case presentation of midgut valvulus and cranipharyngimas

radiology

1. CASE PRESENTATION MODERATORS- DR. M. H BHUYAN ( PROF AND HOD ) DR. P. BORAH DR. D.J BORPATRAGOHAIN DR. L. NATH DR. P.N TAYE DR. S. S BISWAS PRESENTED BY- DR. VIPIN KUMAR

2. CLINICAL HISTORY A 9 year old male patient came to our department with complaints of intermittent abdominal pain along with vomiting for last 5 years.

3. CLINICAL EXAMINATION GENERAL EXAMINATION: NAD CVS : NAD CNS : NAD Respiratory system: NAD P/A : Mild tenderness noted in the epigastric region

4. IMAGING Ba Meal Follow T hrough

5. IMAGING USG

6. DIAGNOSIS Based on history, Xray USG and CT findings the case was diagnosed as midgut malrotation leading to midgut volvulus.

7. DISCUSSION Intestinal malrotation  is a congenital anomaly that results from an abnormal rotation of the gut as it returns to the abdominal cavity during embryogenesis. It predisposes to   midgut volvulus and  internal hernias. Malrotation is accompanied by abnormal bowel fixation by mesenteric bands or absence of fixation of portions of the bowel , leading to increased risk of bowel obstruction, acute or chronic volvulus and bowel necrosis.

8. Pathology During normal embryogenesis, the bowel which is in the form of U shaped loop, herniates into the base of the umbilical cord and rapidly elongates. Between  6th to 10th week  the U loop undergoes  3 rotations  in a step wise manner.  First  it rotates by 90° in the anticlockwise direction along the axis of the superior mesenteric artery. At the end of this first rotation the upper limb of the U, or the future ileum lies on the right and the lower limb of U or the future colon lies on the left. At the end of 10th week, the midgut retracts back into the abdominal cavity and undergoes an additional 180 degrees anticlockwise rotation. The net rotation of the entire midgut is 270° anticlockwise (90° + 90° +90° ). This brings the cecum (developed from the lower limb of the U) to the right side and the duodenojejunal junction goes to the left of the midline.

9. There is fixation of the duodenum to the retroperitoneum with the ligament of Treitz located in the left upper quadrant of abdomen. There is fixation of the right colon with the cecum in the right lower quadrant and the left colon from the splenic flexure to the left lower quadrant. The mesentery will then arise in an oblique path from the ligament of Treitz to the terminal ileum, in a straight line from the left upper quadrant to the right lower quadrant. This gives the mesentery a broad base which cannot undergo volvulus.

10. In malrotation, this does not occur and, as a result, the mesentery acts as a short root, which can act as a pedicle around which volvulus can occur. Midgut volvulus is a complication of malrotation in which clockwise twisting of the bowel around the SMA axis occurs because of the narrowed mesenteric attachment. It leads to small bowel obstruction, occlusion of the superior mesenteric vessels and ischemia. Abnormal peritoneal bands passing from the caecum to cross the duodenum (Ladd’s bands) are often present in malrotated patients and they may contribute towards partial duodenal obstruction.

11. CLINICAL PRESENTATION The classical presentation of malrotation is of bilious vomiting, usually within the 1 st year of life, but symptoms may present at any age. In older children, intermittent obstruction can occur, with chronic or recurrent abdominal pain and vomiting .

12. RADIOLOGICAL FEATURES

13. Fluoroscopy In the setting of volvulus findings include: corkscrew sign tapering or breaking of the bowel in complete obstruction  malrotated bowel configuration

14. The  corkscrew sign  describes the spiral appearance of the distal duodenum and proximal jejunum seen in  midgut volvulus . In patients with  malrotation  and volvulus, the distal duodenum and proximal jejunum do not cross the midline and instead pass in an inferior direction. These loops twist on a shortened  small bowel mesentery , resulting in the characteristic corkscrew appearance.

15. Ultrasound Ultrasound findings include  Clockwise “whirlpool ” sign of twisted mesenteric veins around the superior mesenteric artery inverted SMA/SMV relationship Vigorous peristalsis of the dilated duodenum proximal to the obstruction thickened wall of small bowel distal to the obstruction dilated fluid-filled loops of small bowel free intra-abdominal fluid

16. CT Findings include: whirlpool sign of twisted mesentery malrotated bowel configuration inverted SMA/SMV relationship bowel obstruction free fluid/free gas in advanced cases

17. Treatment Urgent surgical repair ( Ladd procedure ) is required to prevent ischemia or to resect infarcted bowel loops.

18. Differential diagnosis Intussusception Other causes of small bowel obstruction Pyloric stenosis Duodenal atresia

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20. CLINICAL HISTORY A 7 year old female patient presented to our department with complains of Headache for past 2 months . Decreased vision for past 1 month which has aggravated more significantly in last 15 days.

21. ON PHYSICAL EXAMINATION Respiratory system- NAD. CVS- NAD Abdomen- NAD CNS- On cranial nerve examination, the patient had decreased perception to light P upillary reaction was absent bilaterally

22. IMAGING FINDINGS CT

23. MRI

24. DIAGNOSIS Based on CT and MRI findings the case was diagnosed as craniopharyngioma .

25. DISCUSSION Craniopharyngioma is a benign(WHO grade I), often partly cystic sellar / suprasellar mass that probably arises from epithelial remnants of Rathke pouch. There are two pathological types of craniopharyngiomas and they differ in appearance, epidemiology and prognosis Adamantinomatous (90%)- multilobulated , partially solid but mostly cystic suprasellar mass. Papillary (10%)- discrete encapsulated mass that does not adhere to adjacent brain.

26. Epidemiology Craniopharyngioma accounts for 6-10% of all pediatric brain tumors and slightly more than half of suprasellar neoplasms. It has a s imilar incidence in males and females Adamantinomatous CPs have a bimodal age distribution with a large peak between 5 and 15 years and a second smaller peak at 45- 60 years. Papillary CPs almost always occur in adults with a peak incidence between 40 and 55 years.

27. Clinical features Patients most commonly present with visual disturbances, either with or without accompanying headache. Large tumors compress the infundibular stalk ("stalk effect") resulting in abnormal pituitary function (often with mild elevation of prolactin). Endocrine deficiencies including growth failure, delayed puberty, and diabetes insipidus are common.

28. Pathology Craniopharyngiomas are primarily suprasellar tumors (75%). A small intrasellar component is present in 20-25% of cases. Purely intrasellar location is quite uncommon (<5%) They range in size from a few millimeters to several centimeters. Larger tumours can extend in all directions, frequently distorting the optic chiasma , or compressing the midbrain with resulting obstructive hydrocephalus. Rare/ectopic locations include: nasopharynx , posterior fossa, extension down the cervical spine.

29. IMAGING Imaging is critical in evaluating craniopharyngiomas , specifically with regard to tumor location and adjacent structure involvement. While MRI is generally accepted as the best imaging, CT may be additive.

30. CT Adamantinomatous Craniopharyngiomas follow a "rule of ninety," i.e 90% are mixed cystic/solid, 90% are calcified, and 90% enhance. Secondary skull base changes such as enlargement of the sella turcica or erosion of the dorsum sellae can be seen Papillary CPs rarely calcify. They are mostly solid. When they contain intratumoral cysts, the cysts are usually smaller and less complex appearing than those seen with adamantinomatous CPs.

31. MRI The MRI appearance may vary depending on the cystic component. Multiple cysts are common, and intracystic fluid within each cyst varies from hypo- to hyperintense compared with brain on T1WI. Hyperintensity , when present, results from the high protein content of the cyst fluid.  They are variably hyperintense on T2WI and FLAIR. Hyperintensity extending along the optic tracts is common and usually represents edema, not tumor invasion . The cyst walls and solid nodules typically enhance following contrast administration .

32. On Magnetic resonance spectroscopy, craniopharyngiomas demonstrate characteristic lactate or lipid peaks, with only small amounts of other metabolites .

33. Adamantinomatous craniopharyngioma

34. Papillary craniopharyngioma

35. TREATMENT & PROGNOSIS Gross total resection is the best treatment option.

36. DIFFERENTIAL DIAGNOSIS Rathke cleft cyst Pituitary macroadenoma

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