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Moderator : R4. 박미란
Presenter : R1. 강지은
Problem Case Conference
2010.7.2
반복되는 역류 및 그렁거림을
주소로 내원한 10개월 남아
Brief History
 Male, GA 40wks, 3190gm, NFS delivery at local hospital , without PNP
 2009년 8월 (생후 1개월) : Pneumonia , 소화 ...
 2010/3/22 EGD
vocal cord : unusual swelling
esophagus 내부는 ring shaped folding이 일부 관찰되며
eosinophillic esophagitis 와 유사한 소...
 2010/4/2 EGD
vocal cord swelling was much improved compared with prev. EGD
ring shaped morphology of esophagus disappear...
 2010년 4월 (생후 9개월) 본원 호흡기 분과 및 소화기분과 외래 방문
 2010년 4월 12일 bloody sputum (바닥에 피가 약간 묻어 있었음) 의심
되어 ER 통해 1st admission
방광암
...
 Development (2010년 4월 입원 당시) : 목가누기 , 뒤집기 및 배밀이 가
능 (6-7개월 정도의 발달단계)
P/E
 Wt : 8.8kg (25-50P) Ht : 73.1cm (50P),
 V/S ...
2010년 2월 28일 2010년 3월 3일
2010년 3월 14일 2010년 3월 21일
2010년 4월 13일
(본원 입원당시)
Initial Problem list
 Recurrent regurgitation (생후 7개월부터 시작됨)
 Recurrent pneumonia (생후 1개월, 7개월)
 Grunting sound (생후 2개월...
Initial Ass & Plan
 r/o Hemoptysis
 Recurrent regurgitation
 Recurrent pneumonia (2009/8, 2010/2)
 Grunting sound
 Mi...
Laryngopharyngeal Reflux vs
Gastroesophageal Reflux
P.Stavroulaki , International Journal of Pediatric Otorhinolaryngology...
Laryngopharyngeal Reflux
P.Stavroulaki , International Journal of Pediatric Otorhinolaryngology 2006
 Infant >> Regurgita...
HD#1(4/12)
 Chest CT >> (사진 첨부하기)
No evidence of bronchial artery hypertrophy.
No evidence of bronchiectasis.
No definite...
HD#4(4/15)
 ENT exam >> mild laryngomalacia
 Neurologic exam >>
DTR – (+) , both symmetric
Frog leg position 간헐적으로 관찰됨
...
Diagnosis & Plan
 Recurrent regurgitation
 r/o LPR >> GER : PPI 복용하며 경과 관찰 , L-tube feeding 유지
 Aspiration tendency d/t...
2nd Admission (2010.5.12)
 2010/5/5 퇴원 후 L-tube insertion 부위의 swelling 으로 보호자
제거 후 oral feeding . 구토 지속되어 ER 내원하여 L-tube
...
2010년 5월 11일
HD#3 (5/14)
 Both lung field 의 rale 지속적으로 청진됨.
 EMG /NCS 시행 : 이상 없음
 VFSS f/u : 4/16 에 비해 악화됨
PHARYNGEAL PHASE
1) Laryn...
 왼쪽 눈꺼풀의 ptosis, 왼쪽 팔의 근력 약화 , frog leg position ,
EMG/NCS 상 이상소견 보이지 않고 DTR (+) , Cricopharyngeal
incoordination 이 악화됨.
...
Brain MRI
HD#18 (5/28) – POD #3
 Extubation -> 이후 dyspnea 악화되어 6/1 reintubation
 PED transferred. (6/7)
 Hypothermia , BP 감소 및 le...
Hospital course
생후 1개월
(2009/8)
10개월
(2010.5)
9개월
(2010.4)
8개월
(2010.3)
2개월
(2009/9)
7개월
(2010.2)
Pneumonia
VFSS : aspirat...
1. Atypical teratoid/rhabdoid tumor in brain stem
 Poor swallowing : PEG insertion 등 시행 후
chemotherapy 진행중.
2. Recurrent ...
AT/RT
Dysphagia,
GER
Central
hypotonia
Associated
lung problem
Recurrent
pneumonia
Bronchiolitis
obliterans
?
?
증상별
Review
Case
BO &
AT/RT
증상별
Review
Case
BO &
AT/RT
Recurrent pneumonia in children
 Recurrent pneumonia : 2 episodes of pneumonia in a
single year, or 3 episodes over any t...
Recurrent pneumonia in children -
study
 2952 Patients admitted to The Hospital for Sick
Children, Toronto, with pneumoni...
Recurrent pneumonia in children
Timing of Underlying illness Diagnosis Relative to the
Number of Pneumonia Episodes
Abdull...
 Aspiration disorder >>
 Mean age : 6.3years
 Cerebral palsy (>50%)
 Recurrent pneumonia prior to diagnosis of
underly...
Aspiration syndrome
 All conditions in which foreign substances are
inhaled into the lungs
 Aspiration syndrome
 Oral o...
Dysphagia in children
 Act of swallowing
 Oral phase
 chew solid food to semisolid consistency , liquid to the back
of ...
 Most commonly presents as feeding or respiratory
difficulty
 Newborns and infants
 Sucking difficulty / or Sucking wit...
 Repeated episode of respiratory infection and experience
chronic bronchial congestion
 Examination
 Finding a cause of...
 Video analysis of sucking and swallowing
 Chromosomal karyotyping (Prader willi syndrome –
specific DNA test)
 Brain M...
Selected neurologic cause of Dysphagia
Question
 Recurrent pneumonia 혹은
aspiration syndrome 을 보이는 경우
CNS Imaging 을 반드시 시행해야
하는가?
Brain tumors in children under 1
years
 Intracranial neoplasm in neonates and infants
 Diagnostic & Therapeutic problem
...
 Department of Neurosurgery, Seoul National University
Hospital
 During 20 years (1977-1996) , 21 children
 Mean age : ...
 IICP sign : 11 patients (large head, vomiting, irritability)
 Head circumference : 4 patients 에서 90p 미만임. 이중 1
명은 tumor...
Brain tumors in children under 1 years –
study
S.K. Chung et al, J Korean Med Sci 1998;13: 65-70
Neurologic evaluation of infant
Tone
Hypotonic
Hypotonic and
weak
Development
Motor delay
only
Motor and
cognitive delay
C...
 Supraspinal conditions : brain, brainstem , cervical spinal junction
- Central hypotonia
 Basal ganglia, red nucleus da...
(A) pull to sit, (B) scarf sign, (C) shoulder suspension, and (D) ventral suspension.
Central hypotonia vs Peripheral
hypotonia
Amirsalari S. et al., Pak J Med Sci 2008;24(5):744-47
Central hypotonia vs Peripheral
hypotonia
Amirsalari S. et al., Pak J Med Sci 2008;24(5):744-47
증상별
Review
Case
BO &
AT/RT
AT/RT with recurrent pneumonia–
case
 1st admission due to pneumonia at age 2 months →
two subsequent admission at age 4 ...
AT/RT with recurrent pneumonia–
case
 During 3rd admission , left side upper and lower
extremity weakness and hypotonia ,...
Brain tumor with aspiration pneumonia-
case
 C/C : 5year old girl, choking
and breathless/ short
generalized convulsion
...
Brain tumor with aspiration pneumonia-
case
 barium esophagogram
 Next 6 weeks, dizziness,
unsteady on feet started .
De...
Brain tumor with chronic intermittent
stridor – case
 C/C : Persistent noisy breathing (2months ago)
 30 months old prev...
Brain tumor with chronic intermittent
stridor – case
 6 weeks later : dyspnea on exertion – oxygen
supplementation , cort...
Brain tumor presenting as GER
 Case 1.
 A boy, at age of 8 months started to vomit
 Chest , skull x-ray , Barium meal f...
증상별
Review
Case
BO &
AT/RT
Bronchiolitis Obliterans in children
 Bronchiolitis obliterans : histologically defined by the
presence of granulation ti...
Bronchiolitis Obliterans in
children
Etiology of bronchiolitis obliterans
1. Combination of history, physical exam . Infectious
disease evaluation, imaging study, PFT , lung biopsy
(occasionally)
...
HRCT – bronchiolitis obliterans
 Lung biopsy : gold standard for diagnosis
 But because heterogenous distribution of airway involvement,
open lung biops...
Atypical teratoid/rhabdoid
tumor
 Highly malignant, CNS tumor occurs less than 2 years
of age
 1ST Described in 1987 by ...
AT/RT clinical and radiographic
feature
 Signs and symptom : reflect location of the tumor
 Young patients with posterio...
Atypical teratoid/rhabdoid tumor-
Pathology
 AT/RT contains sheets of rhabdoid cells against a
background of primitive ne...
Atypical teratoid/rhabdoid tumor-
Pathology
 Immunohistochemistry :
 Rhabdoid cells : vimentin (+) , smoth muscle
actin ...
 Surgery
 Chmotherapy : post surgical adjuvant therapy
 Combination of cyclophosphamide, cisplatin,
etoposide, vincrist...
ATRT- associated lung problm ?
1. Metastasis
 High incidence of leptomeningeal dissemination (59% of
them at relapse) , b...
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  • LPR ; 표에서 나타나듯 reflux pattern 및 pathophysiology 에서 차이가 있고 (LPR : upper esophageal sphincter 의 문제 , GER: lower esophageal sphincter 의 문제. LPR 은 약물 치료에 더디게 반응하는 경향이 있다. )
  • 또한 LPR 은 infant 에서 regurgitation 등을 야기시킬 수 있고 laryngomalacia 등을 유발하는 원인이 되며, 이 환아에서 내시경 소견상 vocal cord 의 swelling 이 현저하게 관찰된 점 등을 보아 굳이 미리 진단을 내리자면 GER 보다는 LPR 에 더 가깝다고 볼 수 있을것입니다.
  • 7월 11일 경 부터는 식이 진행이 잘 되기 시작하였으며 항생제를 중단하였고
    7월 14일 입원 8일째 hyponatremia 및 hyperkalemia 확인되었으며, 환아의 이전 event 로 인해 brain sono f/u 하였고
    이때 우연히 확인한 abdomen sono 상 adrenal gland 의 hyperplasia 관찰되었습니다.

    이에 이날 17-ohp 검사를 시행하였습니다.
  • Toronto 에서 2000 년에 발표한 article 에서
  • Recurrent pneumonia 의 주된 원인인 aspiration syndrome 은 foreign substance 가 lung 으로 흡인될 수 있는 모든 condition 을 통틀어서 말하는 것으로
    GER 및 swallowing dysfunction즉 cricopharyngeal incoordination , 그리고 neurologic disorder 나 structural abnormality 가 모두 포함됩니다.
  • 이중에서 swallowing difficulty 즉 dysphagia 에 대해 간략하게 설명드리겠습니다.
    삼키는 작용은 총 3단계로 음식을 oral cavity 뒤쪽으로 보내는 oral phase 가 있으며 뇌신경 5, 7, 8번이 관여하게 되며 pharyngeal phase 때 soft palate
    의상승으로 nasal cavity 가 폐쇄되고 동시에 혀가 음식을 laryngopharynx 쪽으로 밀어주게 되고 laryx 가 앞 위쪽으로 움직여서 glottis 를 막는 일이 동시에 일어납니다.
    이 모든 작용에 관여하는 것이 V, VII, X, XI, XII 번 뇌신경으로 환아에서는 이 과정에서 특히 문제가 있었습니다. Esophageal phase 에서는 exophageal motility 에 의해 음식을 위로 밀어주게 되고 이 세 단계중 하나만 문제가 있어도 dysphagia 가 발생하게 됩니다.
  • 그림으로 표시하면 왼쪽그림과 같고, 오른쪽 그림과 같은 구조물들의 상호작용에 의해 삼키는 작용이 일어나게 됩니다.
  • 신생아와 영아에서의 dysphagia 는 sucking difficulty 나 sucking 은 있지만 swallowing 을 잘 하지 못하는 경우가 있으며 이 경우 nasal reflux 와 failure to weight gain
    식이 도중에 기침 및 apnea, feeding 중 noisy breathing sound 등이 발생하게 되고 aspiration 이 있을 경우 chocking 이나 coughing , tachypnea, cyanosis 등의 증상으로 감지할 수 있는 경우도 있으나 silent aspiration 도 있을 수 있는데 이는 미숙아의 75%, 신생아의 50% 에서 cough reflex 가 소실되어 있기 때문이기도 하며, neurologic insult 를 받은 영아의 경우에도 silent aspiration 이 있기 때문에 aspiration 증상을 보이지 않을 수 있습니다.
  • 그래서 반복되는 respiratory infection 으로 만성적인 bronchial congestion 이 발생하기도 합니다.

    시행할 수 있는 검사로는 우선 원인을 찾기 위해 oropharyngeal malformation 여부를 확인하는 검사 즉 small jaw 나 large tongue 을 보이지 않는지, cleft lip 이나 cleft palate 가 있지는 않은지, choanal atresia 여부를 확인하고 특히 음식 먹을때 stridor 나 chocking 을 보일 경우 TEF 여부를 확인합니다. 또한 neurologic exam 을 철저히 시행하여야 하며 특히 jaw jerk 나 jaw clonus 가 있을 경우 suprabulbar palsy 를 시사하는 소견일 수 있습니다. Central hypotonia 도 심한 경우에는 uncoordinate suck, swallowing difficulty 와 연관 있을 수 있음을 알고 있어야 합니다.
  • 그래서 swallowing 에 대한 video analysis 를 시행한 후 염색체 검사나 upper motor neuron finding 을 보이는 경우 Brain MRI 를 시행하여 CNS 의 이상 여부 혹은 일부 neuromuscular dystrophy 도 확인할 수 있습니다.
    Congenital myopathy 여부 확인을 위해 EMG/NCS 도 시행하여야 합니다.
  • Dysphagia 를 유발할 수 있는 neurologic 한 원인은 다음과 같습니다.
  • 여기까지 보았을때, 그럼 recurrent pneumonia 혹은 aspiration syndrome 을 보이는 경우에 모든 환아에서 고가의 brain MRI 같은 CNS imaging 을 꼭 해야만하나.. 라는 의문이 들게 됩니다.
  • 1세 미만 아이들에서 brain tumor 의 특성을 보시면 먼저 저희 환아처럼 특징적인 증상이 없는 경우가 많고, immature 한 brain 과 skull 이 increased intracranial pressure 에 적응하는 능력이 있기 때문에 진단이 지연되는 경우가 많습니다. 또한 방사선치료와 항암치료가 제한적으로 사용되기때문에 수술적치료가 가장 효과적인 경우가 많고, 그에 비해 operation 에 있어 mortality 가 꽤 높은 편이라는 문제점들이 있습니다.
  • 다음은 저희병원 신경외과에서 1998년에 1세 미만의 brain tumor 아이들 21명을 대상으로 낸 통계자료로 이 아이들의 평균연령은 5.8개월이었고 첫 증상은 역시 large head 가 가장 많았으며 vomiting이 그 다음이었으며, irritability, seizure ,abnormal eyeball movement 로 나타난 경우가 2case 씩 있었고 apnea, hoarseness, poor weight gain 등을 보인 경우도 1 case 씩 있었습니다.
  • IICP sign 을 보인 경우는 21명중 11명이었고 head circumference 가 90p 미만인 경우가 4명이었으며 이중 1명은 tumor size 가 4-5cm 정도로 컸음에도 불구하고 두위가 3-10p 정도였습니다.
  • 옛날 통계자료여서 ATRT 로 진단명이 붙은 환아는 없지만, 대부분의 환아가 IICP 혹은 seizure 로 인해 발견되었다는 것을 알 수 있습니다.
  • 이처럼 저희 환아와 같은 환아는 아주 적은 case 이고, 앞서 말씀드린 recurrent pneumonia 에서도 258명중 1case 만이 brain tumor 였기 때문에 모든 recurrent pneumonia 및 aspiration syndrome 을 보인 경우에 brain MRI 까지 바로 고려할 필요는 없지만, neurologic examination 을 꼼꼼히, 자주 시행하는 것이 불필요한 다른 검사를 줄이고 빨리 진단할 수 있는 가장 중요한 방법으로 생각됩니다.
    그래서 neurologic evaluation 으로 hypotonic infant 를 구별하는 방법을 간단히 설명드리자면,
    먼저 tone 을 확인하되, 단순히 hypotonia 만 있는지 아니면 muscle strength 도 같이 떨어져 있는지를 구별해야하고, development 를 평가할때 motor와 cognitive delay 여부를 함께 평가해야 합니다.
  • Pull to sit : neck 과 back 의 axial tone 및 sholder 와 arm 의 tone 을 확인하는 것으로을 보는 것으로 신생아는 head lag 를 보일 수 있지만 2개월쯤되면 아주 미약하게 남아있고, strenth 까지 확인할 수 있다.
    Scalf sign 은 누운 자세에서 손을 잡아 가슴을 가로질러 저항이 느껴지지 않을때까지 당기는 것으로 정상적으로 팔꿈치가 턱과 가슴의 midline 까지 도달할 수 있으나 hypotonic infant 에서는 지나갈 수 있다.
    Shoulder suspension test 시 hypotonic infant 는 검사자의 손에서 미끄러져나가려는 경향을 보이고 어깨의 tone 과 함께 head control 도 확인할 수 있다.
    Ventral suspension 은 정상적인경우 팔과 다리를 구부리고 머리를 수평으로 들어올릴수 있다.
  • 뉴욕 golisano children hospital 의 2006년 case 임. 생후 4개월된 아이가 pneumonia 로 3차례 입원하였으며 chest x-ray 상 rt. Upper lobe pneumonia 양상을 보임.
  • Brain MRI : 4cm 크기의 medulla oblongata 에서부터 발생한 mass 가 forth ventricle 을 누르고 있음.
  • INI-1 의 중요성 : PNET component 만 보이더라도 INI-1 negative 면 AT/RT 로 확진하기에 충분하다!!!!
  • Transcript of "Slide 1"

    1. 1. Moderator : R4. 박미란 Presenter : R1. 강지은 Problem Case Conference 2010.7.2 반복되는 역류 및 그렁거림을 주소로 내원한 10개월 남아
    2. 2. Brief History  Male, GA 40wks, 3190gm, NFS delivery at local hospital , without PNP  2009년 8월 (생후 1개월) : Pneumonia , 소화 아동병원에 5일간 입원  2009년 9월부터 그렁거림 지속됨: 순천향병원에서 경과 관찰.  2010년 2월 (생후 7개월)부터 수유 후 코, 입으로 역류되는 양상 관찰되 고 다시 pneumonia 발생하여 세브란스 병원 입원. 2010년 3월에 GER 에 대한 검사 시행 후 L-tube feeding 시작하고 PPI 및 motilium 복용 시작  Esophagography (2010.3.12) : Definite GE reflux 혹은 aspiration 관찰되지 않음.  VFSS (2010.3.18) : liquid 에서 delayed swallowing reflex 로 인한 spontaneous posterior falling, incomplete velopharyngeal port closure, liquid 에서 pre-swallowing, post swallowing aspiration 관찰됨.
    3. 3.  2010/3/22 EGD vocal cord : unusual swelling esophagus 내부는 ring shaped folding이 일부 관찰되며 eosinophillic esophagitis 와 유사한 소견 관찰됨. >> biopsy 상에는 chronic nonspecific inflammation without eosinophil >> correlated with GERD  2010/3/23 24 hr PH monitoring acid exposure upright 20.4%, recumbant 12.0%, Total 14.3% Bolus exposure (acid percent time ) upright 2.2%, recumbant 4.3%, total 3.8% (all reflux percent time ) upright 4.1%, recumbant 5.2%, total 4.9% Brief History – 외부병원 검사 결과
    4. 4.  2010/4/2 EGD vocal cord swelling was much improved compared with prev. EGD ring shaped morphology of esophagus disappear  Allergy test >> egg white, cow milk, peanut, soybean, pork, egg yolk : all negative total IgE ; 13.4 kIU/L (normal)  IgG/A/M : 609/20/156 (normal)  2010/2 Sputum culture : S. pneumonia Respiratory virus : (-) Brief History – 외부병원 검사 결과
    5. 5.  2010년 4월 (생후 9개월) 본원 호흡기 분과 및 소화기분과 외래 방문  2010년 4월 12일 bloody sputum (바닥에 피가 약간 묻어 있었음) 의심 되어 ER 통해 1st admission 방광암 <Family History> 1st Admission (2010.4/12~4.16)
    6. 6.  Development (2010년 4월 입원 당시) : 목가누기 , 뒤집기 및 배밀이 가 능 (6-7개월 정도의 발달단계) P/E  Wt : 8.8kg (25-50P) Ht : 73.1cm (50P),  V/S : BP 118/86mmHg, HR 146회/min, RR 40회/min, BT 37.1’C  Chest : coarse breathing sound with severe ronchi, w/o rale  N/E : 이상소견 없음. Initial lab  특이소견 없음 1st Admission (2010.4/12~4.16)
    7. 7. 2010년 2월 28일 2010년 3월 3일
    8. 8. 2010년 3월 14일 2010년 3월 21일
    9. 9. 2010년 4월 13일 (본원 입원당시)
    10. 10. Initial Problem list  Recurrent regurgitation (생후 7개월부터 시작됨)  Recurrent pneumonia (생후 1개월, 7개월)  Grunting sound (생후 2개월)  r/o Hemoptysis  Mild developmental delay
    11. 11. Initial Ass & Plan  r/o Hemoptysis  Recurrent regurgitation  Recurrent pneumonia (2009/8, 2010/2)  Grunting sound  Mild developmental delay 1. Primary lung disease r/o bronchiectasis Plan 1. Chest CT 시행. 2. L-tube feeding 유지하면서 PPI 치료 후 경과 관찰 3. VFSS f/u 4. Metabolic w/u 등 고려 2. r/o Laryngopharyngeal reflux , r/o GER 3. r/o Cricopharyngeal incoordination
    12. 12. Laryngopharyngeal Reflux vs Gastroesophageal Reflux P.Stavroulaki , International Journal of Pediatric Otorhinolaryngology 2006 Higher among infants (20-40%) than among children or adults (7-20%)
    13. 13. Laryngopharyngeal Reflux P.Stavroulaki , International Journal of Pediatric Otorhinolaryngology 2006  Infant >> Regurgitation/ vomiting, dysphagia, anorexia, growth failure, abnormal crying, sleep disorder, irritability or torticollis, upper airway problem (recurrent croup, laryngomalacia, subglottic stenosis), chronic respiratory disease  Children >>  Laryngotracheal complains – chronic cough , dyspnea,  Pharyngeal complains : persistent sore throat, globus sensation, referred otalgia, dental erosion  Rhinologic complains : nasal obstruction, rhinorrhea, headache  Chronic respiratory disease  Gastrointestinal symptoms: regurgitation/vomiting, nausea, chest or abdominal pain, heartburn
    14. 14. HD#1(4/12)  Chest CT >> (사진 첨부하기) No evidence of bronchial artery hypertrophy. No evidence of bronchiectasis. No definite evidence of active lung lesion. Multifocal peripheral atelectasis of both lungs. Mosaic pattern in both lower lung. Limited evaluation of the esophagus due to collapsed state.  Metabolic w/u >> MS/MS : negative , Lactic acid : 1.0 , Pyruvate 0.6, ammonia 44 TFT – free T4 1.06, T3 113, TSH 1.01 , Amino acid (s) : normal Carnitine (s) : normal , Organic acid (u) : normal 1st Admission (2010.4/12~4.16)
    15. 15. HD#4(4/15)  ENT exam >> mild laryngomalacia  Neurologic exam >> DTR – (+) , both symmetric Frog leg position 간헐적으로 관찰됨  VFSS >> ORAL PHASE 0) sucking : G 1) Tongue movement, bolus formation and mastication 등은 비교적 적절함. 2) Premature bolus loss는 관찰되지 않음. PHARYNGEAL PHASE 1) Laryngeal elevation 및 epiglottic closure가 비교적 적절함. 2) Small fluid에서 aspiration, Yoplait 에서 penetration 관찰됨. 3) Yoplait 에서 vallecular residue Gr1, pyriform sinus residue Gr1 으로 남음 4) Pharyngeal wall coating은 관찰되지 않음. 5) Pharyngeal triggering은 비교적 적절함. ESOPHAGEAL PHASE 1) UES relaxation은 비교적 적절함. 2) Pharyngeal reflux는 관찰되지 않음. 1st Admission (2010.4/12~4.16)
    16. 16. Diagnosis & Plan  Recurrent regurgitation  r/o LPR >> GER : PPI 복용하며 경과 관찰 , L-tube feeding 유지  Aspiration tendency d/t cricopharyngeal incoordination, LPR  Bronchiolitis obliterans  Budesonide nebulizer start.  Mild developmental delay  Hypotonia  Metabolic w/u 상 이상 없음.  EMG/NCS 외래에서 시행하기로 함. 1st Admission (2010.4/12~4.16)
    17. 17. 2nd Admission (2010.5.12)  2010/5/5 퇴원 후 L-tube insertion 부위의 swelling 으로 보호자 제거 후 oral feeding . 구토 지속되어 ER 내원하여 L-tube insertion  2010/5/9, 2010/5/12 : 구토 지속되고 dyspnea, lip cyanosis 보여 ER 통해 2nd admission  V/S BP : 131/98mmHg-HR : 163회/min- RR : 64회/min –BT : 38 'C  HC : 42.5cm (25-50p) , BW : 8.4kg (<- 8.8kg (2010.4))
    18. 18. 2010년 5월 11일
    19. 19. HD#3 (5/14)  Both lung field 의 rale 지속적으로 청진됨.  EMG /NCS 시행 : 이상 없음  VFSS f/u : 4/16 에 비해 악화됨 PHARYNGEAL PHASE 1) Laryngeal elevation이 감소되어 있으며 epiglottic closure가 다소 불완전함 2) Small fluid에서 aspiration관찰되며 YP에서 penetration관찰됨 3) Vallecular residue: SF/YP - Gr 1/1 Pyriform sinus residue: SF/YP - Gr 1/2 4) Pharyngeal wall coating이 관찰됨. 5) Pharyngeal triggering이 지연되어 있음. ESOPHAGEAL PHASE 1) UES relaxation은 비교적 적절함. 2) Pharyngeal reflux는 관찰되지 않음. 3) Esophagus까지 관찰하였으며, 이상소견 관찰되지 않음 HD#8 (5/19)
    20. 20.  왼쪽 눈꺼풀의 ptosis, 왼쪽 팔의 근력 약화 , frog leg position , EMG/NCS 상 이상소견 보이지 않고 DTR (+) , Cricopharyngeal incoordination 이 악화됨. - r/o central hypotonia - Brain MRI 시행함.  Lobulating contour well enhancing cystic and solid mass involving the medulla oblongata extending left lateral recess. -Suspicious obstructing fourth ventiricle. -accompanying hydrocephalus. DDx. 1. ATRT 2. Unusual ependymoma 3. medulloblastoma, less likely. - 5/20 EVD insertion  신경외과로 전과됨. 5/25 craniotomy and tumor removal HD#10 (5/21)
    21. 21. Brain MRI
    22. 22. HD#18 (5/28) – POD #3  Extubation -> 이후 dyspnea 악화되어 6/1 reintubation  PED transferred. (6/7)  Hypothermia , BP 감소 및 leukemoid reaction : r/o septic shock – Tazo+AMK start  Chemotx. 시행 예정이었으나 cyanosis 및 desaturation 반복되 고 CO2 retention -> lung care 위해 서5병동으로 전동. 현재 antibiotics 유지하며 nebulizer 시행중.  UGIS : 향후 feeding 진행 위해 시행 예정이었으나 swallowing 되지 않아 시행하지 못함. Hospital course HD#31 (6/9) HD#34 (6/12)
    23. 23. Hospital course 생후 1개월 (2009/8) 10개월 (2010.5) 9개월 (2010.4) 8개월 (2010.3) 2개월 (2009/9) 7개월 (2010.2) Pneumonia VFSS : aspiration Pneumonia 왼쪽 눈꺼풀의 ptosisPneumonia Budesonide nebulizer Grunting sound VFSS : aspiration Laryngeal elevation 감소 Chest CT : bronchiolitis obliterans L-tube feeding, PPI 사용 Brain MRI : brainstem mass Regurgitation
    24. 24. 1. Atypical teratoid/rhabdoid tumor in brain stem  Poor swallowing : PEG insertion 등 시행 후 chemotherapy 진행중. 2. Recurrent pneumonia → Bronchiolitis obliterans  Budesonide nebulizer 유지 중임  Infection control 및 lung care 진행중 Final Assessment & Plan
    25. 25. AT/RT Dysphagia, GER Central hypotonia Associated lung problem Recurrent pneumonia Bronchiolitis obliterans ? ?
    26. 26. 증상별 Review Case BO & AT/RT
    27. 27. 증상별 Review Case BO & AT/RT
    28. 28. Recurrent pneumonia in children  Recurrent pneumonia : 2 episodes of pneumonia in a single year, or 3 episodes over any time period. The chest radiograph should show resolution of radiologic changes between episodes.  Results from deficiencies in the local pulmonary or systemic host defences or from underlying disorders that modify the lung defences 1. Congenital malformation of upper of lower respiratory tract and cardiovascular system 2. Recurrent aspiration 3. Defects in the clearance of airway secretions- cystic fibrosis and ciliary abnormalities 4. Disorders of systemic and local immunities.
    29. 29. Recurrent pneumonia in children - study  2952 Patients admitted to The Hospital for Sick Children, Toronto, with pneumonia during 10 years  Recurrent pneumonia : 238 patients  Mean age : 3.7 years (2.5months ~15.6years)  Underlying illness >>  Aspiration syndrome (47.9%) due to oropharyngeal incoordination  Immune disorder(14.3%)  Congenital heart disease (9.2%)  Bronchial asthma (8.0%)  Anomalies of the respiratory system (7.6%)  Gastroesophageal reflux (5.4%)  Unknown (7.6%) Abdullah F. ect .Arch Pediatr Adolesc Med. 2000;154:190-194
    30. 30. Recurrent pneumonia in children Timing of Underlying illness Diagnosis Relative to the Number of Pneumonia Episodes Abdullah F. ect .Arch Pediatr Adolesc Med. 2000;154:190-194
    31. 31.  Aspiration disorder >>  Mean age : 6.3years  Cerebral palsy (>50%)  Recurrent pneumonia prior to diagnosis of underlying aspiration disorder : 4 case  Nemaline rod myopathy (EMG 상 진단됨)  Brainstem tumor (recurrent bilateral pneumonia from silent aspiration without neurological sign) Recurrent pneumonia in children Abdullah F. ect .Arch Pediatr Adolesc Med. 2000;154:190-194
    32. 32. Aspiration syndrome  All conditions in which foreign substances are inhaled into the lungs  Aspiration syndrome  Oral or gastric contents associated with gastroesophageal reflux (GER)  Swallowing dysfunction  Cricopharyngeal incoordination : transient pharyngeal muscle dysfunction , involves cricopharyngeal muscle spasm or achalasia of the superior esophageal sphincter. Who have a normal suck reflex but have incoordination during swallowing -> possibly associated with cerebral palsy  Neurological disorders  Structural abnormalities.
    33. 33. Dysphagia in children  Act of swallowing  Oral phase  chew solid food to semisolid consistency , liquid to the back of the oral cavity  Cranial nerve V,(trigeminal) VII(facial), XII(hypoglossal)  Coordination activity : jaw, facial, orobucal, lingual muscle  Pharyngeal phase  Soft palate rise : closes nasal cavity  tongue pushes food into the laryngopharynx  Laynx moves up and forward , closing glottis  Cranial nerve V, VII, X, XI, XII  Esophageal phase  Food pushed down toward the stomach, by esophageal motility Disturbance in act of swallowing : Dysphagia
    34. 34.  Most commonly presents as feeding or respiratory difficulty  Newborns and infants  Sucking difficulty / or Sucking without swallowing  Pooling of food in the mouth, slow sucking , nasal reflux, failure to weight gain , coughing during feeding , apnea, noisy breathing during feeding  Aspiration : choking, coughing , tachypnea, cyanosis “Silent” aspiration - Cough reflex is absent in 75% of premature infant and 50% of newborn - Absent in infants who have sufferd a neurologic insult Dysphagia in children
    35. 35.  Repeated episode of respiratory infection and experience chronic bronchial congestion  Examination  Finding a cause of dysphagia  Oropharyngeal malformation 여부 : small jaw, large tongue like Pierre –Robin syndrome or Beckwith syndorme , or cleft lip and palate, mandibular ankylosis , choanal atresia -> sucking difficulty  Feeding 시 choking 과 stridor 를 보일 경우 : TEF 의심  Thorough Neurologic Examination !! (cranial nerve V, VII, IX, X, XI,XII) - Jaw jerk or Jaw clonus : suprabulbar palsy - Muscle tone , strength, evaluation of stretch reflex and sensation - Central hypotonia 도 심한 경우에는 uncoordinate suck, swallow 와 연관 있을 수 있음. - Congenital myopathy, dystrophy 가 종종 dysphagia 와 연관되어 있음. Dysphagia in children
    36. 36.  Video analysis of sucking and swallowing  Chromosomal karyotyping (Prader willi syndrome – specific DNA test)  Brain MRI : significant upper motor neuron finding 을 보이는 경우 필요함!! (CNS injury 나 다양한 malformation , 일부 neuromuscular dystrophy를 감 별할 수 있음)  EMG/NCS Dysphagia in children
    37. 37. Selected neurologic cause of Dysphagia
    38. 38. Question  Recurrent pneumonia 혹은 aspiration syndrome 을 보이는 경우 CNS Imaging 을 반드시 시행해야 하는가?
    39. 39. Brain tumors in children under 1 years  Intracranial neoplasm in neonates and infants  Diagnostic & Therapeutic problem 1. Lack of specificity in the symptoms 2. The ability of the immature brain and skull to adapt to increased intracranial pressure -> Diagnostic Delay!! 3. Surgical resection of the tumor is the most effective therapeutic modality due to limited roles of radiation and chemotherapy 4. High operative mortality
    40. 40.  Department of Neurosurgery, Seoul National University Hospital  During 20 years (1977-1996) , 21 children  Mean age : 5.8 months Brain tumors in children under 1 years – SNUCH study S.K. Chung et al, J Korean Med Sci 1998;13: 65-70
    41. 41.  IICP sign : 11 patients (large head, vomiting, irritability)  Head circumference : 4 patients 에서 90p 미만임. 이중 1 명은 tumor size 가 4-5cm 임에도 3-10P.  Congenital tumor : 2 case (diagnosed with fetal US)  Prognosis : depend on pathological diagnosis, extent of surgical removal , general condition (allows chemotherapy) and patients’ permission to treat Brain tumors in children under 1 years – SNUCH study
    42. 42. Brain tumors in children under 1 years – study S.K. Chung et al, J Korean Med Sci 1998;13: 65-70
    43. 43. Neurologic evaluation of infant Tone Hypotonic Hypotonic and weak Development Motor delay only Motor and cognitive delay Careful history , examination and frequently requiring serial examinations !!!!!
    44. 44.  Supraspinal conditions : brain, brainstem , cervical spinal junction - Central hypotonia  Basal ganglia, red nucleus damage : extremity 의 passive movement 에 대한 저항의 증가  Cerebellum damage : axial, appendicular hypotonia  Segmental conditons: anterior horn cell , peripheral nerve, neuromuscular junction, muscle - Motor unit hypotonia  Afferent & efferent limb reflex 가 저하됨. Neurologic evaluation of infant
    45. 45. (A) pull to sit, (B) scarf sign, (C) shoulder suspension, and (D) ventral suspension.
    46. 46. Central hypotonia vs Peripheral hypotonia Amirsalari S. et al., Pak J Med Sci 2008;24(5):744-47
    47. 47. Central hypotonia vs Peripheral hypotonia Amirsalari S. et al., Pak J Med Sci 2008;24(5):744-47
    48. 48. 증상별 Review Case BO & AT/RT
    49. 49. AT/RT with recurrent pneumonia– case  1st admission due to pneumonia at age 2 months → two subsequent admission at age 4 months  Chest x-ray during 3 admission showed rt. upper lobe pneumonia .  w/u for recurrent pneumonia  Immunoglobulin level : normal  Culture, pertussis w/u , enterovirus, chlamydia – all (-)  Hypoallergic diet – unsuccessful  Barium swallowing study : nasopharyngeal reflux, mild GER and aspiration Diala et al. Golisano Children’s Hospital. Pediatric annals 35:9 ,2006
    50. 50. AT/RT with recurrent pneumonia– case  During 3rd admission , left side upper and lower extremity weakness and hypotonia , left head tilt, rt. tougue deviation , left eye ptosis detected in P/E → brain MRI : trilobed cystic mass arising within the left foramen of Luschka → Excisional biopsy : atypical rhabdoid- teratoid tumor Diala et al. Golisano Children’s Hospital. Pediatric annals 35:9 ,2006
    51. 51. Brain tumor with aspiration pneumonia- case  C/C : 5year old girl, choking and breathless/ short generalized convulsion  Past history : Noisy, irregular breathing at night beginning at age 18 months – diagnosed as mild asthma  On admission , she was postictal and cyanotic, with costal retractions and crackles heard at both lung bases  Dx : hypoxic convulsion associated with aspiration pneumonia Oades et al. Hillingdon hospital, AJDC vol 146(2), 1992
    52. 52. Brain tumor with aspiration pneumonia- case  barium esophagogram  Next 6 weeks, dizziness, unsteady on feet started . Developed apnea, pneumonia  N/E : truncal ataxia/ fasciculating tongue/ poor gag reflex  Brain MRI was performed. -> Gr 2 astrocytoma was confirmed by biopsy Oades et al. Hillingdon hospital, AJDC vol 146(2), 1992
    53. 53. Brain tumor with chronic intermittent stridor – case  C/C : Persistent noisy breathing (2months ago)  30 months old previously hearlthy girl  P/I : Upper respiratory infection with rhinorrhea, cough, noisy breathing , wheezing 2 months earlier. All symptoms had resolved except abnormal breathing  P/E : unremarkable  Examination:  Neck lateral x-ray : subglottic narrowing  Bronchoscopy : collapsing, inflamed, edematous arytenoid/ paradoxical movement of vocal cord on inspiration  Dx : Layngomalacia with paradoxical movement of vocal cord.  Tx : PPI (for suspected GER) A Brain Tumor Presenting as Chronic Intermittent Stridor- Erin et al. Oregon health and Sciences university 2008,
    54. 54. Brain tumor with chronic intermittent stridor – case  6 weeks later : dyspnea on exertion – oxygen supplementation , corticosteroid & epinephrine tx.  MRI scan (evaluate for possible Arnold – Chiari malformation)  Posterior displacement of the medulla by a 0.8x 1.6x 2.7cm mass  Final Dx : anaplastic astrocytoma with features of a plomorphic xanthoastrocytoma A Brain Tumor Presenting as Chronic Intermittent Stridor- Erin et al. Oregon health and Sciences university 2008,
    55. 55. Brain tumor presenting as GER  Case 1.  A boy, at age of 8 months started to vomit  Chest , skull x-ray , Barium meal follow thorough examination, jejunal biopsy, metabolic screening, abdomen CT -> 이상 없음  Vomiting continud to 11months -> wt 5.7kg (<3p) , Ht 72cm(10p), head circumference 44cm (3p)  Barium meal examination : GER , hiatal hernia -> Nissen’s fundoplication  After operation, stridor and sign of pseudobulbar palsy  Brain scan : dilatation of ventricle, tumor mass surrounding fourth ventricle -> biopsy 상 poorly differentiated glioma Brain stem glioma presenting as gastro-oesophageal reflux, Mahony et al. Archives of Disease in Childhood, 1987, 62
    56. 56. 증상별 Review Case BO & AT/RT
    57. 57. Bronchiolitis Obliterans in children  Bronchiolitis obliterans : histologically defined by the presence of granulation tissue plugs within the lamina of small airways and/or complete destruction of small airways leaving a scar  Chronic obstructive lung disease insult to the low respiratory tract , result in narrowing and/or complete obliteration of the small airways.  HRCT of children with respiratory disease has enhanced the ability of clinicians to diagnose BO noninvasively  Etiology : drug, exposures to toxic fumes, allergy reaction, collagen vascular disease, organ transplantation, or infection  In children ; following severe infection is m/c (usually adenovirus)  In adults : occupational inhalation injury, hypersensitivity pneumonia, autoimmune disorder
    58. 58. Bronchiolitis Obliterans in children Etiology of bronchiolitis obliterans
    59. 59. 1. Combination of history, physical exam . Infectious disease evaluation, imaging study, PFT , lung biopsy (occasionally)  Viral culture (especially adenovirus ) , Mycoplasma culture/PCR  Imaging : 40% of the patients had patchy consolidation and focal atelectasis on initial imaging ( in contrast to the usually unremarkable radiographs of patients with classical viral bronchiolitis)  HRCT : mosaic perfusion, vascular attenuation, central bronchiectasis (29 of 30 children with bronchiolitis obliteranse – Kim et al . Chest 2001) Bronchiolitis Obliterans in children Diagnosis of bronchiolitis obliterans
    60. 60. HRCT – bronchiolitis obliterans
    61. 61.  Lung biopsy : gold standard for diagnosis  But because heterogenous distribution of airway involvement, open lung biopsies nondiagnostic in up to 1/3 of patients.  Treatment  Azithromycin  Systemic corticosteroids  Frequently used.  Corticosteroid would not be expected to favorably impact the largely fibrotic component of the disease.  Should be given early while disease process is in the developing phase before airway fibrosis is complete  Pulse tx : IV methyl PD 30mg /kg over 1h daily for 3 days, repeated monthly, for 3-6 months – fewer side effect compared with oral corticosteroids.  IV immunoglobulin (1-2g/kg) : monthly Bronchiolitis Obliterans in children
    62. 62. Atypical teratoid/rhabdoid tumor  Highly malignant, CNS tumor occurs less than 2 years of age  1ST Described in 1987 by Rorke et al. – PNET or choroid plexus carcinoma prior to its recognition.  15% of children less than 36 months with malignant brain tumors have AT/RT (Pediatric Oncology Group and Pediatric Brain Tumor Consortium study )  Histologically resembles Rhabdoid tumor of the kidney – accurate diagnosis is difficult.  Frequently located in the posterior fossa, but also be located in supratentorial or multifocal compartment.
    63. 63. AT/RT clinical and radiographic feature  Signs and symptom : reflect location of the tumor  Young patients with posterior fossa tumors : hydrocephalus , early morning headache, vomiting, lethargy, regression of motor skills  Because of highly malignant tumor, typically have a fairly short history of progressive symptoms measured in days to weeks (unusual for symptoms to present for more than a few months)  No way to distinguish AT/RT from other malignant brain tumor based on clinical history  Imaging : nonspecific  Posterior fossa : invade cerebellar pontine angle, may have area of necrosis  Supratentorial : large, invasive, necrotic components
    64. 64. Atypical teratoid/rhabdoid tumor- Pathology  AT/RT contains sheets of rhabdoid cells against a background of primitive neuroectodermal cells, mesenchymal cells, or epithelial cells . Some tumors are composed almost entirely of rhabdoid cell (round nucleus with prominent nucleolus and abundant cytoplasm )
    65. 65. Atypical teratoid/rhabdoid tumor- Pathology  Immunohistochemistry :  Rhabdoid cells : vimentin (+) , smoth muscle actin (+) , epithelial membrane antigen (+)  Primitive neuroectodermal cells: NFP (+) GFAP(+)  Molecular cytogenetic screening :  Abnormal tumor suppressor gene , INI-1 (especially rhabdoid tumor, 최근 immunohistochemical staining 법이 개발됨. ) -> INI-1 mutation (stain 되지 않음) + PNET w/o clear rhabdoid component : AT/RT 로 확 진하기에 충분함!!
    66. 66.  Surgery  Chmotherapy : post surgical adjuvant therapy  Combination of cyclophosphamide, cisplatin, etoposide, vincristine, carboplatin, ifosfamide  Of the 33 patients with AT/RT – 69% progressed by 12 -24 weeks on therapy, 83% progressed by 12 months. Median survival was 193 days)  Radiation therapy Atypical teratoid/rhabdoid tumor- Treatment
    67. 67. ATRT- associated lung problm ? 1. Metastasis  High incidence of leptomeningeal dissemination (59% of them at relapse) , but systemic metastasis to other extraneural sites is extremely rare in children- 0.98% (ali et al. Journal of Neuro-Oncology ,2006) - One report of a patient with atypical teratoid rhabdoid tumor disseminating via a ventriculoperitoneal shunt (Korones et al. Med Pediatr Oncol,1999) - 6 year old AT/RT patient without VP shunt showed lung metastasis after 15 months after diagnosis (Elif et al. Journal of Neuro-Oncology ,2001). - Since craniotomy may cause tumoral implantation into the incised cutaneous tissue and migration of cells into the veins - In central nervous system tumors, metastases may occur soon after initiation of therapy or may be detected several years after diagnosis (mean time : 18 months (3-60months))
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