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“Dude” Isabedra
INTESTINAL ATRESIA
atresia- complete obstruction PRESENTATION:
stenosis- umikot lang syajejuno ileal Polyhydramnios
segment Billous Vomiting
localized late intrauterine vascular insult Abdominal Distension- differential with
Doc Kharl-Kilabot & Gang
6 PEDIATRIC SURGERY (SY 2016-2017) – 1ST SEM Dr. “Dude” Isabedra
Embrology
-3rd – 4th week duodenum develop from distal
portion of the proximal mid gut
-5-6 weeks usually duodenal canal obliterate due to
the proliferation the epithelial cell
-8-10 weeks vacuoles then form and coalesce to
create a lumen.
11 week recanalization
DIAGNOSIS:
prenatal ultrasound (duodenal atresia= Maldevelopment
history and PE - Newborn, vomiting green, Intestinal atresia= Insult)
no meconium with abdominal distention
Plain abdominal x-ray - abdominal gases ETIOLOGY
barium enema- mirco colon/ unused colon failure of recanalization
o 50% with associated anomalies
o 40% have Trisomy 21
35% prematurity
25% other GI anomalies
PATHOLOGY
majority are periampullary- obstruction is
also similar very similar to malrotation
therefore they have almost same s/sx
dilated stomach and duodenum
with vomiting in utero
(Malrotation= Single bubble;
history of polyhydramnios
Intestinal atresia= Multiple)
(pag di na diagnose do contrast barium enema=
Type A. Duodenal web
walang gas or small lng yung gas)
B. may cord
C. desociation talaga
TREATMENT:
without treatment intestinal atresia is
MANIFESTATIONS
incompatible with life
Polyhydramnios
prenatal counseling
Sepsis
preoperative resuscitation- correct
electrolyte imbalance Vomiting and upper abdominal fullness
o consistent findings
surgery
Bilious or nonbilious vomiting
resection or end-end anastomosis
abdomen usually distended by the dilated
postoperative care
stomach and proximal duodenum
INCIDENCE
DIAGNOSIS more common in Caucasians than Hispanics,
Prenatal Ultrasound Blacks, or Asian
History & PE 1-3:1000 live births
Plain Abdominal X-ray - confirmatory 4:1 male to female ratio
o “Double Bubble Sign” – Maternal History: 19% boys, 7% girls
pathognomomic, also seen in Paternal History – 5% boys, 2.5 girls
prenatal UTZ, distended 7% with associated anomalies
stomach and distended ETIOLOGY / PATHOLOGY
duodenum is seen in AP VIEW. Congenital? Acquired? – unsure of the
(yung nag hahati sa kanila cause, not proven yet.
“pylorus”) Genetic Evidence
o Race, male, first born, ABO type
(Multiple=Intestinal atresia Gastric hyperacidity muscle spasm &
Single= Malrotation hypertrophy leading to obstruction of the
Double= Duodenal atresia) gastric outlet
abnormal pyloric innervations
TREATMENT abnormal motility due to decrease
Amniocentesis, Karyotyping pacemaker cells
Counseling marked muscle hyperthrophy partial/complete
Preoperative Resuscitation gastric outlet obstruction
Surgery – “Diamond
anastomosis” (walang CLINICAL MANIFESTATIONS:
nereresect, kasi usually yung -Non-bilios vomiting at 2-8 weeks of life
atretic part asa d2>ampulla -always hungry
of vater>common bile duct kaya pag tinangal -HYPOCHLOREMIC HYPOKALEMIC ALKALOSIS
mo yun wipples nay un) -visible gastric peristaltic wave
Open the proximal and distal segment then do -palpable pyloric tumor
an anastomosis. DIAGNOSIS
POSTOPERATIVE CARE History & PE
Plan X-ray not specific
COMPLICATIONS Ultrasound- “Target Sign” (hypertrophy of
anastomotic leak pyloric muscle)
POA – possibility of adhesion UGIS- “String Sign”
functional obstruction TREATMENT
Tapering Duodenoplasty (kase dilated yung -Preoperative resuscitation
pouch, magkaiba yung lumen nung dalawa, -Surgery FREDET-RAMSTEDT PROCEDURE
or else obstruction ) -Post operative care
-?Non-operative management
PYLORIC STENOSIS
ayusin pag malaki na ang baby. Pangit tingnan pero BILIARY ATRESIA
at least di na kelangan ng surgery. eto yung madalas pinapalabas sa kapwa mo mahal
Omphalocele has many different anomalies, ko.
usually cardiac anomalies kaya hirap operahan. I. GENERAL CONSIDERATIONS:
not a static disease but chronic, progressive liver
3.)Lower midline defects problem.
Bladder exstrophy – urinary bladder lang ang 40% passed meconium at birth – hindi
nakaherniate nadidiagnose at birth agad2x.
Cloacal exstrophy –small bowel ang naka- 1:10,000-12,000 live births
herniate AT may omphalocele. no racial differences or genetic factors
M:F = 1:0.64 (MALE is predisposed!)
** it is a midline defect, na lahat ng nasa lower 3 MAIN TYPES:
midline ay hindi nagfuse sa gitna so pati ang genital 1.) TYPE 1 – atretic part is common bile duct
organ ng bata ay hindi nag fuse. Ang problem sa 2.) TYPE 2 – common hepatic hanggang
bladder/cloacal exstrophy is hindi mo alam kung common bile duct.
male or female ang baby. So kelangan mo magpa- 3.) TYPE 3 – hanggang common hepatic duct.
karyotype. You need karyotyping para ma-identify 4.) TYPE 4 – buong biliary tree kasama ang
ang sex ng baby. In States, pag nirepair yan, gallbladder. So ito ang pinaka-common
ginagawang female muna. Kasi mas mahirap pag type!
gawin muna male.
II. CAUSE: exact cause is not known
How to repair Cloacal Exstrophy? not link to medications taken, or illnesses of the
Usually iclose sa gitna, then saka nalang lagyan ng mother
ari ang bata paglaki niya. Kasi mahirap yan irepair. currently no genetic link known
unlikely to occur more than once in the family
I. Diagnosis:
Prenatal Ultrasound III. PATHOLOGY:
ductular proliferation and portal fibrosis
II. Management: ** pag- nakita niyo ito sa exam question – BILIARY
Counseling ATRESIA na! Ganun lang.
Transfer to tertiary center
Thermoregulation IV. CLINICAL MANIFESTATIONS:
Local Care of Defect Jaundice, clay-colored stools (alcoholic stool),
Hydration and hepatomegaly
Antibiotic coverage splenomegaly
active initially sequelae of cirrhosis – if with
III. Non-operative management: progressive disease magkakaroon ng liver cirrhosis.
Termination of pregnancy average survival (untreated) = 19 mos.
Topical treatment appear healthy at birth
Expectant management usually asymptomatic at 2 WoL (week of life)
jaundice, clay-colored stools, dark urine and
Operative hepatomegaly
Primary closure splenomegaly – S/Sx generally non specific
Skin Closure average survival (untreated) = 19 mos.
Silo
V. DIAGNOSIS:
History and PE
Day 4
I. Hirschsprung’s disease
II. Anorectal Malformation
HISRCHSPRUNG’S DISEASE
GENETIC ETIOLOGY
Deletion of 10q11.2 and q21.2
Chromosome 13q22 recessive susceptibility
locus
Error in endothelin B receptor gene (EDNRB)
Neuroenteric cells migrate distally along the exon 4
course of the vagus nerve.
5th week AOG – esophagus
7th week AOG – midgut
12th week AOG – complete migration
Transmural migration of neuroenteric cells
PATHOPHYSIOLOGY
(+) Enterocolitis
Complete obstruction at birth
Delayed passage of meconium followed by
repeated obstruction
Mild constipation followed by acute
obstruction
Mild symptoms followed by enterocolitis
Mild chronic constipation
INCIDENCE
1:2000 live births, 70-80% males DIAGNOSTIC
Familial predisposition – rare History & PE
25% with associated anomalies in familial
cases
Most are isolated in non-familial cases
Observed in all races but is less common in
blacks
Most common – short segment (44%) – Recto
sigmoid
Least common – Cecum (start of colon) – 1%
Pwede bang magkaron ng HD sa cecum pero
wala sa recto sigmoid? – NO
o Transmural migration – neuroenteric cells
Radiography
nag arrest migration sa acending, hindi
o Plain abdominal x-ray
yan lalagtaw, hindi pupunta sa descending
Toxic megacolon: full of gas in the
o sa recto sigmoid, hangang dun lang un –
bowel
hindi pwedeng skip lesion.
CLINICAL PRESENTATION
Most present with intestinal obstruction and
constipation.
Failure to pass meconium within the 1st 24
hours of life – 95% of patients with HD
Explosive discharge of fluid stools and air on
DRE
MANAGEMENT
Non-surgical
o NGT
o Colonic washout
Surgical
o Goal: to resect the ganglionic part with the
megacolon
o 3 stage procedure
o 2 stage procedure
o 1 stage (Primary) procedure
Open
Laparoscopic
Incision less
Transanal (Incisionless)
• History and PE
• Barium enema
• Rectal biopsy
• Admitted day before the surgery
• Clear liquid diet (ORS)
• Laxative/colonic washouts
• IV antibiotics 1 hour prior to surgery
• Final colonic washout in the OR
Doc Kharl-Kilabot & Gang
20 PEDIATRIC SURGERY (SY 2016-2017) – 1ST SEM Dr. “Dude” Isabedra
DIAGNOSIS
History
PE
o Abdominal distention
o Flat abdomen
o No sphincter
CLASSIFICATION
ANORECTAL MALFORMATION History and PE
o Radiography
o Babygram – AP view
o Invertogram (Rice-Wangesteen) - before
o Prone cross-table lateral view
Anal marker: (-) rectal pouch/gas (high
tide)
o Perineal/Hypogastric ultrasound - before
o Contrast studies
Recto-upper vagina
Distal contonogram?
INCIDENCE o Anatomic Classification
Not known High
1:4000/5000 Male: rectovesical, rectoprostatic
2/3 of males have high types Female: rectouppervaginal
2/3 of females have low types Intermediate
VACTERL Low
Anocutaneous
Anovestibular
Anocutaneousfistula
MANAGEMENT
Low lesions
o Perineal anoplasty
High lesions
o Colostomy at birth
o PSARP at 4-6 months
o Closure of colostomy after 3 months
o Radiogrphic Classification:
Distance of Rectal pouch from anal
marker
High type = >1cm
Low type = <1cm
CT Scan
MRI
TREATMENT
Surgery TERATOMA
o Complete excision Derived from totipotential cell with at least
o Provide accurate staging 2 germ layers
information Cells in the proximity of Hensen’s nodes
Biopsy Usally midline or para-axial in location
Chemotherapy 80% benign but can be malignant
Gonadal – older children
HEPATOBLASTOMA Extragonadal – younger children
Fetal origin tissues Sacro-coccygeal – Newborn
All pediatric tumors = blastoma common in females – 80%
Most common childhood HEPATIC incomplete twinning
malignancy most secrte AFP, bHCG
M:F = 1.7:1 deletion of chromosome 1 & 6
18 mos – 3 y/o – most common with calcification
Secretes AFP <10% - risk of malignancy at birth
LoH 11p5.5 & 1p36 >75% after 1 y/o
Trisomy 20 or 2
CLINICAL PRESENTATION
Asymptomatic mass
Failure to thrive
Rarely produce sexual precocity
Anemia – elaboration of cytokines
Some present acutely with tumor rupture
DIAGNOSIS
History / PE
Liver function tests
AFP
Radiography
CT Scan / MRI
Biopsy
TREATMENT
Surgery
o Excision
DIAGNOSIS
o Biopsy
History / PE
Chemotherapy
Radiography
Radiotherapy
CT Scan
Liver transplantation
MRI
TREATMENT
Surgery
Doc Kharl-Kilabot & Gang
25 PEDIATRIC SURGERY (SY 2016-2017) – 1ST SEM Dr. “Dude” Isabedra