Case report demo 1
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NATIONAL HOSPITAL NATIONAL HOSPITAL OF PEDIATRICSOF PEDIATRICS
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AN ATYPICAL RATHKES CLEFT CYST AN ATYPICAL RATHKES CLEFT CYST AND AND
DIFFERENTIAL DIAGNOSISDIFFERENTIAL DIAGNOSIS
Reporter: DR. Hong Nhung Le
Imaging Diagnostic Department
NATIONAL HOSPITAL OF PEADIATRICS
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INDIVIDUAL INFORMATIONINDIVIDUAL INFORMATION
• Name: HOAI LINH PHAM
• Sex: Female
• Date of birth: November, 9th, 2000
• Address: 516 Alley, Tran Tat Van street, Kien An district, Hai Phong city
• Telephone number:01696309762
• Date of examination: June, 21st, 2012- NHP
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CLINICAL MANIFESTATIONCLINICAL MANIFESTATION
• Transient headache last for 3 months in recent year
• At the time of examination : headache attacks 3 times a week in average
• No visual disturbance, no hemianopsia.
• Individual history: normal development
• Family history: no special finding.
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SUBCLINICAL TESTSUBCLINICAL TEST• Bone Age: approximately 10 years
• Endocrinological Test: Normal pituitary funtion
GH= 1.8 µg/l (BT <5.0 µg/l)
Prolactin 7.3 µg/l (BT <15.0 µg/l)
Thyrotropin 1.0 mU/l (BT 0.1–4.0 mU/l)
Luteinizing hormone 21.30 IU/l (BT 15–67 IU/l);
Follicle-stimulating hormone 15.50 IU/l (BT 20–40 IU/l);
Adrenocorticotropic hormone 16.3 pg/ml (BT 4.4–48.0 pg/ml)
Cortisol 10.8 nmol/L (BT 3.2–13.9 nmol/L)
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MRI FindingsMRI Findings(T2W, Axial)(T2W, Axial)
Cystic mass: D=15mm
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MRI FindingsMRI Findings(T1W, sagital)(T1W, sagital)
Cystic mass in the Sellar and supprasellar extension
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MRI FindingsMRI Findings((FLAIR coronal)FLAIR coronal)
Hypersign compared to CSF
mass
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MRI FindingsMRI Findings(T1W Axial-Postcontrast)(T1W Axial-Postcontrast)
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MRI FindingsMRI Findings(T1W Axial-Postcontrast)(T1W Axial-Postcontrast)
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MRI FindingsMRI Findings(T1W Axial-Postcontrast)(T1W Axial-Postcontrast)
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MRI RESULTMRI RESULT
Cystic mass in the sella and suprasellar extension:
AN ATYPICAL RATHKES CLEFT CYST?
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DIFFERENTIAL DIAGNOSISDIFFERENTIAL DIAGNOSIS
• Rathkes cleft cyst• Epidermoid cyst • Craniopharygioma (CR)
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RATHKES CLEFT CYST
EPIDERMOID CYST
CRANIOPHARY-GIOMA
DEMOGRAPHICS At any ageGender: F>M
At any age Adamatinomatous: 5-15 yearPapilary: above 50 yearGender: M=F
FEATURE - 40% infrasella, 60% suprasellar extension- Size: 5-15mm
- Suprasellar- Varying size
- 75% suprasella; 21% combination; 4% infrasella- Size >5cm
CLINICAL SIGNS • Asymtomatic• Symtomatic: - Pituitary disfuntion - Headache - Visual disturbance
- Visual disturbance
- Headache - Visual disturbance- Pituitary disfuntion
DIFFERENTIAL DIAGNOSIS
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RATHKES CLEFT CYST
EPIDERMOID CYST
CRANIOPHARY-GIOMA
MRI - Varying signal. - Intracystic nodule : 70%.- FLAIR:hypersign - No internal enhance;
+/- rim of compressed pituitary
- Varying signal- TIWI: hypersignal- No enhance or
minimal rim enhance,
- Restriction on DWI
- 90%Calcified, solid, cyst
- FLAIR=hyper
- 90% Enhance =rim(capsule)+nodule(solid)
CT-SCANNER - 75% hypointense,- Nonenhanced
From -100 to +30 HU Adamatinomatous: 90% calci
DIAGNOSTICCHECKLIST
Intracystic nodule FAT on CT Strong enhancement
DIFFERENTIAL DIAGNOSIS
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RATHKES CLEFT CYST
EPIDERMOID CYST
CRANIOPHARY-GIOMA
PROGNOSIS - Most stable.- May shrink and disappear.- Noneoplasm
- Most stable- Noneoplasm
- Slow growing benign neoplasm- Survival>10Y:60%
TREATMENT - Conservative- Aspiration/excision if Symtomatic
- Primary sugery - Surgery and radiation
RECURRENCE - Rate<1/3 - Rate<1/3 - Size > 5 cm: ~80%- Size <5 cm: ~20%
DIFFERENTIAL DIAGNOSIS
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TYPICAL RATHKES CLEFT CYSTTYPICAL RATHKES CLEFT CYST
Intracystic nodule
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INTRACYSTIC NODULEINTRACYSTIC NODULE (Continuing)(Continuing)
Hypersignal on T1W Hyposignal on T2W
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ATYPICAL RATHKES CLEFT CYSTATYPICAL RATHKES CLEFT CYST
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TYPICAL EPIDERMOID CYSTTYPICAL EPIDERMOID CYST T1 W coronal Precontrast:T1 W coronal Precontrast:
Cystic mass in the suprasella
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TYPICAL EPIDERMOID CYSTTYPICAL EPIDERMOID CYST T1 W coronal PostcontrastT1 W coronal Postcontrast
Rim enhanced mass
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EPIDERMOID CYST EPIDERMOID CYST DWI-ADCDWI-ADC
Restriction on DWI
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TYPICAL CRANIOPHARYGIOMATYPICAL CRANIOPHARYGIOMA
Strong enhancement at capsule and solid structure
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DISCUSSIONDISCUSSION
• Imaging technique on MRI.
• Embryology of Rathkes pouch and Rathkes cleft cyst.
• Diagnostic checklist.
• Treatment strategy
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IMAGING TECHNIQUEIMAGING TECHNIQUE
• High resolution:2-3mm (thick)
• Sagital T1 pre+postcontrast • Coronal T1 pre+postcontrast• Axial T2W
• Dynamic gadolium enhance coronal T1 for microadenoma (20s subsequence)
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EMBRYOLOGYEMBRYOLOGY
A: Infundibulum and Rathke's pouch develop from neural ectoderm and oral ectoderm, respectively.
B: Rathke's pouch constricts at base.
C: Rathke's pouch completely separates from oral epithelium.
D: Adenohypophysis is formed by development of pars distalis, pars tuberalis, and pars intermedia; neurohypophysis is formed by development of pars nervosa, infundibular stem (median eminence)
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DIAGNOSTIC CHECKLISTDIAGNOSTIC CHECKLIST
• On MR images, Rathke's cleft cysts (RCC) show various signal intensities.
• The key figure considered to be indicative of RCC is intracystic nodule.
• Finding intracystic nodule difficult and overlook when similar to signal of cystic surrounding.
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TREATMENT STRATEGYTREATMENT STRATEGY
• Symtomatic Rathkes cleft cyst (RC)and Epidermoid cyst (EC) have the same treatment strategy.
• Symptomatic RCC or EC should be treated carefully with simple evacuation, irrigation, and biopsy via a transsphenoidal route.
• Craniopharygioma require a different treatment strategy, including the choice of meticulous dissection from the hypothalamus or radiation or both.
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CONCLUSIONCONCLUSION
• Our case demonstrates any potential lesion may occur. We should take the follow-up examination regularly by MRI to evaluate the lesion’s progress (6),(10)
• If the headache or any other symtom involving the cyst development, decision for extensive surgery must be made on the basis of histopathologic analysis. (11)
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REFERENCEREFERENCE
4. Voelker JL, Campbell RL, Muller J. Clinical, radiographic, and pathological features of symptomatic Rathke's cleft cysts. J Neurosurg 1991;74:535-544
5. Keyaki A, Hirano A, Llena JF. Asymptomatic and symptomatic Rathke's cleft cysts. Histological study of 45 cases. Neurol Med Chir (Tokyo)1989;29:88-93
6. El-Mahdy W, Powell M. Transsphenoidal management of 28 symptomatic Rathke's cleft cysts, with special reference to visual and hormonal recovery. Neurosurgery 1998;42:7-17
10. Osborn W Diagnostic Imaging 2000;:875-877; 892-895 11 Woo Mok Byun, Oh Lyong Kim, and Dong sug Kim MR Imaging Findings of
Rathke's Cleft Cysts: Significance of Intracystic Nodules AJNR Am J Neuroradiol 2000 21: 485-488
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THANKS FOR YOUR THANKS FOR YOUR ATTENTION!ATTENTION!