1. NATIONAL HOSPITALNATIONAL HOSPITAL
OF PEDIATRICSOF PEDIATRICS

2. AN ATYPICAL RATHKES CLEFT CYSTAN ATYPICAL RATHKES CLEFT CYST
ANDAND
DIFFERENTIAL DIAGNOSISDIFFERENTIAL DIAGNOSIS
Reporter: DR. Hong Nhung Le
Imaging Diagnostic Department
NATIONAL HOSPITAL OF PEADIATRICS

3. 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

4. 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.

5. 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)

6. MRI FindingsMRI Findings
(T2W, Axial)(T2W, Axial)
Cystic mass: D=15mm

7. MRI FindingsMRI Findings
(T1W, sagital)(T1W, sagital)
Cystic mass in the Sellar and supprasellar extension

8. MRI FindingsMRI Findings
((FLAIR coronal)FLAIR coronal)
Hypersign compared to CSF
mass

9. MRI FindingsMRI Findings
(T1W Axial-Postcontrast)(T1W Axial-Postcontrast)

10. MRI FindingsMRI Findings
(T1W Axial-Postcontrast)(T1W Axial-Postcontrast)

11. MRI FindingsMRI Findings
(T1W Axial-Postcontrast)(T1W Axial-Postcontrast)

12. MRI RESULTMRI RESULT
Cystic mass in the sella and suprasellar extension:
AN ATYPICAL RATHKES CLEFT CYST?

13. DIFFERENTIAL DIAGNOSISDIFFERENTIAL DIAGNOSIS
• Rathkes cleft cyst
• Epidermoid cyst
• Craniopharygioma (CR)

14. RATHKES
CLEFT CYST
EPIDERMOID
CYST
CRANIOPHARY-
GIOMA
DEMOGRAPHICS At any age
Gender: F>M
At any age Adamatinomatous:
5-15 year
Papilary: above 50
year
Gender: 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

15. 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
DIAGNOSTIC
CHECKLIST
Intracystic nodule FAT on CT Strong
enhancement
DIFFERENTIAL DIAGNOSIS

16. 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

17. TYPICAL RATHKES CLEFT CYSTTYPICAL RATHKES CLEFT CYST
Intracystic nodule

18. INTRACYSTIC NODULEINTRACYSTIC NODULE
(Continuing)(Continuing)
Hypersignal on T1W Hyposignal on T2W

19. ATYPICAL RATHKES CLEFT CYSTATYPICAL RATHKES CLEFT CYST

20. TYPICAL EPIDERMOID CYSTTYPICAL EPIDERMOID CYST
T1 W coronal Precontrast:T1 W coronal Precontrast:
Cystic mass in the suprasella

21. TYPICAL EPIDERMOID CYSTTYPICAL EPIDERMOID CYST
T1 W coronal PostcontrastT1 W coronal Postcontrast
Rim enhanced mass

22. EPIDERMOID CYSTEPIDERMOID CYST
DWI-ADCDWI-ADC
Restriction on DWI

23. TYPICAL CRANIOPHARYGIOMATYPICAL CRANIOPHARYGIOMA
Strong enhancement at capsule and solid structure

24. DISCUSSIONDISCUSSION
• Imaging technique on MRI.
• Embryology of Rathkes pouch and
Rathkes cleft cyst.
• Diagnostic checklist.
• Treatment strategy

25. 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)

26. 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)

27. 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.

28. 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.

29. 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)

30. 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

31. THANKS FOR YOURTHANKS FOR YOUR
ATTENTION!ATTENTION!