile-seizures

132
Conclusions (cont.)

Among children who have their first

febrile seizure before their first birthday,
half will have at least one more.

http://www.epilepsy.com/learn/types-seizures/febrile-seizures

133
Conclusions (cont.)

Long-term outlook is excellent.

http://www.epilepsy.com/learn/types-seizures/febrile-seizures

134
135