Ghasaq nadhom amp Widad najeeb Supervised by DrAli Farooq Case 4 2 years old male presented with gross abdominal distension associated with vomitingChronic constipation he had a history of delayed ID: 919716
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Slide1
Pediatric surgery IIBY: Ghasaq nadhom & Widad najeeb
Supervised by: Dr.Ali Farooq
Slide2Case 4:2 years old male presented with gross abdominal distension associated with vomiting,Chronic constipation, he had a history of delayed passage of meconium more than 48hr at birthThe radiology study shows this picture
what is the diagnosis & what’s this imaging ?Hirschsprung's disease barium enemaNarrow segment distally, transitional zone , dilated segment proximally
Slide3Hirschsprung's disease=congenital megacolon=colonic aganglionosisIs a functional intestinal obstruction due to congenital absence of ganglion cells in the recto-sigmoid regionthe aganglionosis may be in a short segment , long segment or even total colon
Slide4What is etiology & genetics of HP disease?The ganglion cells are derived from neural crest of embryo, which undergo migration through GIT from proximal to distal after differentiation into mature ganglion cells. In infant with HD this process is disturbed & the ganglion cells are absent in distal bowel. The genesis of Hirschsprung's disease is multifactorial (genetic + environmental). The most common identified gene is RET proto-oncogen
Slide5Diagnosis by :- Clinical presentation1-Neonatal intestinal obstruction 2-Chronic constipation: some patients present in later childhood with chronic constipation especially those with short segment HD, they usually had history of delayed passage of meconium more than 48hr., failure to thrive, malnutrition, hypoproteinemia
, anemia, gross abdominal distension.3-Enterocolitis : 10% of children with HD present with fever , abdominal distension , offensive diarrhea due to Hirschsprung's disease associated enterocolitis (HAEC).stasis of fecal materials in the colon lead to bacterial overgrowth & invasion of Clostridium
difficilli
& Rota virus to the bowel wall causing secondary infection (
enterocolitis
)which may cause life threatening toxic
megacolon
.
Slide6investigation :-1)Barium enema : Recto-sigmoid ratio less than 1 (transvers diameter of rectum / transverse diameter of sigmoid). Retention of contrast material in the colon more than 24hr post filming. Neonate with HD may have false –ve result.
2) Anorectal manometry:
recto-anal inhibitory reflex is defined as reflex relaxation of internal anal
sphincter in response to rectal distension.
This is normally present in normal children & absent in HD. But it is not reliable
in neonate because of immaturity of the reflex & artifact created by movement & crying of the baby.
3) Rectal biopsy:
give the definitive diagnosis of HD by histological confirmation of (absence of ganglions, hypertrophy of nerve fibers, increase staining of acetyl choline esterase) in both
submucosal
plexus (
meisner
) &
meyenteric
plexus
aurbach
). It can be done either by suction rectal biopsy (mucosa & submucosa) or by full thickness rectal biopsy.
Slide7management :-Pre- operative Resuscitation for neonate with intestinal obstruction or children with enterocolitis by I.V.F , electrolytes , broad spectrum antibiotics , nasogastric tube decompression , digital rectal stimulation or colonic irrigation with normal saline enemaEmergency stoma is indicated if the above measures failed.
Slide8Surgical treatment for Hirschsprung's disease:-A-three stages (Usually):- 1. Stoma formation: as an emergency procedure or electively performed to protect future colonic anastomosis.
Slide92. Pull-through procedure: resection of aganglionic segment & bringing the normally innervated bowel down to be anastomosed with the low rectum or anus. Pull-through procedures may be :-Swenson pull-through. Soave ( endorectal pull-through ). Duhamel (retrorectal pull-through).Rehbein pull-through.
Trans-anal pull-through.Laparoscopic pull-through. 3. Closure of stoma. B- Two stages (standard): colostomy & pull through. C- One stage (for selected cases)
Slide10Case 5:A mother who suffered from polyhydraminous has given birth to a baby who has Down’s syndrome,The baby started to develop bilious vomiting after birth.on examination of the
perineum,there was anal orific. A Plain abdomial x-ray was taken and shows this sign below. the condition was suspected on prenatal Ultrasound.
Slide11What is the sign called?Double bubble sign What are the DDX?Intestinal atresiaMalrotation of midgut &midgut volvulusAnnular pancreasLadds band
What is the DX?Intestinal atrasia
Slide12Duodenal atresia &stenosis Embryology :Failure of recanalization at 8th -10th weeks of gestation85% distal to the ampulla of vatter
50% association with anomalies & 30% is down syndrome (TEF. Malrotation of midgut,renal anomalies and
congenital heart disease
)
Slide13Types:
What is this called? anomaly windsock
Slide14DiagnosisPrenatally: U\SClinial presentations:Bile staind vomitingDehydration ,electrolyte disturbance
Delayed or no passage of meconiumNo or mild distentionPremature baby will lead to growth retardation
Post
natally
: erect
abdominal
X-ray :
Double
bubble
sign
Upper GIT contrast?
Slide15Management :1- correct fluid & electrolyte disturbance2- decompression by NG tube3-antibiotec coverage4-premature baby may need assisted ventilation 5- phototherapy and IVF if jaundice6- search for associated anomalies!!
Surgery1-doudeno-doudenostomy2-doudenotomy
Prognosis?
Slide16Jejuno-ileal atresiaVascular accident in LATE GESTATION.Low incidance of anomalies (less 10%)High incidance of cystic fibrosis
Slide17Types :
Slide18Presentation:obvious distention Inc. risk of perforation in proximal bowel & Subsequent peritonitisTenderness, edema and erythema of abdominal wall
Altered blood per rectum in type 3b
Slide19Diagnosis:Describe what you see?
Air-fluid level ground-glass appearance
Slide20Treatment :Treatment of perforationLadds procedureSingle resectionType IIIb & IV end with short bowel syndrome
Slide21Case 6:A 3-day-old male infant presented to the ER after respiratory distress had been developed. On history, the parent reported increased work of breathing starting the previous day. On examination ,his respiratory rate 70 cycle PM ,heart rate 161 BPM
,blood pressure 92/47mmHg, and had a pulse oximetry reading of 91% on room air.
cyanotic and
decreased breath sounds on the left side of the chest
.
His past medical history was significant for caesarian delivery at 39 + 3 weeks for failure to progress
.
His APGARs were 7 (1 minute) and 8 (5 minutes)
Slide22What is the most likely diagnosis?CDHWhat do you suspect to find on clinical examination of the baby?On examination:Scaphoid abdomen
increase antero-posterior diameter of the chestIn ipsilateral side Decrease breathing sound
Bowel sound heard in the chest
Shifting cardiac impulse to the right
Slide23Congenital Diaphragmatic HerniaEmbryology:Failure of fusion of septum transversum and pleuroperitoneal membranes between 4th and 8th
week of gestation.
Slide24Anatomical types:1- Bochdalek hernia : 85-90%Lt sided in 80-90%2-Morgagni hernia : 2-6%Incidentally 3- Hiatus hernia
Slide25PathophysiologyPulmonary hypoplasiaPulmonary hypertention((Persistent fetal circulation
Slide26Presentation:Low APGAR scoreCyanosis TachypneaGrunting Later Recurrent chest infection
Slide27Diagnosis:Prenatal U\S: at 24-26wkPolyhydraminousEchogenic chest massBowel loop & liver in the chest Mediastinal shifting
Slide28Post natally: chest X-ray :shows air & fluid level in the chestMediastinal shiftDecrease intestinal gas in abdomen Tip of NG tube in the chestBarium study:
Slide29Treatment:Resuscitation:NG tube for decompressionEndotracheal intubation & O2… bag mask!!IV line and fluid therapyEcho study
Respiratory stabilization before surgery include:1-inhaled nitric oxide2-surfactant therapy3-mechanical ventilation: HFOV4-ECMO
Slide30Slide31Surgery:
Slide32:Fetal intervention1- prenatal steroid therapy2- trachial occlusion3-intrauterine surgical repair.
Slide33Case 7:2 days old male baby presented with delay in passage of meconium , abdominal distention and vomiting, mother noticed of passing dark urine.On examination the patient was conscious , irritable, frequent crying & on inspection of the perineum the patient has absent anal orifice . Imaging study was done for him. As shown below .
Slide34What is the investigation called?invertogramDescribe what you seeHow many types are there ? Which one Why? The case is?
High lying &low lying typeThe case is of high type
Slide35Anorectal malformation & imperforate anus.1-absence of anal orifice2-agenesis of anal canal3-fistulous connectionHigh type: Mostly in male Difficult to manage (decompression & colostomy)High association with other anomalies
Poor functional prognosis Low type:More in femaleNo colostomy needed)
)
Easily treated
Good functional prognosis
Slide36important step in examination is missed , what is it ?VACTERL associationV-vertebral anomaliesA-anal atresiaC-cardiovascular anomalies
T-tracheoesophageal fistulaE-esophageal atresia
R
-renal\radial anomalies
L
-limb defects
Slide37Classification:
Slide38Management: No decision should be made before 24 hr. why?1-IV Fluid2- antibiotic3-search for other anomalies. Colostomy for high type .. Best
site?whycolostomy Divided descending 1-provide complete diversion of stool
2- permit doing distal
colostogram
to give the detailed anatomy of the defect before surgery
3-better protection of the
anorectoplasty
repaire
Slide39Surgery: 1- simple anal cut-back2-V-Y anoplasty3- anal transposition
Slide404-limitted posterior Ano-Rectoplasty PARP5-Posterior Sagittal Ano-Rectoplasty PSARP6- Anterior Sagittal Ano-Rectoplasty ASARPCLOACA need reconstruction surgery
Slide41Thank you