Division of Perinatology Department of Child Health...
Transcript of Division of Perinatology Department of Child Health...
![Page 1: Division of Perinatology Department of Child Health ...ocw.usu.ac.id/course/download/1110000107-growth-and-development-system/... · other intestinal atresia. IftImperforate anus.](https://reader033.fdocuments.in/reader033/viewer/2022041502/5e228c12d2d3e271c931ed08/html5/thumbnails/1.jpg)
Division of PerinatologyDivision of PerinatologyDepartment of Child Health Medical School
University of Sumatera Utara
![Page 2: Division of Perinatology Department of Child Health ...ocw.usu.ac.id/course/download/1110000107-growth-and-development-system/... · other intestinal atresia. IftImperforate anus.](https://reader033.fdocuments.in/reader033/viewer/2022041502/5e228c12d2d3e271c931ed08/html5/thumbnails/2.jpg)
CONGENITAL ANOMALIESCONGENITAL ANOMALIES
OtolaryngologyNeurological disordersC di l di dCardiovascular disordersRespiratory disordersGastrointestinal disordersGastrointestinal disordersGenitourinary disorders
![Page 3: Division of Perinatology Department of Child Health ...ocw.usu.ac.id/course/download/1110000107-growth-and-development-system/... · other intestinal atresia. IftImperforate anus.](https://reader033.fdocuments.in/reader033/viewer/2022041502/5e228c12d2d3e271c931ed08/html5/thumbnails/3.jpg)
OtolaryngologyOtolaryngology
Choanal atresia
Bilateral atresia:Bilateral atresia:Cyanosis, alleviated
by cryingR i t di tRespiratory distressDifficulty with feeding
Unilateral atresia:Presented usually
later in life
![Page 4: Division of Perinatology Department of Child Health ...ocw.usu.ac.id/course/download/1110000107-growth-and-development-system/... · other intestinal atresia. IftImperforate anus.](https://reader033.fdocuments.in/reader033/viewer/2022041502/5e228c12d2d3e271c931ed08/html5/thumbnails/4.jpg)
Diagnosis Ch l t iChoanal atesia
Fully examination head & neckP 6F ft th t i t t ilPass 6F soft catheter into nostrilMinimize congestion by avoiding multiple attempts p pDirect examination into the nostrils (flexible fiberoptic bronchoscope)
![Page 5: Division of Perinatology Department of Child Health ...ocw.usu.ac.id/course/download/1110000107-growth-and-development-system/... · other intestinal atresia. IftImperforate anus.](https://reader033.fdocuments.in/reader033/viewer/2022041502/5e228c12d2d3e271c931ed08/html5/thumbnails/5.jpg)
Cleft lip ± palateCleft lip ± palate
![Page 6: Division of Perinatology Department of Child Health ...ocw.usu.ac.id/course/download/1110000107-growth-and-development-system/... · other intestinal atresia. IftImperforate anus.](https://reader033.fdocuments.in/reader033/viewer/2022041502/5e228c12d2d3e271c931ed08/html5/thumbnails/6.jpg)
Cleft lip ± palate (CL/P)
• Etiology: failure of normal orofacial development during the 6th-12th wks of embryonic life
• Management:Management:Pre-operative: feeding problem
•Encourage breast-feeding•Other devices:Haberman•Other devices:Haberman•Check weight gain frequently (weekly)
•Operative:•3-5 mo: closure lip, nasal, alveolar clefts•8-10 mo: palatoplasty
Post-operative: prevention of infectionost ope at e p e e t o o ect o
![Page 7: Division of Perinatology Department of Child Health ...ocw.usu.ac.id/course/download/1110000107-growth-and-development-system/... · other intestinal atresia. IftImperforate anus.](https://reader033.fdocuments.in/reader033/viewer/2022041502/5e228c12d2d3e271c931ed08/html5/thumbnails/7.jpg)
Pierre-Robin sequence
Sequence: stunted growth of mandible→sequence growth of mandible→displacement of tongue→ clefting of palate (roof of mouth)
Airway patency problem, if severe → tracheostomyyFeeding poblem, if severe, may need
t t l tgastrostomy placementFirst stage repair:tongue-lip adhesion (glossopexy)lip adhesion (glossopexy)
![Page 8: Division of Perinatology Department of Child Health ...ocw.usu.ac.id/course/download/1110000107-growth-and-development-system/... · other intestinal atresia. IftImperforate anus.](https://reader033.fdocuments.in/reader033/viewer/2022041502/5e228c12d2d3e271c931ed08/html5/thumbnails/8.jpg)
Pierre-Robin sequence
Tracheostomy
Distraction Osteogenesis After reconstruction
![Page 9: Division of Perinatology Department of Child Health ...ocw.usu.ac.id/course/download/1110000107-growth-and-development-system/... · other intestinal atresia. IftImperforate anus.](https://reader033.fdocuments.in/reader033/viewer/2022041502/5e228c12d2d3e271c931ed08/html5/thumbnails/9.jpg)
N l i l di dNeurological disorders
HydrocephalusMeningomyelocele
![Page 10: Division of Perinatology Department of Child Health ...ocw.usu.ac.id/course/download/1110000107-growth-and-development-system/... · other intestinal atresia. IftImperforate anus.](https://reader033.fdocuments.in/reader033/viewer/2022041502/5e228c12d2d3e271c931ed08/html5/thumbnails/10.jpg)
Congenital hydrocephalus: etiologiesAccumulation of CSFAccumulation of CSF
Oversecretion of CSF:Papilloma of the choroid plexusPapilloma of the choroid plexusIncreased venous pressure
Obstruction of CSF pathwaysIntraventricular block at the level of the foramen of Munro, 3rd ventricle, Sylvian aqueduct or 4th ventricle aqueduct, or 4th ventricle Extraventricular block from inflammation or tumours
Deficient resorption :Venous hypertension Abnormalities of the arachnoid villi Unknown mechanisms
![Page 11: Division of Perinatology Department of Child Health ...ocw.usu.ac.id/course/download/1110000107-growth-and-development-system/... · other intestinal atresia. IftImperforate anus.](https://reader033.fdocuments.in/reader033/viewer/2022041502/5e228c12d2d3e271c931ed08/html5/thumbnails/11.jpg)
Clinical presentation and Diagnosis
Macrocephaly rapid Macrocephaly, rapid ↑ head circumference, large anterior and anterior and posterior fontanelle, splitting suturesCNS l th CNS:lethargy, hypotonia, impaired gaze, poor feedingPE:↑↑HC, tense fontanelle, splitting sutures
![Page 12: Division of Perinatology Department of Child Health ...ocw.usu.ac.id/course/download/1110000107-growth-and-development-system/... · other intestinal atresia. IftImperforate anus.](https://reader033.fdocuments.in/reader033/viewer/2022041502/5e228c12d2d3e271c931ed08/html5/thumbnails/12.jpg)
Treatment
P i t t t Progressive: treatment (VP shunt)
Non-progressive (brainatrophy): notreatment
Aqueductal stenosisMyelomeningocele with associated
treatmentBrain malformation: holoprosencephaly, lissencephalywith associated
HydrocephalusCongenital omm ni ting
lissencephalyHydrancephalyAtrophy post infection
communicating hydrocephalus
![Page 13: Division of Perinatology Department of Child Health ...ocw.usu.ac.id/course/download/1110000107-growth-and-development-system/... · other intestinal atresia. IftImperforate anus.](https://reader033.fdocuments.in/reader033/viewer/2022041502/5e228c12d2d3e271c931ed08/html5/thumbnails/13.jpg)
Meningocele: a protrusion of Meningocele: a protrusion of meninges only through a bony defect in the vertebral columnMyelomeningocele: a protrusion of meninges and protrusion of meninges and spinal cord through a bony defect in the vertebral
lcolumnSpina bifida occulta: vertebral defect in the vertebral defect in the absence of spinal cord or meningeal herniation
![Page 14: Division of Perinatology Department of Child Health ...ocw.usu.ac.id/course/download/1110000107-growth-and-development-system/... · other intestinal atresia. IftImperforate anus.](https://reader033.fdocuments.in/reader033/viewer/2022041502/5e228c12d2d3e271c931ed08/html5/thumbnails/14.jpg)
Respiratory disorders
Esophageal atresia and tracheoesophageal fistulaCongenital diaphragmatic hernia
![Page 15: Division of Perinatology Department of Child Health ...ocw.usu.ac.id/course/download/1110000107-growth-and-development-system/... · other intestinal atresia. IftImperforate anus.](https://reader033.fdocuments.in/reader033/viewer/2022041502/5e228c12d2d3e271c931ed08/html5/thumbnails/15.jpg)
Tracheoesophageal fistulaFail of esophagotracheal septum to separate the esophagus andFail of esophagotracheal septum to separate the esophagus and the trachea
![Page 16: Division of Perinatology Department of Child Health ...ocw.usu.ac.id/course/download/1110000107-growth-and-development-system/... · other intestinal atresia. IftImperforate anus.](https://reader033.fdocuments.in/reader033/viewer/2022041502/5e228c12d2d3e271c931ed08/html5/thumbnails/16.jpg)
Types of TEF
Di i (1) NG i ti (2) XDiagnosis: (1) NG insertion; (2) X-ray; (3) fluoroscopy; (4) bronchoscopy
![Page 17: Division of Perinatology Department of Child Health ...ocw.usu.ac.id/course/download/1110000107-growth-and-development-system/... · other intestinal atresia. IftImperforate anus.](https://reader033.fdocuments.in/reader033/viewer/2022041502/5e228c12d2d3e271c931ed08/html5/thumbnails/17.jpg)
Stabilization then transportTracheoesophageal Fistula & Esophageal ac eoesop agea stu a & sop ageaAtresia
Head: keep at 450 angleInsert NGT & suctionAvoid bag mask intubate if neededAvoid bag-mask, intubate if neededPrevent potential aspiration through fistula: minimize abdominal manipulation
![Page 18: Division of Perinatology Department of Child Health ...ocw.usu.ac.id/course/download/1110000107-growth-and-development-system/... · other intestinal atresia. IftImperforate anus.](https://reader033.fdocuments.in/reader033/viewer/2022041502/5e228c12d2d3e271c931ed08/html5/thumbnails/18.jpg)
Congenital Diaphragmatic HerniaA diaphragmatic defect occurs at 8-10 wks of gestationA diaphragmatic defect occurs at 8 10 wks of gestation, owing to failure of closure of the pleuroperitoneal canal, thus the presence of the abdominal viscera in the thoracic
itcavity
![Page 19: Division of Perinatology Department of Child Health ...ocw.usu.ac.id/course/download/1110000107-growth-and-development-system/... · other intestinal atresia. IftImperforate anus.](https://reader033.fdocuments.in/reader033/viewer/2022041502/5e228c12d2d3e271c931ed08/html5/thumbnails/19.jpg)
• Left : common – 70%Left : common 70% (through foramen of Bochdalek)I iti l t t t• Initial treatment: stabilization of pulmonary function, prevention of PPHN
• Surgical repair: after CR stablestable
• Mortality ~40%
![Page 20: Division of Perinatology Department of Child Health ...ocw.usu.ac.id/course/download/1110000107-growth-and-development-system/... · other intestinal atresia. IftImperforate anus.](https://reader033.fdocuments.in/reader033/viewer/2022041502/5e228c12d2d3e271c931ed08/html5/thumbnails/20.jpg)
Stabilization then transport ofcongenital diaph agmatic he niacongenital diaphragmatic hernia
Avoid bag-mask & intubate promptlyInsert NGT & suctionInsert umbilical arterial and venous linesNothing by Mouth (NPO)T ansfe to the t eatment centeTransfer to the treatment center
![Page 21: Division of Perinatology Department of Child Health ...ocw.usu.ac.id/course/download/1110000107-growth-and-development-system/... · other intestinal atresia. IftImperforate anus.](https://reader033.fdocuments.in/reader033/viewer/2022041502/5e228c12d2d3e271c931ed08/html5/thumbnails/21.jpg)
Gastrointestinal disorders
GastroschisisOmphaloceleOmphaloceleImperforate anus
![Page 22: Division of Perinatology Department of Child Health ...ocw.usu.ac.id/course/download/1110000107-growth-and-development-system/... · other intestinal atresia. IftImperforate anus.](https://reader033.fdocuments.in/reader033/viewer/2022041502/5e228c12d2d3e271c931ed08/html5/thumbnails/22.jpg)
How do you differentiate omphalocele and gastroschisis?
t hi i omphalocelegastroschisis
![Page 23: Division of Perinatology Department of Child Health ...ocw.usu.ac.id/course/download/1110000107-growth-and-development-system/... · other intestinal atresia. IftImperforate anus.](https://reader033.fdocuments.in/reader033/viewer/2022041502/5e228c12d2d3e271c931ed08/html5/thumbnails/23.jpg)
PathogenesisGastroschisis:
Umbilical veins 2 → 1Left moves centrallyLeft moves centrallyRight: regresses and may creating a weak spot (~6wk of gestation) and created the defect when intestines returns into the defect when intestines returns into the abdominal cavity at ~10th week of gestation)
Teratogen exposureGenetic
Omphalocele:Intestinal loops fail to return to the abdominal Intestinal loops fail to return to the abdominal cavity at ~ 11 wkDue to abnormal embryonic development, thus hi h f i d d f & h l high rate of associated defects & chromosomal abnormalities
![Page 24: Division of Perinatology Department of Child Health ...ocw.usu.ac.id/course/download/1110000107-growth-and-development-system/... · other intestinal atresia. IftImperforate anus.](https://reader033.fdocuments.in/reader033/viewer/2022041502/5e228c12d2d3e271c931ed08/html5/thumbnails/24.jpg)
Stabilization then transportStab at o t e t a spo tGastroschisis, Omphalocele
Prevent hypothermia: cover with clear plastic, avoid gauze that usually
Omphalocele: examine for Cardiac, Renal, Imperforate anus, avoid gauze that usually
stick to the bowel)Insert NGT & suctionP b l i h i
p ,Chromosome:Trisomy 13,18,21,Beckwith-Wiedemann Syndrome
Prevent bowel ischemia: place the baby on sidePrevent infection: start IV
y(large tongue, gigantism, hypoglycemia), CHARGE association.
antibiotic : Ampicillin + Gentamycin
association.Gastroschisis: look for other intestinal atresia
![Page 25: Division of Perinatology Department of Child Health ...ocw.usu.ac.id/course/download/1110000107-growth-and-development-system/... · other intestinal atresia. IftImperforate anus.](https://reader033.fdocuments.in/reader033/viewer/2022041502/5e228c12d2d3e271c931ed08/html5/thumbnails/25.jpg)
I f tImperforate anus
![Page 26: Division of Perinatology Department of Child Health ...ocw.usu.ac.id/course/download/1110000107-growth-and-development-system/... · other intestinal atresia. IftImperforate anus.](https://reader033.fdocuments.in/reader033/viewer/2022041502/5e228c12d2d3e271c931ed08/html5/thumbnails/26.jpg)
Diagnosis & Treatment
Dilatation for perineal or vestibular fistulaAll other fistulae required distal colostogram to q gdetermine the anatomyColostomy for all other fistula
fDefinitive reconstruction
![Page 27: Division of Perinatology Department of Child Health ...ocw.usu.ac.id/course/download/1110000107-growth-and-development-system/... · other intestinal atresia. IftImperforate anus.](https://reader033.fdocuments.in/reader033/viewer/2022041502/5e228c12d2d3e271c931ed08/html5/thumbnails/27.jpg)
Genitourinary disorders
Bladder exstrophyBladder exstrophy
• Failure of midline fusion including deep structures eg. Symphisis pubis and bladder wallwall
• Surgery:•OrthopedicsU l i•Urologist
• Nephrologist and psychologistp y g
![Page 28: Division of Perinatology Department of Child Health ...ocw.usu.ac.id/course/download/1110000107-growth-and-development-system/... · other intestinal atresia. IftImperforate anus.](https://reader033.fdocuments.in/reader033/viewer/2022041502/5e228c12d2d3e271c931ed08/html5/thumbnails/28.jpg)
![Page 29: Division of Perinatology Department of Child Health ...ocw.usu.ac.id/course/download/1110000107-growth-and-development-system/... · other intestinal atresia. IftImperforate anus.](https://reader033.fdocuments.in/reader033/viewer/2022041502/5e228c12d2d3e271c931ed08/html5/thumbnails/29.jpg)
Common lethal anomaliesCommon lethal anomalies
Trisomy 13Trisomy 18
![Page 30: Division of Perinatology Department of Child Health ...ocw.usu.ac.id/course/download/1110000107-growth-and-development-system/... · other intestinal atresia. IftImperforate anus.](https://reader033.fdocuments.in/reader033/viewer/2022041502/5e228c12d2d3e271c931ed08/html5/thumbnails/30.jpg)
Trisomy 13 Cutis aplasia
Microtia + low setMicrotia + low set
Micrognathia
Cleft L/PMidline defect
Polydactyly
![Page 31: Division of Perinatology Department of Child Health ...ocw.usu.ac.id/course/download/1110000107-growth-and-development-system/... · other intestinal atresia. IftImperforate anus.](https://reader033.fdocuments.in/reader033/viewer/2022041502/5e228c12d2d3e271c931ed08/html5/thumbnails/31.jpg)
P i t i tTrisomy 18
Prominent occiput•Small for gestation•Heart diseases•Renal anomalies•Abnormal genitalia•Severe mental
Clenched hand
Lower-set earSevere mental
retardation
Clenched handMicrognathia
Hypoplastic nail
Rocker bottom feet
![Page 32: Division of Perinatology Department of Child Health ...ocw.usu.ac.id/course/download/1110000107-growth-and-development-system/... · other intestinal atresia. IftImperforate anus.](https://reader033.fdocuments.in/reader033/viewer/2022041502/5e228c12d2d3e271c931ed08/html5/thumbnails/32.jpg)