Sunday, January 30, 2011

Successful treatment of metastatic hepatic Epithelioid Hemangioendothelioma with Thalidomide: a case report

Hepatic Epithelioid Hemangioendothelioma is a rare malignancy arising from the vascular endothelial cells within the liver. Historically the disease is characterized as bad treatment of choice responds to both chemotherapy and radiation therapy, liver resection or transplant if feasible. For patients with advanced disease reports on long-term therapeutic benefits of Conventional cytotoxic treatment are very limited. Due to the rarity of this malignancy there are no structured therapeutic research, but a small number of cases still respond well to treatment with inhibitors of angiogenesis reported. Thalidomide was originally as an anti emetic but a potent inhibitor of vascular neo Genesis is developed, and promoting the malignant vascular endothelial cells could offer potential in the treatment of hepatic Epithelioid Hemangioendothelioma by blocking.

We describe the case Caucasian British woman, at the age of 53 years of hepatic mass malignant Lymphadenopathy, pulmonary metastases certified as hepatic Epithelioid Hemangioendothelioma on biopsy were presented. After unproductive treatment with interferon our patients on Thalidomide started 400 mg daily. It has successfully manages to therapy for the last seven years was and remained asymptomatic with radiologically stable disease and minimum treatment-related side effects.

There is currently no standard treatment for advanced hepatic Epithelioid Hemangioendothelioma. The role for thalidomide and potentially other inhibitors of vascular neo Genesis helps the treatment of patients with metastatic hepatic Epithelioid Hemangioendothelioma our case.


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Wednesday, January 26, 2011

Cholesteatoma of Concha Bullosa of: a case report

Cholesteatoma is relatively common in the middle ear cavity, but it can rarely be seen in paranasal sinuses and it is a published case of Cholesteatoma within the Concha Bullosa in English literature, to the best of our knowledge.

An 81-year-old Caucasian woman was established, nasal obstruction, headache and diplopia complain. TRANS-nasal was preferred to endoscopic approach for endoscopic and radiological assessment. The diagnosis of Cholesteatoma was founded by histopathological evaluation of mass within the Concha Bullosa.

Although it happens rarely, Cholesteatoma should be considered in the differential diagnosis of slow growing and destructive sinuses masses.


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Sunday, January 23, 2011

Simultaneous chest stroma Sarcoma and breast carcinoma: a case report

Breast cancer is one of the main health problems in the world and affects a large number of women across the globe. This group of neoplasms rarely presented as bilateral disease and, if it happens usually occurs within the we report same histological type a rare case of concurrent bilateral breast cancer with two different types of histology, a carcinoma of the breast and a chest Sarcoma in a 42-year-old woman in accordance with our hospital.

A 42-year-old Caucasian woman admitted to our Institute in August 1999, presented with a node in the left breast 3.0 x 2.5 cm and in the right breast one 1.0 cm suspected the viciousness and clinically negative with a armpit. Invasive mammary Carcinoma (right chest) and Sarcoma (left chest) had uncovered biopsies. Was presented to bilateral modified radical mastectomy. A histological study showed an invasive mammary carcinoma degree lobular Pleomorphic type II invasion of seven of the 19 excised axillary node right chest and the left chest, a sarcoma of the MOM stroma for Immunohistochemistry study for epithelial biomarker negative and positive for Vimentin. Later wall and axillary node on the left was presented you for chemotherapy (six cycles of 75 mg / m2 5-fluorouracil, epirubicin and cyclophosphamide) followed by radiotherapy of the thoracic. Hormone receptors were positive in the right breast tumor and tamoxifen 20 mg was followed by Letrozole, 2.5 mg, daily (five years) required on a daily basis (five years). She introduced the negative trend in the last consultation no characters.

The risk of developing bilateral breast cancer is about 1% every year in a similar histological type, but it is higher in tumors with lobular histology. In this case, the patient, two histologically distinct tumors proof thus presents a rare situation.


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Thursday, January 20, 2011

Twin reversed arterial perfusion (TRAP) sequence relating to VACTERL Association: a case report

Twin room of reverse arterial perfusion (TRAP) sequence is a rare complication of multiple pregnancy caused by defects in the early embryogenesis. The pump twin delivers the Acardiac recipient twin with blood, and although the pump twin is usually structurally normal, congenital anomalies is sometimes reported. We report a unique case twin vice versa arterial perfusion sequence with a prenatal diagnosis of the VACTERL Association in survivors pump twins.

A 24-year-old Caucasian woman on 11 weeks pregnancy with a Monochorionic Monoamniotic twin pregnancy presented. A reverse arterial River was apparently normal recorded on a Doppler twins at the smaller, grossly abnormal twin imaging study from the larger and Diagnostics twin vice versa arterial perfusion sequence was made. Heart activity was twin 16 weeks pregnancy undetectable in the receiver. For more detailed assessment to 18 weeks gestation revealed multiple congenital anomalies of the survivors pump twin in accordance with a diagnosis of VACTERL Association. A live children girl by elective caesarean section at 39 weeks came. She underwent extensive surgery with below normal development in the age of two.

The coexistence of two rare and complex conditions in this unique case raises interesting questions about the role of the early shortcomings in embryogenesis and their subsequent effects on fetal development. In this case also underlines the importance of the prenatal diagnosis of major congenital anomalies of plan treatment to reduce morbidity and aid the survival of affected children.


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Monday, January 17, 2011

Squamous Cell Carcinoma rectum to present in a man: a case report

Primary squamous cell carcinomas of the Colorectum are very rare. Until now, to the best of our knowledge, there are only 114 cases of squamous cell carcinoma in the Colorectum reported literature. Here we report a case of squamous cell carcinoma of the rectum in the ethnic Kashmiri people in northern India.

The case of a 60-year-old and old males (Asian) with a pure squamous cell carcinoma of the rectum is shown here. The patient operated a curative with concomitant chemotherapy. Two years after the initial curative resection of the tumor, he is still alive.

The prognosis for squamous cell carcinoma of the Colorectum is worse than for adenocarcinoma due to delayed diagnosis. Etiopathogenicity of squamous cell carcinoma of the Colorectum is discussed. Surgical resection of the lesion appears the treatment of choice. Chemotherapy, also helps improve the prognosis.


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Friday, January 14, 2011

Benign multicystic peritoneal Mesothelioma: a case report

We report the case of a patient with a benign multicystic peritoneal mesothelioma and describe its appearance on computed tomography scans and ultrasonography, in correlation with gross clinical and pathological findings.


A 72-year-old Caucasian woman presented to our emergency department with acute abdomen signs and symptoms. A clinical examination revealed a painful palpable mass in her left abdomen. Abdominal ultrasonography and computed tomography demonstrated the presence of a large cystic mass in her left upper abdomen, adjacent to her left hemidiaphragm. The lower border of the mass extended to the upper margin of her pelvis. A complete resection of the lesion was performed. Pathological analysis showed a benign multicystic peritoneal mesothelioma.


Benign multicystic peritoneal mesothelioma is a rare lesion with a non-specific appearance on imaging. Its diagnosis always requires pathological analysis.


Benign multicystic peritoneal mesothelioma is an uncommon lesion arising from the peritoneal mesothelium. It is often diffuse and shows a marked predilection for the surfaces of the pelvic viscera [1-8]. In our case report, the lesion was solitary and situated in the left abdomen. This disease is a rare medical entity and there are challenges in determining its origin, pathogenesis, diagnosis and therapy.


A 72-year-old Caucasian woman was admitted to our surgical department having experienced diffuse abdominal pain and discomfort, nausea and vomiting for the previous two days. Her medical history included diabetes mellitus and arterial hypertension, for which she was on medication. She had no relevant family history and did not smoke or drink alcohol.


On physical examination, she showed signs of acute abdomen and a palpable painful mass in her left abdomen was noted. She was tachycardic and laboratory tests showed a white blood cell count of 13,000 cells per cubic millimeter. Her chest and abdominal radiographs did not reveal any abnormalities.


An ultrasonography (US) examination demonstrated a complex cystic mass with internal septa, without increased vascularity. The source organ could not be identified (Figure 1).


Computed tomography (CT) examination demonstrated a large 18.7 × 13.2 × 22cm, intra-peritoneal hypodense mass in her upper left abdomen, lying between the great curvature of the stomach, the spleen and the tail of the pancreas, and extending caudally to the upper margin of the pelvis, causing pressure over the adjacent organs. The mass demonstrated no internal septa and no enhancement after the intravenous administration of a contrast medium. No abnormal lymphadenopathy was present (Figure 2, Figure 3 and Figure 4).


She underwent an urgent operation and the multicystic mass was found to occupy her entire left abdomen, adherent to the spleen. A complete resection of the lesion and splenectomy were performed. She had an uneventful post-operative recovery and a post-splenectomy prophylaxis was used.


Gross examination of the specimen showed a large gelatinous cystic mass containing multiple smaller cystic spaces. Her immunohistochemical stains were positive for calretinin and cytokeratins, confirming the mesothelial origin of the mass. The final diagnosis was benign multicystic peritoneal mesothelioma. (Figures 5 and 6).


 (Hematoxylin and eosin stain, original magnification × 400).


(Hematoxylin and eosin stain, original magnification × 100).


Six months post-operatively, she had experienced no recurrence and was free of symptoms.


Mesotheliomas are mesenchymal neoplasms originating from the serous lining of the pleural, pericardial or peritoneal space. Multicystic peritoneal mesothelioma involves the peritoneum or extra-peritoneal space, omentum, pelvic or abdominal viscera. It most commonly arises from the pelvic surfaces of the peritoneum and has benign or indolent biologic behavior. Multicystic mesothelioma of the peritoneum was first described in 1979 by Mennemeyer and Smith and since then approximately 130 cases have been described in the literature. It is an intermediate-grade tumor, among the benign adenomatoid tumors of the peritoneum and the more common malignant asbestos-related peritoneal mesothelioma. It is not related to prior asbestos exposure and may recur locally [1-11].


On histological examination, the mesothelial cells lining the cysts may vary from flattened and endothelial-like to cuboidal. The thin-walled cysts may be filled with eosinophilic, serous fluid. Inflammatory cells and fibrous elements may be found within the stroma between the cysts. Foci of mesothelial hyperplasia may also be present [2].


It is usually large at the time of diagnosis (mean diameter, 13 cm). Multifocality, free floating cysts and unilocular cysts have been reported [2].


It commonly occurs in young to middle-aged women (mean age, 37 years). The presenting symptoms are chronic or intermittent lower abdominal or pelvic pain, tenderness, or distension with an abdominal or pelvic mass and, rarely, dyspareunia, constipation and urinary hesitancy and/or frequency. Women with this lesion often have a history of prior pelvic surgery, endometriosis or pelvic inflammatory disease [1-3,5,7-10].


The pathogenesis of benign multicystic peritoneal mesothelioma is unclear and there is some controversy regarding its neoplastic and reactive nature [2,6]. The fact that the great majority of patients are women of reproductive age suggests that a key role is played by female sex hormones in its pathogenesis [5].


US demonstrates multiseptated anechoic cysts. The fluid within the cysts is generally anechoic, but the cysts may contain echoes from debris or hemorrhage. The number and complexity of septations, as well as the size of the cysts, are quite variable. Calcification has not been described in multicystic mesothelioma. CT provides more information about the location and extent of the mass, and demonstrates a well-defined, low-attenuation mass with non-calcified septa. The septa become enhanced following intravenous administration of a contrast material. Magnetic resonance imaging (MRI) provides additional coronal and sagittal planes. The watery serous content has low signal intensity on T1-weighted images and intermediate-to-high signal intensity on T2-weighted images. Septal enhancement has been reported [1,2].


The differential diagnosis includes lymphangioma, other mesenteric and/or omental cysts, cystic teratoma, pseudomyxoma peritonei, cystic smooth muscle tumors, visceral cysts, cystic mucinous neoplasms of the pancreas, non-pancreatic pseudocysts, endometriosis, cystic adenomatoid tumor and cystic mesonephric duct remnants. When multicystic mesothelioma is located solely in the pelvis in women, tubo-ovarian abscess, hydrosalpinx, cystic ovarian neoplasms (ovarian cystadenoma, cystadenocarcinoma) and cystic forms of endosalpingiosis should be considered in the differential diagnosis. Lymphangiomas often occur in younger patients and can be identified if they contain predominantly chylous fluid and microscopically lymphoid aggregates and smooth muscle in their walls. Mesenteric cysts are generally unilocular and contain serous secretions, with no discernible wall or internal septa. Teratomas contain fat and calcification. Pseudomyxoma peritonei can be distinguished when there is co-existing omental caking, soft-tissue peritoneal nodules and scalloping of the serosal margins of the liver or spleen. The cystic component in cystic adenomatoid tumor is usually accompanied by a recognizable solid component. Malignant neoplasms are suggested by ancillary signs such as intramural nodules, ascites, necrosis or peritoneal carcinomatosis, and the source organ can usually be identified [1,5-7,9,10].


Multicystic mesothelioma is seldom diagnosed at pre-operative imaging because it is exceedingly rare; the diagnosis requires histological evaluation.


The treatment of choice is complete surgical excision. Complete removal of the cystic lesion, if possible, is the best treatment and the only hope in avoiding local recurrence. Aggressive surgical approaches including cytoreductive surgery with peritonectomy are recommended [5,6]. Hormonal therapy with anti-estrogens and gonadotrophin-releasing analogues, sclerotherapy with tetracycline, hyperthermic peritoneal perfusion with cisplatin and peritonectomy with intra-peritoneal chemotherapy have also been attempted in individual cases with varied degrees of success. Adjuvant chemotherapy and radiotherapy are not indicated as this tumor has a prevailing benign character [5,6].


About 50 percent of the patients experience a recurrence one to 27 years after the initial diagnosis and malignant transformation has very rarely been reported [1-3]. Thus, routine follow-up imaging is required post-operatively in all patients [4].


The prognosis is excellent and the only death that has ever been reported in the literature occurred in the case of a patient who refused to undergo resection 12 years after diagnosis [5].


Benign multicystic peritoneal mesothelioma is a very rare benign cystic tumor. This lesion has a non-specific appearance on imaging which does not permit differential diagnosis from other cystic lesions and always requires histological evaluation. It has a high recurrence rate after surgical resection but malignant transformation has very rarely been reported. A systematic follow-up of these patients is required and further resection or other therapy may be indicated.


(CT): Computed tomography; (MRI): magnetic resonance imaging; (US): ultrasonography.


Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


The authors declare that they have no competing interests.


XP performed the chart review and prepared the manuscript. EA, AE and CT carried out the operation. LP was the pathologist who examined the specimen. AP, NS and TC participated in the preparation of the manuscript. All authors read and approved the final manuscript.


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Tuesday, January 11, 2011

Highly dosed steroid treatment for idiopathic fiber optic Perineuritis: a case series

It has been reported that the prognosis of optic perineuritis may be poor when initiation of treatment is delayed. Here we report the successful treatment of three patients with idiopathic optic perineuritis, including two in whom initiation of therapy was delayed.


Three Japanese patients (two women aged 73 and 66 years, and one man aged 27 years) presented with loss of vision (for five months, several months, and two months respectively) and pain on eye movement in the third case only, and were diagnosed as having idiopathic optic perineuritis. Fat-suppressed T2-weighted magnetic resonance images showed high signal intensity areas around the affected optic nerves, suggesting the presence of optic perineuritis. Two patients received steroid pulse therapy and the third was given high-dose steroid therapy. The visual acuity improved in all three cases.


High-dose steroid therapy may be effective for idiopathic perineuritis in patients without optic nerve atrophy, even if initial treatment (including moderate-dose steroids) has failed.


Idiopathic optic perineuritis has been reported as a type of orbital inflammatory pseudotumor [1-3]. Currently, the diagnosis of optic perineuritis is most commonly based on magnetic resonance image (MRI) findings along with the clinical characteristics. Although some reported cases have been diagnosed by pathologic examinations, the distinction between optic neuritis and optic perineuritis is generally radiographic [4]. The characteristic differences between idiopathic optic perineuritis and idiopathic optic neuritis are as follows [5]: The age distribution of the former is wide and it particularly affects elderly patients, and a paracentral scotoma or an arcuate defect are frequent findings. The onset is slow (usually over several weeks), and recovery is often poor in patients with optic perineuritis when treatment is delayed. The response to corticosteroids is often dramatic, although recurrence is common with tapering of therapy. Here, we report the successful treatment of three patients with idiopathic optic perineuritis who received high-dose steroid therapy.


A 73-year-old Japanese woman had noticed a decrease in vision in her right eye for five months. On examination, the visual acuity on her right side was 20/60. She was treated with prednisolone at doses of up to 30 mg/day for eyelid swelling. After five months, however, her acuity was only 20/400 on her right side. A right relative afferent pupillary defect was present. Goldmann perimetry showed an arcuate scotoma of her right eye. Laboratory tests revealed a CRP of 0.9 mg/dl (normal range: 0.0-0.3) and an ESR of 38 mm/hour, while ACE, FTA, and ANCA were all within the normal range. The results of hematology tests, renal and liver function tests, urine analysis, chest radiography, and computed tomography were all within normal limits. Fat-suppressed T2-weighted MR images revealed a high signal intensity area around her right optic nerve and moderate swelling of her right extraocular muscles, suggesting inflammation of her optic nerve sheaths and extraocular muscles (Figure 1). Steroid pulse therapy was initiated. After four days, the vision of her right eye improved to 20/80. One month after steroid pulse therapy, fat-suppressed T1-weighted MR images showed persistence of the high signal intensity area around her right optic nerve and moderate swelling of her right extraocular muscles (Figure 1). Subsequently, the steroid dose was gradually tapered. There has been no recurrence of symptoms after an observation period of 22 months.


Coronal image (a) and axial image (b) from a 73-year-old Japanese woman with idiopathic optic perineuritis. There is a high signal intensity area around the right optic nerve and moderate swelling of the right extraocular muscles, suggesting inflammation around the optic nerve sheath and the extraocular muscles. Fat-suppressed T1-weighted magnetic resonance image obtained one month after steroid pulse therapy. This axial image (c) shows persistence of the high signal intensity area around the right optic nerve and moderate swelling of the right extraocular muscles. The extraocular muscles showed persistent moderate swelling (not visible on this image).


A 66-year-old Japanese woman presented to our hospital with decreased vision in her right eye that had persisted for several months. Vision was 20/300 on her right side and a relative afferent pupillary defect was detected, although there were no abnormal intraocular findings. Goldmann perimetry showed an arcuate scotoma of her right eye. Laboratory tests revealed a CRP of 0.3 mg/dl, while the ESR, ACE, FTA, and ANCA were all within the normal range. The results of hematology tests, renal and liver function tests, urine analysis, chest radiography, and computed tomography were all within normal limits. MR images showed high-intensity areas in her right optic nerve sheath on fat-suppressed T2-weighted images and fat-suppressed T1-weighted images (Figure 2); these findings suggesting inflammation of her optic nerve sheath. Treatment with prednisolone (40 mg/day) was initiated. Subsequently, the steroid dose was gradually tapered. After two months, there was a recurrence of symptoms in her right eye, so prednisolone (40 mg/day) was started again. Subsequently, the steroid dose was tapered more gradually and her vision was 20/20 on the right side after 11 months. Recurrence of symptoms has not been detected after follow-up for 19 months.


Coronal image (a) and axial image (b) from a 66-year-old Japanese woman show high-intensity areas in the right optic nerve sheath. Fat-suppressed T1-weighted post-contrast coronal image (c) shows high-intensity areas in the right optic nerve sheath. The optic nerve sheath is enlarged and enhanced on both sides.


A 27-year-old Japanese man came to our hospital with blurred vision in the upper field of his left eye and ocular pain/headache associated with eye movement that had persisted for two months. His visual acuity was 20/300 on his left side. There was swelling and erythema of his left optic disc. Goldmann perimetry showed enlargement of Mariotte's blind spot and a paracentral scotoma of his left eye. Laboratory tests revealed a CRP of 0.5 mg/dl, while the ESR, ACE, FTA, and ANCA were all within the normal range. The results of hematology tests, renal and liver function tests, urine analysis, chest radiography, and computed tomography were all within normal limits. MR images revealed no abnormalities in his brain. On fat-suppressed T2-weighted images, the area around his left optic nerve showed a high intensity (Figure 3). Steroid pulse therapy was initiated. After seven days, his vision improved to 20/15 on the left. Subsequently, the dose of steroids was gradually reduced. No recurrence has been noted after 15 months.


T2-weighted coronal (a) and axial (b) images demonstrate strong hyperintensity around the left optic nerve of a 27-year-old Japanese man.


The prognosis of optic perineuritis has been reported to be poor when initiation of treatment is delayed [5]. However, our first two cases both responded well to steroid therapy and achieved a good visual prognosis, despite the interval between the onset of symptoms and initiation of treatment being longer than six months. Concerning the steroid dose, recurrence was observed in Case 2 after treatment with prednisolone at a daily dose of 40 mg in the early stage of her illness, and Case 1 showed recurrence after receiving prednisolone at a dose of 30 mg/day at her previous hospital. After we performed steroid pulse therapy at our hospital for Cases 1 and 3, there was no recurrence in Case 3, and no subsequent recurrence in Case 1. Purvin et al. reported recurrence of optic perineuritis in 4 out of 14 patients treated with oral steroids at doses of 60-80 mg/day [5].


Perimetry was performed up to isopter I-1e in all of our patients. The innermost isopter of the central visual field that showed a response was isopter I-4e in Case 1, isopter I-2e in Case 2, and isopter I-1e in Case 3. Thus, Cases 1 and 2 did not respond to isopter I-1e, suggesting the presence of central depression. Perimetry of the peripheral visual fields including the paracentral field revealed arcuate constriction on the downside of isopter V-4e in Case 1, who showed a generalized decrease of sensitivity. In Case 2, depression was seen on the upside of Mariotte's blind spot in isopter I-4e, indicating a paracentral scotoma. In Case 3, scotomata were observed at three sites on the upside of Mariotte's blind spot in isopter III-4e. These results suggest that reduced vision was at least partly ascribable to a decrease of central visual field sensitivity in Cases 1 and 2, whereas vision was reduced despite the lack of a central scotoma or reduction of central field sensitivity in Case 3. Therefore, the reduced visual acuity was related to central or generalized depression of vision due to optic perineuritis in Cases 1 and 2. In Case 3, vision may have been reduced because scotomata involved the fixation point.


This case series had the following limitation. The best diagnostic sequence for optic perineuritis is post-contrast fat-suppressed T1-weighted images. On other images, the area of hyperintensity around the optic nerve could represent an increase of cerebrospinal fluid that would occur if there was optic atrophy. However, we did not obtain fat-suppressed T1-weighted images in all three cases, although such images are required for the definite diagnosis of optic perineuritis.


Two patients with optic perineuritis who underwent steroid pulse therapy showed no recurrence, as did one patient receiving high-dose prednisolone. Our results suggest that steroid pulse therapy or high-dose prednisolone may be effective for idiopathic optic perineuritis in patients without optic nerve atrophy, even if initial treatment (including moderate-dose steroids) has failed.


ACE: angiotensin-converting enzyme; ANCA: anti-neutrophilic cytoplasmic antibodies; CRP: C-reactive protein; ESR: erythrocyte sedimentation rate; FTA: fluorescent treponemal-antibody; MR: magnetic resonance;


Written informed consent was obtained from the patients for publication of this case series and any accompanying images. Copies of the written consents are available for review by the Editor-in-Chief of this journal.


The authors declare that they have no competing interests.


MT, HN, TM and HF analyzed and interpreted the patient data. MT was a major contributor in writing the manuscript. All authors read and approved the final manuscript.


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Saturday, January 8, 2011

A non-recurrent laryngeal of nerve inferior in a man Thyroidectomy subject: a case report

A non-recurrent variant of the inferior laryngeal nerve has been seldom reported. These reports are mostly based on cadaveric dissection studies or large chart review studies in which the emphasis is placed on the determination of the frequency of the variation, and not on the clinical appearance of this variant. We graphically describe the intraoperative identification of a non-recurrent inferior laryngeal nerve.


A 44-year old Caucasian man was referred to the Head and Neck Surgery Outpatient Clinic with the diagnosis of a nodular mass in his left thyroid lobe that had been growing for one year. A fine needle aspiration puncture was compatible with thyroid papillary cancer. It was decided that the patient should undergo total thyroidectomy. During surgery, a non-recurrent right inferior laryngeal nerve was noted. This nerve emanated from the right vagus nerve, entering the larynx 3 cm after its origin. The nerve did not show a recurrent course. The nerve on the left side had a normal configuration. The surgery and post-operative period were uneventful, and the patient had no change in his voice.


This paper allows those interested to become acquainted with the normal intraoperative appearance of a non-recurrent inferior laryngeal nerve. This will undoubtedly be of significance for all of those performing invasive diagnostic and surgical procedures in the neck and upper thoracic regions, in order to minimize the risk of iatrogenic injury to this nerve. This is of extreme importance, since a unilateral lesion of this nerve may result in permanent hoarseness, and a bilateral lesion may lead to aphonia and life-threatening dyspnea.


The inferior laryngeal nerve (ILN) is traditionally named recurrent due to the fact that, branching off the vagus nerve, it usually describes a loop as it turns upwards, passing under the subclavian artery on the right and under the ligamentum arteriosum on the left [1].


The ILN provides innervation to all larynx intrinsic muscles, except for the cricothyroid muscle. From a sensorial point of view, it innervates the mucosal surface of the larynx below the vocal cords [2]. Injury to this nerve may thus result in paralysis of the vocal cord on the same side, leading to permanent hoarseness. If the lesion is bilateral, aphonia and life-threatening dyspnea may ensue as a result of medial placement of the paralytic vocal cords, which can obstruct the glottis [3].


The relatively long course of the inferior laryngeal nerves place them at risk of iatrogenic injury in numerous procedures involving the cervical and upper thoracic regions [1]. Among these, surgical procedures in the cervical region, namely thyroidectomies, are particularly common. In this last setting, for example, permanent injury to the recurrent laryngeal nerve (ILN) is reported in 0.25 to 2.6% of cases, with rates >8% in case of reoperation [4]. It has been demonstrated that dissection and visualization of the ILN during such procedures significantly reduces the risk of lesion to this nerve [5]. To accomplish this, it is instrumental to have a sound knowledge of the normal and variant forms of the ILN [6].


A 44-year-old Caucasian man was referred to the Head and Neck Surgery Outpatient Clinic with the diagnosis of a nodular mass in his left thyroid lobe that had been growing since the previous year. His previous medical history was unremarkable. No other enlarged masses or lymph nodes were palpated in the physical examination or detected in the imagiological study of the neck with ultrasound and computed tomography (CT) scan. A fine needle aspiration puncture was performed and the result was compatible with thyroid papillary cancer. In the Group Decision Clinic it was decided that the patient should undergo total thyroidectomy.


During surgery, a non-recurrent right inferior laryngeal nerve was noted (Figure 1). This nerve emanated from the right vagus nerve almost at a straight angle, entering the larynx 3 cm after its origin. The nerve did not show a recurrent course. The nerve on the left side had a normal configuration coming upwards in the tracheo-esophageal groove, in a recurrent fashion, presumably from the left vagus nerve. The surgery and post-operative period was uneventful, and our patient had no change in his voice.


 The right Inferior Laryngeal Nerve (ILN) is seen originating perpendicularly from the Vagus Nerve (VN) and entering the Larynx between the Crycoid Cartilage (CC) and the Thyroid Cartilage (TC), just below the Crico-Thyroid Muscle (CT). The Thyroid Gland (TG) is being retracted medially to increase exposure of ILN and allowing visualization of the Trachea (TR). The right ILN does not have any recurrent path.


The reported incidence of the non-recurrent ILN is widely variable. In some series, the proportion of this variation is null [3-7] whereas in others it is as high as 3.9% [8-10]. In the largest series reported, including 6637 observations of the ILN during neck surgery, the frequency of the non-recurrent ILN was 0.54% (17 cases in 3098) on the right and 0.07% on the left (2 cases in 2846), corresponding to a global prevalence of 0.32% [11].


Despite the discrepancies in the relative proportion of this variant of the ILN, most authors agree that this variant is most common on the right side. The embryological basis for this finding seems to be a vascular disorder known as arteria lusoria in which the fourth right aortic arch is abnormally absorbed [12]. Consequently, this vessel fails to drag the right recurrent laryngeal nerve (ILN) caudally when the heart descends, and the neck elongates during embryonic development [9,13,14]. This anomaly generally leads to a right subclavian artery that originates as a branch of a normal aortic arch and passes upward to the right behind the esophagus. The incidence of this vascular malformation is reportedly as high as 0.5 to 2% of the general population [15]. Even though it is usually asymptomatic, nearly 5% of these patients experience dysphagia (dysphagia lusoria) or symptoms related to artery tortuosity, and premature atherosclerosis. Rarely, it is associated with aneurism formation [15].


On the left side, a non-recurrent ILN has only been observed in cases of dextrocardia [13].


Theoretically, the pre-operative diagnosis of a non-recurrent ILN could be attempted with imaging studies, namely CT scan or magnetic resonance imaging (MRI), to visualize the arteria lusoria [14]. However, although the presence of anatomical variants of the ILN have been associated with a higher risk of iatrogenic injury during surgery in the head and upper thorax region [4], it has not yet been shown that the systematic use of pre-operative imaging studies can minimize this risk.


Invasive procedures in the head and neck region that may compromise the ILN are part of everyday clinical and surgical practice. A sound knowledge of the normal morphology and most frequent variants of the ILN, including its non-recurrent variant, can help doctors to minimize the risk of iatrogenic lesion to this nerve.


Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal


The authors declare that they have no competing interests.


All authors have read and approved the final manuscript. DC played a major role in writing the manuscript and analyzed the patient's data. AP aided in the editing of the manuscript and analyzed the patient's data. DS participated in the writing and editing of the manuscript and analyzed the patient's data. JRS aided in the editing of the manuscript and analyzed the patient's data.


View the original article here

Wednesday, January 5, 2011

Benign cystic mesothelioma of the plant presentation into a woman: a case report

Benign cystic mesothelioma or peritoneal inclusion cysts are rare benign abdominal tumors usually occurring in females of reproductive age. These cysts present as abdominopelvic pain or masses but are often found on imaging or incidentally at surgery. They are commonly associated with pelvic inflammatory disease, endometriosis, or ovarian cysts. We report what is, to the best of our knowledge, the first case of a benign cystic mesothelioma complicating a presentation of acute appendicitis.


A 19-year-old Irish Caucasian woman presented with abdominal pain. Imaging suggested appendicitis with abscess formation. She was treated with antibiotics and scheduled for interval appendicectomy. At laparoscopy, an unusual cystic mass was found arising from the appendix. Histology revealed benign cystic mesothelioma.


We report what is, to the best of our knowledge, the first case of a benign cystic mesothelioma arising from the appendix and complicating a presentation of acute appendicitis. This is a benign pathology, but recurrences are not uncommon. Benign cystic mesothelioma should be included in the differential when investigating pelvic masses or abscesses associated with either appendicitis or pelvic inflammatory disease in women.


Benign cystic mesothelioma (BCM) or peritoneal inclusion cysts are rare abdominal tumors usually occurring in women of reproductive age. These cysts present as abdominal or pelvic pain or masses but are often found on imaging or incidentally at surgery. There have been many cases described associated with pelvic inflammatory disease, endometriosis, or ovarian cysts. We describe the first case of a benign cystic mesothelioma arising from the appendix and complicating a presentation of acute appendicitis.


A 19-year-old Irish Caucasian woman presented to the hospital with a three-day history of abdominal pain and fever. The pain was gradual in onset and associated with nausea and one episode of vomiting. She had no urinary symptoms, and her last menstrual period had finished the previous day. She had no surgical history, and her medical history was significant only for viral meningitis two years previously. She denied any history of sexually transmitted disease or recent urinary tract infection. She was not taking regular medications and had no allergies. On examination, her vital signs were normal except for mild pyrexia of 37.4 °C. Examination of the abdomen revealed a tender mass in the right iliac fossa.


Laboratory investigations included a white cell count of 10,500 cells/mm, hemoglobin of 13.3 g/dl, and platelets of 212,000/mm. Urea and electrolytes were within normal ranges. Urine analysis was negative for leucocytes and urinary ßHCG was negative.


A computed tomography (CT) scan of the abdomen and pelvis was requested and showed a 10.4 × 4.5 × 3.8 cm loculated cystic mass in the right pelvis that appeared to contain the tip of the appendix (Figure 1). The patient remained febrile. Clinically, we made a working diagnosis of an appendix mass but considered a tubo-ovarian abscess as a differential. The patient was treated with intravenous antibiotics, and radiological drainage of the abscess was arranged. An ultrasound-guided drain was placed in the largest locule via the right iliac fossa. Unusually, 30 ml of serous fluid but no pus was aspirated. The drain was removed after three days with no further output. Drained fluid was sent for culture, and peripheral blood cultures showed no growth after 72 hours of incubation. After five days, intravenous antibiotics the patient was clinically well. She was discharged and readmitted two weeks later for an interval diagnostic laparoscopy, as we were now suspicious of a non-infective pathology based on the drain output. The patient consented to an appendicectomy if no other pathology was found.


At laparoscopy, a multiloculated, thin-walled and translucent cystic mass was seen in the right iliac fossa (Figure 2). Adherent to the cystic mass was a spherical, smooth-walled cyst in continuity with the tip of the appendix. The rest of the appendix, caecum, and large and small bowel appeared grossly normal. Both ovaries and the uterus were visualized and found to be normal. The diagnosis was not clear at this point, but our differential included a mucinous cystadenoma or adenocarcinoma (pseudomyxoma peritonei). The lesion appeared very friable, and we were concerned we would rupture it and contaminate the pelvis with the cyst fluid. We made a decision to convert to an open procedure using a Lanz incision to safely perform an appendicectomy and remove the cystic mass. The incision incorporated the previous drain site.


Operative photograph showing thin-walled cystic mass in the right iliac fossa above the appendix.


Macroscopy showed a 12-cm appendix with an attached 4 × 4 × 3 cm smooth cyst containing clear fluid. Numerous smaller translucent cysts up to 0.7 cm in diameter were loosely attached to and easily separated from the larger cyst (Figure 3). We concluded that the radiological drain had entered one of these cysts. Histological analysis revealed cysts lined with flattened mesothelial cells, and the walls were composed of loose connective tissue with occasional chronic inflammatory cells (Figure 4). These findings were consistent with a histological diagnosis of a multiloculated benign cystic mesothelioma. The appendix showed resolving appendicitis with perforation at the tip. The patient was discharged well on the second postoperative day and was also well at six-week and three-month follow-up.


 Immediately below the 15-cm ruler in the photograph. Membranes of the remainder of the multiloculated cyst after removal from the appendix are seen lying toward the bottom of the photograph. The cyst had ruptured in transit from the operating table to the specimen photography table in the operating room.


 Microscopy showing cysts lined with flattened mesothelial cell and the walls composed of loose connective tissue with occasional chronic inflammatory cells.


Benign cystic mesothelioma or peritoneal inclusion cysts are rare but well-described benign tumors of unknown etiology. First described by Plaut in 1928 (1), they are cystic mesothelial proliferations. They are thought to be due to an inflammatory reaction. They usually occur in the peritoneal cavity in the abdomen or pelvis, and the most common predisposing factors in the clinical history are previous surgery, pelvic inflammatory disease, or endometriosis. These conditions are believed to interfere with peritoneal reabsorption. This would tend to support a hypothesis of BCM being reactive and inflammatory rather than neoplastic (2). These conditions tend to occur in women of reproductive age, but cases have been reported in men (3). The most common sites are the serosal surfaces of the ovary and uterus, but cases outside the abdomen have been described, including the pleural cavity (4). Typical microscopic findings are a single layer of flattened mesothelial cells sometimes described as a hobnail configuration. Squamous metaplasia and papillae may also be seen (3).


The clinical presentation is usually abdominal or pelvic pain, a mass found clinically or radiologically, or an incidental surgical finding (5). BCM is considered to be a benign inflammatory process; however, malignant transformation has been reported (6). Diagnostic modalities include ultrasound and CT, but preoperative diagnosis is often not conclusive and there are no protocols for diagnostic imaging. The main differentials are ovarian cysts, ovarian tumors (benign or malignant), or cystic lymphangioma. When presenting acutely with signs of infection as in the case described here, pelvic inflammatory disease complicated by abscess would be the most common differential as associations with appendicitis are very rare.


Management currently involves surgical resection, but recurrences are well documented. There are no protocols for surgical management, and the literature is based on case reports and small case series. Laparoscopic resections have been described. While laparoscopy is an elegant tool for investigation of masses or pain in women, we believed open surgery to be safer when a malignant process was suspected owing to the possibility of cyst rupture and seeding. Follow-up after surgical resection includes clinical review and ultrasound or CT, but again there are no guidelines.


BCM involving the appendix is very rare. Only four other cases have been reported. Two were in middle-aged women presenting with abdominal pain and suspected appendicitis where cysts were found adjacent to but not involving the appendix (7, 8). In a third case, a BCM was found incidentally beside an otherwise unremarkable appendix at laparotomy for sigmoid diverticular disease (9). The other case involved a 28-year-old man who presented with appendicitis in which a 25-cm separate cystic mass was found (10). Our patient's case is unique in that the BCM was in direct continuity with the tip of the appendix and presented with both clinical signs and histological evidence of acute appendicitis.


BCM is a rare benign tumor, but surgeons should include it in the differential when investigating abdominal masses or pain in women of reproductive age. This is the first reported case of a BCM arising from the appendix which complicated a presentation of acute appendicitis. Surgery is the authors' recommended treatment, but patients should be advised of the possibility of recurrence.


The authors declare that they have no competing interests.


DB O'C drafted and conceived the manuscript, DB assisted in the drafting and editing of the final manuscript, and MA performed critical revisions of the manuscript.


DB O'C and MA performed the operation. All authors read and approved the final manuscript.


The authors have written informed consent from the patient for the publication in a medical journal of the manuscript and images. A copy of this consent can be made available to the editorial team.


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Sunday, January 2, 2011

Tamsulosin-induced severe hypotonia during general anesthesia: a case report

Tamsulosin, a selective a1-adrenergic receptor (a1-AR) antagonist, is a widely prescribed first-line agent for benign prostatic hypertrophy (BPH). Its interaction with anesthetic agents has not been described.


We report the case of 54-year-old Asian man undergoing elective left thyroid lobectomy. The only medication the patient was taking was tamsulosin 0.4 mg for the past year for BPH. He developed persistent hypotension during the maintenance phase of anesthesia while receiving oxygen, nitrous oxide and 1% isoflurane. The hypotension could have been attributable to a possible interaction between inhalational anesthetic and tamsulosin.


Vigilance for unexpected hypotension is important in surgical patients who are treated with selective a1-AR blockers. If hypotension occurs, vasopressors that act directly on adrenergic receptors could be more effective.


Tamsulosin (benzenesulfonamide) is a uroselective a1A-adrenergic receptor (a1A-AR) antagonist primarily used for patients with benign prostatic hyperplasia (BPH) presenting with lower urinary tract symptoms [1]. The recommended dose is 0.4 mg or 0.8 mg with a half-life of nine to 15 hours. It is extensively metabolized by the liver.


Currently, a1-adrenergic receptor (a1-AR) antagonists are commonly prescribed by physicians as first-line agents to treat BPH, a common condition of aging men. a1-AR antagonists exert their effects by blocking a1-AR-mediated contraction of the prostatic smooth muscle cells and bladder neck [2]. There are three a1-AR subtypes: a1A, a1B and a1D. Terazosin, doxazosin and alfuzosin are a1-AR antagonists that show equal affinity for all a1-AR subtypes [3]. Tamsulosin does not interfere with blood pressure control and has a low potential to cause vasodilation [4]. It is selective for a1A-and a1D-AR subtypes but has less affinity for the a1B subtype.


A 54-year-old, 80-kg Asian man was scheduled for left thyroid lobectomy for a thyroid nodule. He was clinically euthyroid with normal thyroid function test results. The patient's medical history was insignificant except for BPH. His New York Heart Association classification was class I. For BPH, he was being treated with tamsulosin 0.4 mg at night for one year. He was a smoker and had been smoking approximately 20 cigarettes a day for the past 24 years. He had no known history of allergy to any drug. He consented to general anesthesia for thyroid lobectomy.


Before induction of anesthesia, the patient's recorded blood pressure (BP) was 137/88 mm Hg, heart rate was 72 bpm, and temperature was 36.5°C. Cefazolin 1 g was administered intravenously (IV) in the operating room without any adverse effects. After placement of routine monitors and preoxygenation, general anesthesia was induced with sodium thiopentone 5 mg/kg, pethidine 1 mg/kg and atracurium 0.5 mg/kg, and the patient's trachea was intubated with a reinforced endotracheal tube without any difficulty. The lungs were ventilated with a controlled mode of ventilation with O2/N2O and 1% isoflurane. The two BP readings taken after induction were 110/70 and 100/64 mm Hg. After laryngoscopy, the patient's maximum BP reading was 162/96 mm Hg, and his heart rate was 104 beats/min. Within 10 minutes, the patient's BP decreased to 75/45 mm Hg despite a rapid infusion of 1 L of lactated Ringer's solution. His heart rate remained at 70 bpm, and his oxygen saturation was between 98% and 99%. End-tidal carbon dioxide remained between 33 and 36 mm Hg, with a respiratory rate of 10 breaths/min. Ephedrine 30 mg in divided IV doses only transiently improved the hypotension (BP to 85/40 mm Hg), and frequent phenylephrine boluses (100 µg/bolus) were required to maintain systolic BP above 90 mm Hg. The patient's skin was warm but not diaphoretic. Skin erythema, urticaria, bronchospasm, facial edema and other features of a potential anaphylaxis or anaphylactoid reaction were absent. No ischemic changes were observed on a three-lead electrocardiogram (ECG). General anesthesia was maintained with O2/N2O and isoflurane minimum alveolar concentration (MAC) of 0.8%. At this point, the differential diagnosis of hypotension was primarily directed to deep anesthesia or hypovolemia. While these were being addressed, the surgeon was allowed to proceed. There was continuing hypotension requiring phenylephrine. At the time of surgical incision, the patient moved slightly in response to surgical stimulation. The total duration of surgery was 180 minutes (three hours), and the total estimated blood loss was 70 mL. A total of 1200 µg of phenylephrine was required to maintain the systolic a BP above 90 mm Hg throughout surgery.


At the end of surgery, isoflurane was turned off, and the patient's BP increased to 110/70 mm Hg with a heart rate of 75 bpm. The patient completely recovered from neuromuscular blockade after administration of 0.2 mg of glycopyrrolate and 2.5 mg of neostigmine. The patient's trachea was then extubated, and he was shifted to the postanesthesia care unit. He remained in the recovery room for two hours and remained hemodynamically stable with BP in the range of 120/75 to 140/80 mm Hg and heart rate between 70 and 76 bpm.


BPH is common among older men, with approximately 25% of men being affected after 40 years of age [5]. Histologic evidence of the disease is noted in 8% of men in their 30 s, and the prevalence rapidly increases to more than 70% after the age 60 years [6].


All a1-adrenoceptor antagonists have a similar efficacy in improving symptoms and urinary flow. Many of these agents were initially developed and approved for the treatment of patients with hypertension until the development of tamsulosin. Tamsulosin is a more selective a1A subtype antagonist, which maintains the a-antagonist effect on the prostatic capsule and bladder neck but has less of an effect on the vascular system and BP. Tamsulosin is not indicated in the treatment of patients with hypertension.


A meta-analysis related to the vascular-related safety profile of a1 antagonists was carried out by Nickel et al [7]. They concluded that patients administered alfuzosin, terazosin and doxazosin showed a statistically significant increased risk of developing vascular-related events (dizziness, hypotension or syncope) compared with those taking placebo. Tamsulosin showed only a numerical increase that was not statistically significant.


A MEDLINE search from 1997 to the present revealed no report of any adverse hemodynamic consequences of tamsulosin interaction under anesthesia. Tamsulosin drug interactions have been studied in humans; the use of tamsulosin with nifedipine, enalapril, atenolol, furosemide or digoxin does not require dosage modification when tamsulosin is initiated concomitantly with these agents [8,9]. Tamsulosin is contraindicated with other a1 antagonist and verapamil, which has a1-adrenoceptor antagonist effects in therapeutic doses [10]. It is contraindicated in patients with orthostatic hypotension.


This case may illustrate a drug interaction between tamsulosin and inhalational anesthetic agents. Isoflurane is the most likely anesthetic drug to have interacted because the phenylephrine requirement lasted until the end of the case, and the hypotension resolved when isoflurane was stopped. The hypotension in this patient may have been caused by chronic use of tamsulosin. Isoflurane is a known vasodilator and may have synergetic effect with tamsulosin. This may explain why ephedrine was less effective than phenylephrine in counteracting hypotension. Whereas phenylephrine is a pure a1-AR agonist, ephedrine has both central and peripheral effects. In the clinical doses administered, its peripheral effects may be less than those of phenylephrine. Ephedrine was administered initially because the differential diagnosis considered at that time was either excessive depth of anesthesia or hypovolemia. When a synergetic effect was suspected, phenylephrine was used instead. The point that negated vasodilation caused by an inappropriate depth of anesthesia was that the patient was receiving less than 1 MAC of isoflurane, and he moved in response to surgical stimulation at the time of incision. Hypovolemia was ruled out when the BP did not rise after administration of 1 L of crystalloid.


The degree of hypotension was exceptional compared with the concentration of anesthetic agent used. The other differential diagnosis for this vasodilated hypotensive state included anaphylaxis and myocardial ischemia. Because the patient did not have any other features of anaphylaxis and had a subsequent negative ECG result for ischemia, these alternative diagnoses were rejected. Other anesthetic drugs used in this patient were thiopentone, pethidine and atracurium. Pethidine and atracurium can both cause histamine release, but no additional clinical signs of histamine release were apparent in this patient. The dramatic increase in BP when isoflurane was turned off goes in favor of some kind of interaction, although the possibility of genetic sensitivity to isoflurane cannot be ruled out completely.


As a result of the recent increase in prescriptions of tamsulosin for aging men with BPH, hypotension under anesthesia associated with this drug may occur. To remove tamsulosin from the circulation would require days of abstinence because tamsulosin has a long half-life of nine to 15 hours.


This case reports hypotension in a patient on tamsulosin under general anesthesia with N2O/O2, atracurium and isoflurane anesthesia. Other factors may have contributed to the decreased BP, and tamsulosin cannot be implicated definitively. This case nevertheless demonstrates the importance of vigilance for unexpected hypotension in patients taking tamsulosin. If hypotension does develop in these patients, a direct-acting vasopressor such as phenylephrine should be used.


a1-AR ANTAGONIST: a1-adrenergic receptor antagonist; BPH: benign prostatic hypertrophy; ECG: electrocardiogram; MAC: minimum alveolar concentration; N2O: nitrous oxide.


Written informed consent was obtained from the patient for publication of this case report. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


The authors declare that they have no competing interests.


Both authors contributed to the clinical management of the case. The initial draft of the case report was written by DK, and repeated revisions were done by both DK and FAK. Both authors read and approved the final version of the manuscript.


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