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Case Presentation
1) Castlemen's disease
2) B/L retroperitoneal lymphangiectasia
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By,
Dr.R.S.Shah (Professor and Head of the unit-c, Department of surgery, Smt. SCL. Hsp, Saraspur)
Dr.M.B.Patel (Associate Professor, Unit-c, Department of surgery, Smt. SCL Hsp, Saraspur)
Dr.H.N.Vyas (Assistant Professor, Unit-c, Department of surgery, Smt. SCL Hsp, Saraspur)
Dr Kirti Prajapati, Dr.Aman Bhatia, Dr Keyur Surti (Resident, Unit-c, Department of Surgery, Smt SCL Hsp, Saraspur) |
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B/L RETROPERITONEAL LYMPHANGIOMA |
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| A) SHORT CLINICAL HISTORY |
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| A, 19 years old Dilbahar Bechubhai presented with c/o swelling in both inguinal area accompanied with sever pain but no-fever for 1 day with h/o similar attacks of pain and swelling 3-5 times in past. Patient typically gave h/o swelling that appeared on straining and disappear on lying down who on examination showed irreducible swelling in both inguinal region with local tenderness and red colored skin. Patient had tachycardia in order of 110/min, but rest of the examination was normal. Patient was not residing in filarial belt of INDIA |
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| B) INVESTIGATION |
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| Patient underwent all routine investigation before operation as follows, |
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| I) Blood investigations: |
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Hb: 7.5
TC: 4000
DC: 64/34/01/01
Blood group: AB +ve
Prothrombin time: Test-21 Control-16
RBS: 108
Blood urea: 28
S.Cr: 0.9 |
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| II) Radiological investigation: |
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X-ray abdomen (erect): few non-significant air fluid levels seen.
Usg: right sided inguino-scrotal hernia with herniating bowel loops with minimal hydrocele fluid. Testes and epididymis were normal. Left sided moderate hydrocele with hernia with altered echo texture of left testes with herniating bowel loops. |
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| C) TREATMENT: |
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| Patient was prepared for operation keeping strangulated inguinal hernia in mind. Patient was induced under spinal anesthesia which was supplemented later on by general anesthesia. |
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| At first, Patient was explored via inguinal incision on right side. Inguinal canal opened and. cord with its contents dissected out. There was no apparent hernial sac. But there were numerous dilated tortuous lymphatic channels making most of the bulk of the cord, running from DIR to SIR. |
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| At this point decision was made to perform exploratory laparotomy. Exploratory laparotomy was done via midline abdominal incision. Peroperatively multiple dilated tortuous lymphatic channels were found traversing from the DIR to retroperitoneum. On retroperitoneal dissection on both sides, Para aortic and paracaval dilated lymphatics seen. Virtually, whole of the retroperitoneum was studded with dilated lymphatics. Ureter was positively identified and safeguarded. Now, these bunches of dilated lymphatics were ligated as high as possible and also at DIR level and severed on both sides. Right side Bassini's repair done and incision closed. |
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| During postop period patient developed respiratory distress with features of shock which was due to adrenal insufficiency as it was revealed later by patient that he used to take steroid medications for body building,but it was controlled with due medical care. Patient also developed wound infection which was taken care of by regular dressing otherwise the convalescence period was satisfactory. |
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| D) DISCUSSION: |
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| Lymphangiomas are lymphatic analogue of hemangiomas. They are considered congenital lesions and divided into two types. |
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| I) Capillary Lymphangiomas and |
| II) Cavernous Lymphangiomas. |
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| They are thought to represent isolated and sequestered segments of lymphatic systems. Cavernous Lymphangiomas commonly found in neck or axilla but very rarely in retroperitoneum. Simple capillary Lymphangiomas tend to occur subcutaneously in head and neck region as well as in axilla. Rarely, they can also be found in the trunk and internal organs. Localized form of cutaneous lymphangiomas is know as lymphangioma circumscriptum |
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| In 1976, Whimster studied the pathogenesis of the disease and postulated that these cisterns arise from primitive lymph sac that fails to connect with the rest of lymphatic system during embryonic development. In US, they are rare accounting for 4% of all vascular tumours. |
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| Lymphangioma can become evident at any age but greatest incidence occurs at birth or early in life. |
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Cavernous lymphangioma typically occurs during infancy as solitary rubbery nodule with no skin changes located on face, neck, and trunk. Its association with Turner's syndrome and Down's syndrome has been found,
It has tendency to recur. |
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| In the present case patient was young with retroperitoneal lymphangioma which presented with secondary lymphangitis (AEL- acute episodes of lymphangitis). Patient was operated and the mass of lymphangioma was excised which in HPE turned to be cavernous lymphangioma. |
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| E) CONCLUSION: |
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| Lymphangioma is congenital lesion which usually present at early age. It is adequately treated with complete excision of the lesion |
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| F) REFERENCES: |
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Freidberg J: pharyngeal cleft sinuses and cysts & other benign neck lesions. Paedi North Am 989, Dec; 36(6)/45/69
Mandal S: Case of Lymphangioma circumscriptum; Indian J dermatology, 974,34.
Thomson H: cutaneous hemangioma and lymphangioma
Whimster, The pathology of L. circumscriptum, Br J Dermatology, 1976 May; 09,
Chace Gy, King KL, Su CN, Lui WY, Retroperitoneal cystic lymphangioma in ad |
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