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Clinical Pathological Conference. 三軍總醫院 小兒部 黃文欽 / 蔡明志大夫 / 華一鳴大夫. Present illness. A one day-old-male infant was brought to our hospital due to rapid breathing and cyanotic lip

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clinical pathological conference

Clinical Pathological Conference

三軍總醫院 小兒部


present illness
Present illness
  • A one day-old-male infant was brought to our hospital due to rapid breathing and cyanotic lip
  • This baby was a product of 3.1 kg to a primiparous mother through a normal spontaneous vaginal delivery after 40 weeks of gestation
  • The dyspnea was apparent soon after birth and was associated with lip cyanosis
  • The apgar score was 7 at 1 minute and 9 at 5 minutes, respectively
  • Other than these symptoms there were no fever, tachycardia, etc. Prenatal history was not available
physical examinations
Physical examinations
  • An acutelyill looking baby
  • Vital sign: PR: 150/ min RR: 66-74 /min BT:36‘C
  • HEENT: a symmetric head and face, and opened anterior fontanel
  • Chest: symmetric expansion. The breathing sound was generally coarse without rales or wheezing, a mild subcostal retraction
  • Heart: unremarkable, no murmur or thrill
  • Abdomen: soft and flat. The liver was palpable by 3 fingerbreadths
  • Extremities and skin:unremarkable
hospital course
Hospital course
  • After admission he was placed on oxygen mask
  • The laboratory data done on day one as follow:
  • ABG: pH 7.3, pCO2 40.6 mmHg, pO2 24.8 mmHg, HCO3 19.1 mmol/l ( mixedmetabolic and respiratory acidosis )
  • Na 142 mM/l, K 5.1 mM/l Cl 106 mM/l
  • The blood sugar was 45 mg/dl
  • Cr: 0.8mg/dl
  • Chest X-ray film on the next day showedground glass appearanceof the lungandatelectasis in the right lower lung field
hospital course d3
Hospital course D3
  • On hospital day (HD) 3, tachycardia was noted in addition to tachypnea
  • A mild jaundice was also noted
  • Breathing sound was weak without rales
  • Volume decrease of the right hemithorax with hyperinfiltration of the left lung, dextroposition without cardiomegaly, and right lower lung field lesions
  • Oxygen saturation became 74% and blood pressure rose to 75/39 mmHg from 49/20 mmHg after dopamine infusion
hospital course d4
Hospital course D4
  • He was placed on ventilator on HD 4, with FiO2 0.5, after which ABGA revealed pH 7.26, pO2 124 mmHg, pCO2 45.5 mmHg, HCO3 20.6mmol/l, O2 saturation 95% and base excess -6mmol/l( mixedmetabolic and respiratory acidosis )
  • Antibiotics was also given because of persistent right lower lobe lesion
  • Respiratory difficulty did not improve. And his urine output became gradually decreased (shock)
hospital course d49
Hospital course D4
  • Blood glucose level remained in the range of 50-58mg/dl
  • The patient had no fever or jaundice
  • Urine culture grew nothing
  • CxR showed hypervascularity (pulmonarycongestion) in addition to persistent right lower lobe shadow and lung infiltration
hospital course d6
Hospital course D6
  • The respiratory difficulty was still present, and pneumonic infiltration was noted in the left lung
  • ABGA showed pH 7.33, pCO2 44 mmHg, pO2 66.2 mmHg, HCO2 23.8mmol/l, O2 saturation 90% and base excess -1.7 mmol/l (compensated respiratory acidosis)
  • He was still on ventilator with FiO2 0.6, RR 15 and PIP 18.
  • WBC count was 40,000/cmm with 84%neutrophils
hospital course d7
Hospital course D7
  • Rhonchi became manifest, and the breathing sound decreased in the right lower lobe
  • Emphysematous picture was seen in the left lung field
  • Chest ultrasonography showed right lower lobe collapse and suggested pneumonia
laboratory data
Laboratory data
  • WBC:2720/cmm (normoblast 2%, seg 57, lymphocyte 30%, monocyte 10%, band form 3%)
  • RBC 4.99x106/cmm, hemoglobin 16.9gm/dl, hematocrit 51%, platelet 150,000/cmm
  • Ca 8.1 mg/dl, phosphorus 7.4 mg/dl, ionized Ca 3.3 mg/dl
  • lactic dehydrogenase 1088 IU/I, creatine phosphokinase 820 IU/I
  • bilirubin 7.7 mg/dl
  • Prothrombin time was 38.7 sec(19%) and activated PTT 267 sec
hospital course d8
Hospital course D8
  • The baby started to show arrhythmia
  • The blood gas data became aggravated and no self respiration (respiratoryfailure) was noted
  • Oxygen saturation of blood became progressively worse despite ventilator setting of FiO2 0.85
  • Chest wall retraction and pale wet skin were noted. The baby died on hospital day 9

Day 1 Day 4 Day 6 Day 7

PH 7.37.26 7.33

PO2 24.8 124 66.2

PCO2 40.6 45.5 44

HCO3 19.1 20.6 23.8

SaO2 95% 90%

Glucose 45 50-58

WBC 40,0002720

N/L/M 84% 57/30/10

Hgb 16.9

Hct 51%

T/F Ca 8.1/3.3

PO4 7.4

LDH 1088

CPK 820

PT/PTT 38.7/267


Day 2

Day 3

Day 4

Day 9

major problems
Major problems
  • Respiratory distress
  • Atelectasis of right lung with dextroversion
  • Multiple cyst-like structure over the right lung field
  • Shock: BP down , decreased urine output, metabolic acidosis, tachycardia, pale wet skin
minor problems
Minor problems
  • Tachycardia
  • Jaundice
  • Leukocytosis then leukopenia
  • Prolonged PT/PTT
  • Hypocalemia
  • High level of LDH and CPK
  • Emphysematous change of left lung field
  • Birth history: is there Hx of birth trauma during delivery? Meconiusm-stained?
  • Chest CT scan or MRI
  • Echocardiography, chest sonography, abdominal sonography
  • Had EKG been done and the results
  • Blood culture, CRP, CSF culture, virus culture, sputum culture
  • Had bronchoscopy been done and the results
causes of respiratory distress in full term neonate
Causes of respiratory distress in full-term neonate

Common Meconium aspiration pneumonia

Congenital heart disease

Transient tahcypnea

Persistent fetal circulation

Congenital pneumonia

Uncommon Pneumothorax

Congenital anomalies



Vocal cord paralysis

Pleural effusion

Severe anemia

Pulmonary hypoplasia

Pulmonary lymphangiectasia


Causes of Respiratory distress from Congenital Heart Disease

Age of Onset Cause

At birth HLHS

Venous overload lesions:

Severe tricuspid or

pulmonary insufficiency

Large systemic AV fistula

First wk TGA

PDA in small premature infants

HLHS(with more favorable anatomy)

TAPAR, particular with pulmonary venous obstruction


Systemic AV fistula

Critical AS or PS

causes of respiratory distress in full term neonate23
Causes of respiratory distress in full-term neonate

Congenital anomalies Tracheoesophageal fistula

Choanal atresia

Diaphragmatic hernia

Eventration of diaphragm

Cystic adenomatoid malformation

Lobar emphysema

Pierre-Robin syndrome

Thyroid goiter

Congenital pulmonary cyst


Vascular rings and slings

Arteriovenous malformation

Subglottic stenosis

causes of atelectasis in full term neonate
Causes of Atelectasis in full-term neonate

External compression of pulmonary parenchyma

Pleural effusion, pneumothorax, intrathoracic tumors, lobar emphysema, diaphragmatic hernia, cystic adenomatoid malformation

Endobronchial obstruction

Enlarged LN, tumor, cardiac enlargement, foreign body, mucoid plug, broncholithiasis

Intraluminal obstruction

Foreign body, granulomatous tissue, pulmonary abscess

Intrabronchiolar obstruction

Bronchiolitis, interstitial pneumonitis, pneumonia

Respiratory compromise or paralysis

Neuromuscular abnormalities, osseous deformitis

causes of multiple cyst like lesions in lung
Causes of multiple cyst-like lesions in lung
  • Congenital pulmonary cysts
  • Acquired lung cysts (pneumatoceles)
  • Congenital cystic adenomatoid malformation
  • Congenital bronchogenic cysts
  • Neurenteric duplication cysts
  • Shock is characterized by inadequate circulatory provision of oxygen, leads to lactic acidosis
  • Shock is a progressive process
  • Hypovolemic and septic shock are the most common causes of shock in children
  • Cardiogenic shock maybe seen in neonates with congenital heart disease
causes of shock
Causes of shock

Septic Bacterial, Viral, Fungal

Cardiogenic Ischemic insult Cardiomyopathy Congenital heart disease

Distributive Neurogenic



Allergic reactions

Hypovolemic Enteritis, Hemorrhage

Extensive burns

Diabetes insipidus

Adrenal insufficiency

Obstructive Tension pneumothorax Pericardial tamponade

serious systemic illness in newborns differential diagnosis of neonatal septic shock
Serious Systemic Illness in Newborns: Differential Diagnosis of Neonatal Septic Shock

Cardiac Metabolic

Congenital: Hypoplastic left heart Hypoglycemia

syndrome, other structural Dx, PPHN Adrenal disorders: Adrenal

Acquired: Myocarditis, hypovolemic or hemorrhage, insufficiency,

PPHN congenital hyperplasia

Gastrointestinal Inborn errors of metabolism

Necrotizing enterocolitis Neurologic

Spontaneous GI perforation ICH: spontaneous, child abuse

Structural abnormalities Hypoxic-ischemic encephalopathy

Hematologic Neonatal seizures

Neonatal purpura fulminans Infant botulism

Immune-mediated thrombocytopenia Respiratory

Immune-mediated neutropenia Respiratory distress sydrome

Severe anemia Aspiration pneumonia

Malignancies(congenital leukemia) Lung hypoplasia

Hereditary clotting disorders Tracheoesophageal fistula


serious systemic illness in newborns differential diagnosis of neonatal sepsis
Serious Systemic Illness in Newborns: Differential Diagnosis of Neonatal Sepsis


Bacteremia or meningitis (Streptococcus pneumonia, Haemophilus influenza type b, Neisseria meningitidis)

Viral illness (influenza, enteroviruses, hemorrhagic fever group, HSV, RSV, CMV, EBV)

Encephalitis (arboviruses, enteroviruses, HSV)

Rickettsiae (Rocky Mountain spotted fever, Ehrichia, Q fever)


Vaccine reaction (pertussis, influenza, measles)

Toxin-mediated reaction (toxic shock, staphylococcal scalded skin syndrome)

Pneumonia (bacterial, virus, mycobacteria, fungi, allergic reaction)


Causes of Cardiogenic shock

During the 1st week of newborns

Arteriovenous malformation

Hypoplastic left heart syndrome

Coarctation of aorta

Interruption of aortic arch

Aortic stenosis

Transient myocardial ischemia

possible diagnosis
Possible Diagnosis

Respiratory distress+ Atelectasis+ multiple cyst-like

lesions in lung

  • Cystic adenomatoid malformation

Respiratory distress+ Atelectasis

  • Pulmonary hypoplasia
  • Lobar emphysema
  • Diaphragmatic hernia

Respiratory distress+ Shock

  • Hypoplastic left heart syndrome
  • Congenital pneumonia with septic shock
congenital cystic adenomatoid malformation
Congenital Cystic Adenomatoid Malformation
  • Congenital cystic adenomatoid malformation of the lung is anuncommon cause of respiratory distress in neonates and infants
  • Nevertheless, it is one of the most common pulmonary congenital anomalies
  • It consists of hamartomatous or dysplastic lung tissue mixed with more normal lung and usually confined to one lobes
  • It is characterized by a multicystic mass of pulmonary tissuewith an abnormal proliferation of bronchial structures

Congenital Cystic Adenomatoid Malformation

  • The lesion is a type of hamartoma with cysts structure and rarely may have malignant change
  • Associated malformations are present in about 20% of affected infants, like renal agenesis, jejunal atresia, diaphragmatic hernia, hydrocephalus, and skeletal Dx

Congenital Cystic Adenomatoid Malformation

  • Among infants in whom the malformation isdiagnosed postnatally, 50% to 75% will have someform of respiratory distress or compromise, including tachypnea,retractions, and cyanosis
  • The postnatal diagnosis is made with plain chest radiography,which demonstrates multiple air-filled thin-walled cysts thatvary in size

Congenital Cystic Adenomatoid Malformation

  • Type I is composed of variable-size cysts, with at least onedominant cyst (>2 cm in diameter) and this isthe most common (75%) form
  • A type II congenital cystic adenomatoid malformation is composedof smaller, more uniform cysts less than 1 cm in diameter (10%to 15% of all congenital cystic adenomatoid malformations)
  • A type III congenital cystic adenomatoid malformation is a solidmass composed of bronchoalveolar microcysts

Type I congenital cystic adenomatoid malformation.

  • Anteroposterior and (b) lateral chest radiographs of
  • a newborn show a multicystic mass occupying the right
  • hemithorax (note the variable size of the cysts, some of
  • them >2 cm)

Type II congenital cystic adenomatoid malformation. (a) Anteroposterior chest radiograph of a newborn shows a heterogeneous bubbly mass in the left lung displacing mediastinal structures to the right. (b) Coronal T1-weighted image in the same patient shows cysts (arrows) smaller than 2 cm in diameter


Type III congenital cystic adenomatoid malformation. Anteroposterior chest radiograph of a premature girl weighing 700 g shows an irregular microcystic mass affecting the left lung and displacing the mediastinum to the right. There is an associated dextrocardia


Congenital Cystic Adenomatoid Malformation

  • In severe cases, thelung may be hyperexpanded, with mediastinal shift, a flat hemidiaphragm,and herniation of the lung to the contralateral side
  • CT canbe useful for characterizing congenital cystic adenomatoid malformationby showing its location and extent
  • The treatment of choice is lobectomy as soon as possible after delivery
  • In infants subjected to early lobectomy, PPHN secondary to lung hypoplasia has been successfully managed with ECMO
pulmonary hypoplasia
Pulmonary Hypoplasia
  • Pulmonary hypoplasia is defined as deficient or incomplete developmentof the lungs
  • It is characterized by the presence of bothbronchi and alveoli in an underdeveloped lobe
  • It is caused by factors directly or indirectly compromising the thoracic space available for lung growth, such as a congenital diaphragmatic hernia, throacic dystrophy, pleural effusions with fetal hydrops, and cystic adenomatoid malformation
  • Intrathoracic causes, such as a congenital diaphragmatic hernia, are the most common
pulmonary hypoplasia41
Pulmonary Hypoplasia
  • The most common manifestation is early respiratory distressafter birth, cyanosis, tachypnea, hypoxia, hypercapnea, andacidosis
  • Plain radiographs demonstrate decreased aeration of the affectedhemithorax (more frequent in the right lung) and a small thoraciccage
pulmonary hypoplasia42
Pulmonary Hypoplasia
  • A common finding is a displacement of the mediastinum to theside of the hypoplasia
  • In some cases, a cystic appearance is encountered

CxR of a 7-month-old infant shows opacity of the left hemithorax and

small left lung (arrows) with ipsilateral displacement of the mediastinum, secondary to repaired Bochdaleck hernia


Lobar Emphysema

  • Congenital lobar emphysema is characterized by progressive overdistentionof a lobe, sometimes two lobes
  • The most commonly affected lobe is the left upper lobe, followedby the middle lobe
  • Congenital lobar emphysema maybe associated with other anomalies (cardiovascular system isinvolved in 12%–14%)

Lobar Emphysema

  • Most patients become symptomatic during the neonatalperiod, most before 6 months of age
  • Respiratorydistress is the most common symptom at presentation, signs range from mild tachypnea and wheeze to severe dyspnea with cyanosis
  • Diagnosisis obtained by means of chest radiography and CT, which showhyperinflation of the segment or lobe affected

Diaphragmatic Hernia

  • Herniation of abdominal contents into the thoracic cavity
  • Esophageal hiatus-hiatal
  • Paraesophageal
  • Morgagani-retrosternal
  • Bochdalek-posterolateral
  • Congenital diaphargmatic hernia=Bochdalek
  • Profound respiratory distress in the neonatal period
  • Significant mortality (40-50%)

Diaphragmatic Hernia

  • Severe respiratory distress within 1st hour of life
  • A small group will present beyond the neonatal period
  • Absence of breath sounds and shift of heart sounds
  • CxR is usually diagnostic
  • The lateral view frequently demonstrate the posterior portion of diaphragm

Diaphragmatic Eventration

  • A thinned diaphragmatic muscle producing elevation of the entire hemidiaphragm
  • Commonly, the anterior aspect of the hemidiaphragm
  • Most eventrations are asymptomatic and do not require repair
hypoplastic left heart syndrome
Hypoplastic left heart syndrome
  • HLHS occurs in 1% of all congenital heart defect or 9% of such defects in newborns
  • HLHS is the most common cause of death from cardiac defects during the first month of life
hypoplastic left heart syndrome52
Hypoplastic left heart syndrome
  • HLHS includes a group of closely related anomalies characterized by hypoplasia of the LV and encompasses atresia or critical stenosis of the aortic or mitral valves and hypoplasia of the ascending aorta and aortic arch
  • The LA is small
  • Circulatory shock and metabolic acidosis occurs after birth when the PDA closes and higher systemic than pulmonary vascular resistance
hypoplastic left heart syndrome54
Hypoplastic left heart syndrome
  • A neonate with HLHS becomes critically illwithin the first few hours to the first few days of life
  • Tachycardia, dyspnea, pulmonary crackles, weak peripheral pulses, and vasoconstricted extremities are characteristic
  • Heart murmur usually is absent
hypoplastic left heart syndrome55
Hypoplastic left heart syndrome
  • Infants with HLHS usually develop symptoms shortly after birth as following:
  • cyanosis (blue color of the skin, lips, and nailbeds)
  • pale skin
  • sweaty or clammy skin
  • cool skin
  • heavy and/or rapid breathing
  • fast heart rate
hypoplastic left heart syndrome56
Hypoplastic left heart syndrome
  • The ECG almost always showed RVH
  • CxR characteristically show pulmonary venous congestion or pulmonary edema
  • ABG reveal a slightly decreased PO2 and a normal PCO2
  • Severe metabolic acidosis out of proportion to the PCO2 is characteristic of the condition (caused by markedly decreased cardiac output)
hypoplastic left heart syndrome58
Hypoplastic left heart syndrome
  • Echo findings are diagnostic
  • Pulmonary edema and CHF develop in the first week of life, might manifest as sepsis
  • Circulatory shock and progressive hypoxemia and acidosis result in death, usually in the first month of life, like septic shock
total anomalous pulmonary venous return
Total anomalous pulmonary venous return
  • TAPAR accounts for 1% of all congenital heart defects
  • A marked male preponderance for the infracardiac type (M:F ratio of 4:1)
  • No direct communication exists between the pulmonary veins and the LA
  • They drain anomalously into the systemic venous tributaries or into RA
  • Supracardiac: 50%, into R’t SVC
  • Cardiac: 20%, into coronary sinus or RA
  • Infracardiac (subdiaphragmatic): 20%, into portal veins, ductus venosus, hepatic veins, or IVC
  • Mixed type: 10%, combination of the other type
total anomalous pulmonary venous return with pulmonary venous obstruction
Total anomalous pulmonary venous returnwith pulmonary venous obstruction
  • Hx: marked cyanosis and respiratory distress, cyanosis worsens with feeding
  • Moderate to marked cyanosis and tachypnea with retraction are present , fail respond to ventilator
  • Heart murmur is usually absent
  • Hypoxemia does not respond to oxygen inhalation
  • Pulmonary crackles and hepatomegaly are usually present
  • EKG: RVH in the form of tall R waves
  • CxR: “snowman” appearance
  • The heart size is normal or slightly enlarged
  • The lung fields reveal finding of pulmonary edema, which may be confused with pneumonia or HMD

Neonatal pneumonia

  • Infection may be acquired in utero (transplacental),
  • Pneumonia in newborn is often associated with the development of hyaline membranes
  • Atelectasis is a result of damage to the alveolar capillary membrane and leakage of proteins into the alveolus

Pathogens for pneumonia in neonates

Group B ß-hemolytic Streptococcus

Escherichia coli

Listeria monocytogenes

Herpes simplex virus

Haemophilus influenzae

Mycobacterium tuberculosis

Treponema pallidum


Syphilis;Rubella; Varicella zoster


Human immunodeficiency virus


Chlamydia trachomatis


Early Onset Neonatal pneumonia

  • Group B Streptococcus has emerges as a predominant pathogen in neonatal pneumonia
  • This infection occurs most often in very low-birth-weight infants
  • Early-onset pneumonia from group B Streptococcus may progress rapidly to shock or death
  • Diagnosis is confirmed by blood, CSF, virus or urine culture
my impression
My Impression

Congenital Lung lesion

  • Congenital cystic adenomatoid malformation
  • Pulmonary hypoplasia
  • Diaphragmatic hernia or eventration


  • Hypoplastic left heart syndrome
  • Congenital pneumonia with septic shock
diagnostic workup
Diagnostic Workup
  • Chest CT scan
  • Echocardiography
  • Blood culture, sputum culture, CSF culture

Cystic adenomatoid malformation of the lung, bilateral renal agenesis and left heart hypoplasia. An unusual association in Potter's syndrome

We describe an autopsy case of congenital cystic adenomatoid malformation of the lung (CCAM) associated with bilateral renal agenesis. Prenatal ultrasound examination showed additional left heart hypoplastic syndrome. A therapeutic abortion was induced at 23 weeks of gestation. The association CCAM-bilateral renal agenesis is a rare condition (5 cases previously described) which has to be known because of the mitigation effect of the CCAM on the oligohydramnios determined by bilateral renal agenesis. However, this instance is usually associated with oligohydramnios. The pathogenesis of polyhydramnios in isolated CCAM is discussed in regard with these data.

Carles D, Dallay D, Serville F, Maugey-Laulom B, Alberti EM, Tissot H, Weichhold W, Wursten-Guitton

FUnite de Foetopathologie, Groupe Hospitalier Pellegrin, Bordeaux

Ann Pathol. 1992;12(6):367-70.