A 31-year-old woman, a native of Lakshadweep, who went through a life-threatening condition caused by a huge thyroid tumour which had grown into the chest cavity, was saved through a rare surgery at VPS Lakeshore Hospital.

The patient had developed severe stridor and was put on a ventilator as the tumour was blocking her respiratory passage completely. She also developed cardiorespiratory arrest and was intubated by an anaesthesiologist in Lakshadweep before she was airlifted to Kochi with the ventilator support. The mission of shifting the critically ill patient was carried out by four doctors.

Her evaluation showed a huge tumour of thyroid fully occupying the neck and had grossly shifted the respiratory passage or trachea. A CT scan showed that the tumour had grown into the chest and was compressing the heart, bronchus and major blood vessels. She was having lung and cardiac problems as well. The only option left to save the life of the patient was to remove the large tumour though it was a very risky task. Usually, thyroid and other neck tumours are removed by keyhole procedures with no neck scars. But for this patient, as her tumour was too large and extending into the mediastinum, it was decided to open the neck and mid part of the chest (sternum), according to Dr. R. Padmakumar, head of the department of minimally invasive surgery at the hospital who led the surgery.

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Padmakumar R, Chiramel KJ, Pai M, Shams F, Subin P. Management of polycythemia vera by endoscopic parathyroidectomy. J Appl Hematol [serial online] 2020 [cited 2020 Nov 20];11:204-7.

Available from: https://www.jahjournal.org/text.asp?2020/11/4/204/300771

Contents

1. Abtract
2. Introduction
3. Case Report
4. Outcome
5. Discussion
6. Conclusion
7. References

Abstract

Polycythemia vera (PV) is a hemotologic disease. Majority of persons with Polycythemia vera, essential thrombocythemia and primary myelofibrosis show the Janus kinase (JAK) 2 gene– which is needed for the normal development of blood cells. Polycythemia vera is generally controlled with medication: hydroxyurea or pipobroman. Phlebotomy is the mainstay of therapy for PV. Association between hyperparathyroidism and PV has been very sparingly considered. Primary hyperparathyroidism may produce a growth factor, which induces pancytosis, especially in the presence of high levels of ionized calcium. It is found that parathyroid hormone levels (PTH) could influence hemopoiesis through a direct action on hemopoietic precursors. A 52-year-old female presented with complaints of fever associated with headache and generalized weakness.

On evaluation, her hemoglobin value was found to be high (Hb– 18.8 g/dl) with leukocytosis. She was evaluated for polycythemia vera, which showed JAK-2 mutation positivity. She was started on Enteric Coated Aspirin. She was managed initially with twice-weekly venesection to keep packed cell volume <45. It was observed that she was having very high PTH– 569.9 pg/ml and low Vitamin D levels– 8.41 mg/ml. She underwent a Technetium 99M Sestamibi Scan (TcMIBI), which showed a large adenoma involving the left inferior parathyroid gland. She was taken up for endoscopic parathyroid removal through the left axillo-breast approach. Intraoperatively, PTH value had dropped from 569.9 pg/ml to 62 pg/ml. Hemoglobin level decreased to normalcy by the 2^nd week of surgery without any medication or venesection.

Patients with polycythemia vera must be screened for hyperparathyroidism. Early treatment of hyperparathyroidism by parathyroid removal will take care of polycythemia vera as well. Endoscopic parathyroidectomy is very feasible, safe, and well accepted by patients.

Keywords: Hyperparathyroidism, Janus kinase-2 mutation, parathyroid adenoma endoscopic parathyroidectomy, polycythemia vera

Introduction

Polycythemia vera (PV) is a hemotologic disease. In Polycythemia vera, hyperviscosity results from the increased red cell count. The associated increase in white cells and platelets can lead to headache, fatigue, and other symptoms. Thrombus formation of artery or vein may also be a feature. Majority of persons with PV, essential thrombocythemia (ET) and primary myelofibrosis show Janus kinase (JAK) 2 gene, which is needed for the normal development of blood cells. The presence of JAK-2 distinguishes the above disorders from other myeloproliferative disorders (MPD). Hence, it avoids the use of chemotherapeutic agents currently employed in the treatment of MPD.^[1] The identification of JAK-2 and increased RBC are the two significant components of making a diagnosis of PV. Ninety-five percent of Polycythemia vera cases show JAK-2 V617F. Other JAK–2 mutations located in axon 12 can be detected in 2%–5% of cases. JAK-2 V617F mutation-positive patients respond well to treatment compared to mutation-negative patients.

Phlebotomy is the mainstay of therapy for PV. It is done with the aim of keeping the packed cell volume (PCV) <45% in males and 42% in females. It is done with the aim of reducing problems associated with erythrocytosis. The administration of aspirin is advised to reduce ocular migraine and microvascular complications.

High calcium level in polycythemia vera is generally believed to have a link with malignancies such as renal cell carcinoma. High red cell volume can also be induced by chronic lung disease, stenosis of the renal artery, hydronephrosis, chronic smoking, and hepatocellular carcinoma. The link of hyperparathyroidism to polycythemia vera is generally not thought off. There is some functional relationship between parathyroid hormone levels (PTH), osteoblastic and osteoclastic activity as well as hematopoiesis.

Primary hyperparathyroidism generally results from an adenoma, hyperplasia, or carcinoma of parathyroid glands; that may require surgical intervention. The classical biological signs of hyperparathyroidism are hypercalcemia, hypophosphatemia, increased PTH in blood, and increased cyclic adenosine monophosphate urinary elimination. The parathyroid tumor may produce a growth factor which induces pancytosis, especially in the presence of ionized hypercalcemia. Hemopoietic precursors may be directly influenced by PTH, resulting in hemopoiesis. Moderate levels of PTH can induce hematopoiesis but maybe inhibitory at a high concentration. We are presenting a case of polycythemia vera associated with primary hyperparathyroidism due to parathyroid adenoma.

Case Report

A 52-year-old postmenopausal female presented with complaints of fever associated with headache and generalized weakness. She was on treatment for systemic hypertension with telmisartan, chlorthalidone, and nifedipine. On evaluation, her hemoglobin value was found to be high (Hb– 18.8 g/dl) with neutrophilic leukocytosis and mild lymphocytosis. Peripheral smear showed normocytic normochromic blood picture with erythrocytosis. The Serum iron level was 65 mcg/dL. She was evaluated for polycythemia, which showed JAK-2 mutation positivity. She was started on enteric coated aspirin.

Elevated uric acid (10.2 mg/dL) and calcium (13.53 mg/dL) were initially taken as part of polycythemia. But when it increased to 13 and 14.9, respectively, she was evaluated in detail for any parathyroid pathology. It was observed that she was having very high PTH– 569.9 pg/ml and low Vitamin D levels (25-OH Vit. D)– 8.41 mg/ml.

She did not have any bone pain or features of stone diseases. She underwent a Technetium 99M Sestamibi Scan (TcMIBI) double phase parathyroid scintigraphy, which showed large adenoma involving the left inferior parathyroid gland, later confirmed with ultrasound. USG abdomen was unremarkable.

She was managed initially with twice-weekly venesection of 350 ml to 400 ml to keep PCV <45. She was then referred to our department to proceed with endoscopic parathyroidectomy. She was taken up for endoscopic parathyroid removal through the left axillo-breast approach under general anesthesia.

The procedure of endoscopic parathyroidectomy through axillo-breast approach is performed in the following manner. A 10-mm trocar was placed on the anterior axillary fold to introduce the telescope. In the beginning, the telescope aids in dissecting the space. Later, it shows the structure with magnification and delineation. Two working ports of 5 mm were placed in the subplatysmal area. This will expose the sternocleidomastoid and sternohyoid muscles.

The plain medial to the sternocleidomastoid (lateral to the strap muscles) was opened to expose the left lower lobe of the thyroid and the nearby parathyroid adenoma. A large left inferior parathyroid gland adenoma was identified. By blunt dissection, the parathyroid adenoma was mobilized, safeguarding the recurrent laryngeal nerve. The parathyroid vessels were sealed and divided using ultrasonic device. The gland was placed in a custom made endobag and removed in toto through the 10-mm trocar. The drainage tube was placed, and subcuticular closure was performed [Figure 1]. The parathyroidectomy specimen was given for histopathological assessment. Intraoperatively, PTH value had dropped from 569.9 pg/ml to 62 pg/ml. Her course in the hospital was uncomplicated.

Figure 1: Incisions for Endoscopic Parathyroidectomy on left side

HPE revealed a 3.5 cm × 3 cm × 2 cm parathyroid adenoma weighing 5 g. It had mitosis <1/10 HPF. The capsule was intact, and there was no capsular invasion [Figure 2].

Figure 2: Low power view showing perivascular arrangement of tumor cells

Outcome

Her wounds healed well with minimal scarring and the symptoms at the presentation– headache and weakness disappeared. Hemoglobin level decreased to normalcy by the 2^nd week of surgery. Antihypertensives were reduced to single drug– Telmisartan (compared to three drugs preoperatively). Six months following surgery, the PTH value is 28 pg/mL, serum calcium is 9.1 mg/dL, and Hb is 14.3 g/dL. She is continuing the enteric-coated Aspirin– 75 mg once daily.

Discussion

Chronic myeloproliferative neoplasms (CMPNs) are diseases of clonal multipotent stem cells. The four classical subgroups are: chronic myeloid leukemia, primary myelofibrosis (PMF), PV, and ET. Almost all patients having PV, 60%–65% of patients with ET have JAK2 V617F point mutation. JAK2 mutations cause hypersensitivity in hematopoietic progenitor cells towards growth factors and other cytokines. JAK2 V617F mutation-positive PV and ET patients had lower vitamin D levels.^[6] Other than the effects on calcium-phosphate metabolism and bone balance, Vitamin D has an impact on cell proliferation, differentiation and cell adhesion as well as proliferation and apoptosis of tumor cells. In a study on patients with CMPNs, it was observed that there was a high prevalence of Vitamin D deficiency in patients with PV and ET– more so with PV. Vitamin D deficiency was observed in our patient also.

It has been described that PTH may induce hemopoiesis by a direct action on hemopoietic precursors– at a low concentration. It may be inhibitory at a high concentration. In our patient, we found that she had very high PTH but still had erythropoiesis. An article in the Journal of Endocrinology and Metabolism has mentioned about parathyroid adenoma being a risk factor for PV.^[7] Weinstein, in his study, has mentioned that reduction in calcium level in the postoperative period was associated with remission of PV. When the hypercalcemia recurred at a later time, it was again accompanied by pancytosis.^[8] In 4 out of 5 cases, parathyroidectomy resulted in complete and stable remission of PV even without any treatment.^[5] Godeau et al. also reported a case of PV with primary hyperparathyroidism, which responded successfully with the removal of parathyroid adenoma.^[9]

In a case reported in the International Journal of Haemotology, the patient had a JAK-2 status negative and they observed only transient (<1 year) control of PV following parathyroidectomy.^[4] George M Rogers has observed that PTH increases ferric chloride Fe 59 incorporation into erythrocytes of polycythemic mice. He also stated that PTH might increase erythropoiesis not only in pathological situations but also may play a role in the regulation of normal erythropoiesis.^[10]

Multiple endocrine neoplasias (MEN) I and II will have multiple adenomas or hyperplasia of the parathyroid glands. Parathyroid tumor, along with pancreatic and pituitary tumors, is the typical pattern of MEN1. The lesions in MEN-2A are pheochromocytoma, medullary carcinoma thyroid, parathyroid adenoma, or hyperplasia. In addition to the components of MEN-2A; MEN-2B will have oral and submucosal tumors. Detailed evaluation to look for these lesions is essential once a parathyroid adenoma is encountered. We also evaluated our patients fully and excluded MEN.

The surgical results of our patients were excellent is in terms of resolution of PV and hyperparathyroidism. As we proceeded with endoscopic parathyroidectomy the patient was very comfortable and could get excellent cosmetic outcomes without any neck scars. It also encouraged the patient to undergo the procedure at an early stage. It will also motivate her to undergo another procedure in case other parathyroids go in for hyperplasia or adenoma later.

Conclusion

Patients with polycythemia vera must be screened for hyperparathyroidism. Early treatment of hyperparathyroidism by parathyroid removal will take care of polycythemia as well. Endoscopic parathyroidectomy is very feasible, safe, and well accepted by patients.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

Zhan H, Spivak JL. The diagnosis and management of polycythemia vera, essential thrombocythemia and primary myelofibrosis in the JAK2V617F era. Clin Adv Hematol Oncol 2009;7:334-42.

Kiladjian JJ. The spectrum of JAK2-positive myeloproliferative neoplasms. Hematology Am Soc Hematol Educ Program 2012;2012:561-6.

Bae EH, Kim HS, Kim MJ, Kang YU, Kim YH, Kim CS, et al. Hypercalcemia in a patient with polycythemia vera. Chonnam Med J. 2012 ;48:128-9. doi: 10.4068/cmj.2012.48.2.128. Epub 2012 Aug 24. PMID: 22977755; PMCID: PMC3434793

Kulaylat AN, Jung EE, Saunders BD. The role of parathyroidectomy in JAK2 mutation negative polycythemia vera. Int J Hematol 2014;100:615-8

Boivin P, Bernard JF. Polycythaemia and hyperparathyroidism: A fortuitous association? Eur J Haematol 1992;49:153-5.

Yikilmaz AŞ, Akinci S, Bakanay ŞM, Dilek İ. Vitamin D Deficiency and Janus kinase 2 V617F Mutation Status in Essential Thrombocythemia and Polycythemia Vera. Malays J Med Sci. 2020 ;27:70-7. doi:10.21315/mjms2020.27.1.7. Epub 2020 Feb 27. PMID: 32158346; PMCID: PMC7053540

Fallah M, Kharazmi E, Sundquist J, Hemminki K. Nonendocrine cancers associated with benign and malignant parathyroid tumors. J Clin Endocrinol Metab 2011;96:E1108-14

Weinstein RS. Parathyroid carcinoma associated with polycythemia vera. Bone 1991;12:237-9

Godeau P, Bletry O, Brochard C, Hussonois C. Polycythemia vera and primary hyperparathyroidism. Arch Intern Med. 1981 ;141:951-3. PMID: 7235821

Rodgers GM. Hyperparathyroidism associated with polycythemia. Arch Intern Med 1982;142:951-3

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Hyperhidrosis is sweating in excess than that is required for normal thermoregulation.  It begins in either childhood or adolescence. Although any site on the body can be affected by hyperhidrosis, the sites most commonly affected are the palms, soles, and axillae. Hyperhidrosis exists in 3 forms: Emotionally induced hyperhidrosis (in which it affects the palms, soles, and axillae,    Localized hyperhidrosis, Generalized hyperhidrosis. Hyperhidrosis often causes great emotional distress and occupational disability for the patient, regardless of the form.It affects both sexes and affects persons of all ages.In a study of 850 patients with palmar, axillary, or facial hyperhidrosis, 62% of patients reported that sweating began since before they could remember; 33%, since puberty; and 5%, during adulthood.

Hyperhidrosis Treatment Options

There are two options for Hyperhidrosis treatment:

1. Medical management
2. Surgical management

Medical Management – Includes topical and systemic medications for hyperhidrosis treatment.

Sympathectomy – Sympathectomy is the surgical option for hyperhidrosis treatment and involves the surgical destruction of the ganglia responsible for hyperhidrosis.

Authors: Dr. R. Padmakumar, Dr. Madhukara Pai, Dr. Arun kumar S, Dr. Farish Shams, Dr. Zuhail Bin Nazar, Dr. Khaleel Abdulkader, Dr. Mabel Pereira, Dr. Anurag verma

Laparoscopic Lumbar Sympathectomy – Case Report

Laparoscopic Lumbar Sympthectomy for hyperhidrosis treatment of lower limb.

32-year-old male patient from Lakshadweep had reported to us with complaints from his childhood and was operated on right side retroperitoneoscopic lumbar sympathectomy before one year by same team. His complaints resolved completely on the right side. His symptoms are cold limb, profuse sweating of the left lower limb. When he used casual slippers, his slippers slip off and when he wears shoe, it pours out.

The temperature in lower limb was recorded pre-operatively and found to be very low. (less than 88 F) (Figure 1) Routine blood investigations and anaesthetic check-up was done, he was posted for retroperitoneoscopic lumbar sympathectomy under GA. Patient was operated and L2, L3, L4 ganglions removed. Patient had no issues in immediate post-op. Patients symptoms improved drastically in the post-op period. His temperature improved to 97.6 F and his limb became pinkish and was not having the issues of sweating. Patient was discharged on POD-2. (Figure 2)

Discussion

Anatomy

The sympathetic trunk lies lateral to the vertebral bodies for the whole length of the vertebral column. It communicates with the anterior rami of spinal nerves via rami communicants. The sympathetic trunk permits preganglionic fibres of the sympathetic nervous system to ascend to spinal levels superior to T1 and descend to spinal levels inferior to L2/3.

The superior end of it is continued upward through the carotid canal into the skull, and forms a plexus on the internal carotid artery; the inferior part travels in front of the coccyx, where it converges with the other trunk at a structure known as the ganglion impar.

Along the length of the sympathetic trunk are sympathetic ganglia known as paravertebral ganglia.The sympathetic trunk is a fundamental part of the sympathetic nervous system, and part of the autonomic nervous system. It allows nerve fibres to travel to spinal nerves that are superior and inferior to the one in which they originated. Also, number of nerves, such as most of the splanchnic nerves, arise directly from the trunks.

Pathophysiology

Hyperhidrosis may be idiopathic or secondary to other diseases, metabolic disorders, febrile illnesses, or medication use. Generalized hyperhidrosis may be secondary to numerous conditions including the following: Neurologic or neoplastic diseases, spontaneous periodic hypothermia and hyperhidrosis. Localized unilateral or segmental hyperhidrosis is rare and of unknown origin. The condition usually presents on the forearm or forehead in otherwise healthy individuals, without evidence of the typical triggering factors found in essential hyperhidrosis. Localized hyperhidrosis may also be associated with the following: gustatory stimuli, eccrine nevus.

Diagnosis/Evaluation

Diagnostic criteria favouring primary hyperhidrosis include excessive sweating of 6 months or more in duration, with 4 or more of the following:

1. Primarily involving eccrine-dense sites (axillae/palms/soles/craniofacial).
2. Bilateral and symmetric
3. Absent nocturnally
4. Episodes at least weekly

Visible signs of hyperhidrosis are clear.

If direct visualization of the affected areas by hyperhidrosis is desired, the iodine starch test may be used. This test requires spraying of the affected area with a mixture of 0.5-1 g of iodine crystals and 500 g of soluble starch. Areas that produce sweat turn black. If generalized hyperhidrosis is noted one must search for underlying causes by doing Thyroid Function Test, Blood glucose, Urine catecholamines, Uric acid levels, Purified Protein Derivative, and Chest x-ray to rule out Tuberculosis.

Hyperhidrosis Treatment

There are two options for hyperhidrosis treatment:

1. Medical management
2. Surgical management

Medical management

Hyperhidrosis treatment is challenging for both the patient and the physician. Both topical and systemic medications have been used.

Topical agents for hyperhidrosis treatment therapy include topical anticholinergics, boric acid, 2-5% tannic acid solutions, resorcinol, potassium permanganate, formaldehyde, glutaraldehyde, and methenamine. Systemic agents used to treat hyperhidrosis include anticholinergic medications such as propantheline bromide, glycopyrrolate, oxybutynin and benztropine. They are effective because the pre-glandular neurotransmitter for sweat secretion is acetylcholine (although the sympathetic nervous system innervates the eccrine sweat glands).

Other treatment options for hyperhidrosis include iontophoresis and botulinum toxin injections. Botulinum toxin injections are effective because of their anticholinergic effects at the neuromuscular junction and in the postganglionic sympathetic cholinergic nerves in the sweat glands.Radiofrequency ablation and use of microneedle radiofrequency therapy for axillary hyperhidrosis has been recommended.

Surgical management

Sympathectomy has been used as a permanent effective hyperhidrosis treatment method since 1920. Usually, it is reserved for the final treatment option. Sympathectomy involves the surgical destruction of the ganglia responsible for hyperhidrosis. 

Lumbar sympathectomy is an effective hyperhidrosis treatment for lower limb hyperhidrosis. There are two approaches: open and minimal access approach.

In case of minimal access surgery there are transperitoneal and retroperitoneoscopic approach.

Retroperitoneoscopic approach:

The patient is placed in a lateral position. A 15-mm incision is made just below the 12th rib, and retroperitoneal space is created using blunt finger dissection. A custom-made, large balloon is inserted and inflated with the equivalent of 750 mL to 1000 mL of air. The second 10-mm port is placed in line with the first port above the iliac crest.The third and fourth 5-mm ports are placed anterior to the first 2 ports.Peritoneum is retracted anteriorly. The medial border of the psoas muscle is used as a landmark and a chain identified immediately medial to it. The second to fourth lumbar sympathetic ganglia are removed with the intervening chain. The port sites are closed without a drain.

Conclusion

Retroperitoneoscopic approach is better than open, as there is no cutting of muscles, also the blood loss is less, the patient has less post-op pain, also chance of wound infection are less comparatively.The patient goes home early, and goes to work the next day. Its comparatively safer than transperitoneal as the chance of bowel injury, infection is less as we are in the retroperitoneal space. Approaching sympathetic chainis also relatively easier for surgeon in case of retroperitoneoscopy.

References

1. Altman RS, Schwartz RA. Emotionally induced hyperhidrosis. Cutis. 2002 May. 69(5):336-8. [Medline].
2. Ruchinskas R. Hyperhidrosis and anxiety: chicken or egg?. Dermatology. 2007. 214(3):195-6. [Medline].
3. Drott C, Gothberg G, Claes G. Endoscopic transthoracic sympathectomy: an efficient and safe method for the treatment of hyperhidrosis. J Am Acad Dermatol. 1995 Jul. 33(1):78-81. [Medline].
4.  Mader S. S. (2000): Human biology. McGraw-Hill, New York, ISBN 0-07-290584-0; ISBN 0-07-117940-2.
5. Pritchard T. E., Alloway D. (1999): Medical neuroscience. Hayes Barton Press, ISBN 978-1-593772000
6. Rodrigues Masruha M, Lin J, Arita JH, DE Castro Neto EF, Scerni DA, Cavalheiro EA, et al. Spontaneous periodic hypothermia and hyperhidrosis: a possibly novel cerebral neurotransmitter disorder. Dev Med Child Neurol. 2010 Dec 17. [Medline].
7. Mehta S, Ralot T, Masatkar V, Agarwal N, Rana A. A curious case of hourly attacks of disabling episodic spontaneous hypothermia with hyperhidrosis. Indian J Dermatol VenereolLeprol. 2015 Mar-Apr. 81(2):185-6. [Medline].

Related Links

• Thyroglossal Cyst – Endoscopic Excision – First of its kind in Kerala

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Said Ahmad Said Al Hadidi, a 12-year-old Omani boy, presented with severe hypertension and resultant cardiac issues. He was found on evaluation to have large tumours of the adrenal gland bilaterally. These tumours called Pheochromocytoma are rare in children, especially bilateral tumors can be very rare. The boy’s blood pressure was temporarily controlled with three types of drugs in large doses. Sudden blood pressure rises due to these tumours can cause heart attack, stroke or even sudden death.

Adrenal glands also called suprarenal glands are endocrine glands which are located above the kidneys on both sides and has got the major function of secreting a variety of hormones. The Pheochromocytoma is a neuroendocrine tumor which affects the inner layer of the gland that can lead to uncontrolled secretion of Adrenalin and nor-adrenalin which cause dangerous levels of blood pressure.

The patient was prepared for surgery. The Anesthesiology team took care of his vitals during this complex surgery which lasted five hours. The team was led by Laparoscopic Surgeon Dr R Padmakumar and they successfully removed the tumour on both sides completely without blood loss. Dr Madhukar Pai, Dr Khaleel were also actively involved in this major surgery as it was very high risk for patient’s life.

Pheochromocytoma Surgery Team

• Dr. R Padmakumar, Chair- Minimally Invasive Surgery
• Dr. Madhukar Pai
• Dr. Khaleel
• Dr. Zuhail
• Dr. Arun
• Dr. Anurag

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Symptoms of Liposarcoma

You may notice a lump that can be usually painless, but growing slowly. Unfortunately, abdominal tumors can grow to be quite large before they are found. Liposarcoma forming in the abdomen can cause abdominal pain, swelling of the abdomen, blood in the stool, constipation, feeling of fullness sooner when eating.

Types of Liposarcoma

There are four types of liposarcoma

• Well-differentiated liposarcoma
• Myxoid liposarcoma
• Pleomorphic liposarcoma
• Dedifferentiated liposarcoma

A Case Study

82-year-old man came in with onset of pain in the abdomen since one week, inability to have food since few days, and gradually increasing abdominal distension for past six months.

On physical examination, his general condition okay, abdomen grossly distended with huge mass.

CECT (High-dose Contrast-Enhanced Computed Tomography) revealed mass extending from diaphragm to pelvis: ? Liposarcoma.

Midline laparotomy was performed, there was a huge mass in the mesentery of the large bowel. The mass was carefully separated and resected weighing 4.2 kg. There was another mass attached to the small bowel by single vessel causing torsion and obstruction of bowel. Derotation of the bowel was carried out, division of the feeding vessel was done and the mass was resected weiging 1.2 kg.

Liver nodule was taken for biopsy and biopsy report confirmed Liposarcoma

Post-op recovery was good and the patient was taking normal diet.

Dr. Padmakumar and Team performs various cancer surgeries in Kochi.

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Three years old girl weighing only 10 kg was operated successfully through endoscopic method. Girl child hailing from Oman was suffering from a rare congenital condition called branchial cyst of second branchial cleft. The swelling had grown in such a size that she was finding it difficult to bend neck.

She was successfully treated by endoscopic surgery by a team led by Dr. R Padmakumar. The procedure was carried out through a 10 mm cut in the axilla (armpit) and nearby two 3 mm cuts. The entire swelling closely adherent to the main blood vessels (the carotid and jugular veins) and nerve to diaphragm was safely and completely removed. This kind of operation in the neck; that too for such young kid, was performed for the first time in the world. “The team’s expertise in doing endoscopic thyroidectomies for many patients really helped in successfully completing this unique procedure” said Dr. R. Padmakumar. As there is no cut in the neck child revived fast and could go back to Oman in three days time. Dr. Madhukara Pai, Dr. Mary Varghese, Dr. Shaji P.G and Dr. Sayooj Mukundan were part of this historic surgical procedure.

View publications in Journal of Pediatric Surgeryfor more details

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Abstract

We were unable to find reports in the published medical literature of any cases of bowel surgery being successfully performed at such a low hemoglobin level, without blood transfusion or blood products pre or post-surgery, with the patient’s uncomplicated recovery. This study is about such a case. A patient presenting with severe gastrointestinal bleeding was diagnosed with enteric fever and multiple ileal ulcers. He had an extremely low hemoglobin level (2 g/dL) and mild renal and hepatic impairment. He was immediately admitted for right hemicolectomy under general anesthesia though he refused transfusion of blood or blood products prior to, during, or after surgery on religious grounds (Jehovah’ s Witnesses). After the surgery and having survived these potentially life-threatening circumstances, he left the hospital without major complications. In such circumstances, lives may be saved by prompt clinical decision-making, collaboration and swift surgical intervention coupled with the immediate consultation and input of the patient and family.

Keywords

Gastrointestinal bleed; Low hemoglobin level; Jehovah’s witnesses; Multiple terminal ileal ulceration; Enterotomy; Right hemicolectomy, Minimum hemoglobin for surgery.

Core tip:

It is unheard of in the medical history to take up a patient with hemoglobin of 2 g/dL for anesthesia and major bowel surgery, without transfusing blood or blood products prior to, during or after surgery; and saving the life without complications. We would like to report regarding such a patient who was treated at our hospital.

INTRODUCTION

Few, if any case studies in the published medical literature report on the successful, uncomplicated surgical outcome of a patient with hemoglobin of 2 g/dL for anesthesia and major bowel surgery, without blood transfusion or blood products pre or post-surgery. Thus, we would like to report our recent experience treating such a patient at our hospital.

CASE REPORT

A 39-year-old male patient was admitted to Sunrise Hospital, Cochin, Kerala, India; after being referred from a nearby hospital [from where he left against medical advice] with a history of severe lower gastrointestinal bleeding. The diagnosis was enteric fever with bleeding from the gastrointestinal tract. Salmonella typhi “O” and “H” titer was 1/640, which was confirmed by tube agglutination test (stained febrile antigen set, Manufacturer- Span diagnostics ltd, Sachin, Surat, Gujarat, India). His hemoglobin was extremely low (4 g%) at admission. On examination, he was very pale and icteric, with a pulse rate of 120/beats per minute and blood pressure of 110/70 mmHg and mild distension of the abdomen was present. Table 1 shows the blood investigations of the patient at admission. An immediately performed colonoscopy showed multiple terminal ileal ulcerations with diffuse active bleeding that was not amenable to either colonoscopic or radiological intervention at that stage. Hence, immediate surgical intervention was planned.

It was decided as absolutely necessary to transfuse blood and blood products to correct the hemoglobin level, which had dropped to 2 g/dL within 12 h of admission. We discussed with the patient’s relatives the urgent need for blood transfusion, before proceeding with surgery to arrest the ongoing bleeding. However, the patient, his wife and other relatives were firm in their decision not to receive blood or blood products, due to their religious beliefs. Giving erythropoietin alone at that time was not an option in this patient with active bleeding. Thus, we were faced with the option of not performing the surgery at all, but at the almost certain cost of the patient’s life. Wanting to give the patient the chance to survive, it was decided to offer the patient surgery, despite his clinical condition and the treatment constraints mentioned above that make for an extremely risky surgery. The patient was prepared for laparotomy with a high-risk consent that also listed the distinct possibility of death during surgery and the continued unwillingness to receive blood or blood products.

The patient was given general anesthesia, during which minimal intravenous fluids were administered to maintain the blood pressure at 100/60 mmHg, as overhydration could lead to a further drop of in hemoglobin levels. The bowel was found to be thickened at the ileocecal region. Enterotomy was performed and linear ulcers with bleeding base were observed at the terminal ileum. Limited right hemicolectomy was performed, excising the distal 21 cm of ileum including the ulcer, cecum, appendix and a portion of ascending colon. The entire bowel was edematous,and the blood that oozed during resection appeared thin and watery. To reduce the duration of surgery, reconstruction was attempted by side-to-side ileocolic stapler anastomosis, but staplers did not hold due to bowel wall edema. Revision of anastomosis with hand-sewn, end-to-end, ileocolic two-layer suturing was performed with 3-0 Vicryl (polyglycolic acid)-continuous all coat and 3-0 silk, intermittent seromuscular.

Postoperatively, the patient was kept in the intensive care unit for 10 d with nasal oxygen to enrich the available hemoglobin with oxygen. He had features of renal and hepatic impairment, which gradually improved (Table 2). Improvement in hemoglobin level is presented in Figure 1. Total parenteral nutrition and albumin infusion were initiated. He was given intravenous ferric carboxymaltose and recombinant erythropoietin. Small doses of frusemide were given intravenously for the benefit of hemoconcentration.

The patient was put on oral fluids on the fifth day of surgery, which was gradually switched over to soft diet by the seventh day. A high protein, high calorie diet was given together with oral iron supplementation. Gradually, his hemoglobin level improved, as shown in Table 2. The biopsy report showed linear ulcers in the distal ileum, with the largest measuring 1.5 cm. Microscopy showed a mucosal ulcer infiltrated by histiocytes, lymphocytes, plasma cells and occasional neutrophils consistent with typhoid ulcer ileum. No granulomas were present.

DISCUSSION

We were unable to find reports in the published medical literature of any similar cases of bowel surgery being successfully performed at such a low hemoglobin level with the patient’s uncomplicated recovery. Various reports of surgeries performed without blood transfusion in severely anemic patients who does not have the minimum hemoglobin for surgery (hemoglobin level less than 5 g/dL) showed mortality of approximately 50%^[4], which further increased to 91% in the 2 to 3 g/dL group^[5].

Hemoglobin is the major oxygen-carrying protein in blood. Low hemoglobin level is generally defined as less than 13.5 g/dL for men and less than 12 g/dL for women. Unless it is very severe, anemia, perse, does not cause any problems with anesthesia or surgery. Severe anemia can precipitate cardiac arrest due to (1) increased cardiac output; (2) vasodilatation associated with anesthesia; and (3) hemodilution during resuscitation of hemorrhagic shock using saline. The degree of hemodilution is directly associated with acid base imbalance and is proportional to the level of compromise of metabolic recovery, which in turn is projected on to mortality^[6]. In addition, tissue hypoxia due to reduced oxygen-carrying capacity of the blood leads to end-organ damage and systemic immune response syndrome. There also is a risk of surgical site infection, delayed wound healing and bowel leak at the site of the anastomosis.

Jehovah’s Witnesses are a Christian denomination with non trinitarian beliefs distinct from mainstream Christianity and have a worldwide membership of over 7.78 million people. Their beliefs are based on interpretations of the Bible and they prefer to use their own translation. They refuse blood transfusions, which they consider to be a violation of God’s law based on their interpretation of Acts 15:28, 29. Since 1961, the willing acceptance of blood transfusion by an unrepentant member has been grounds for expulsion from the religion. Watch Tower Society literature directs Witnesses to refuse blood transfusions, even in “a life-or-death situation”^[7]. They refuse transfusions of whole blood or of any of its four primary components – red cells, white cells, platelets and plasma (serum)^[7,8].

It is always a dilemma for the surgeon to decide whether to urgently operate on a bleeding patient with hemorrhagic shock without blood transfusion. We believe that life-saving interventions should not be delayed for patients with active bleeding, even though blood transfusion may be refused. Atabek and colleagues have reported a case of active bleeding in a Jehovah’s Witness patient, where early surgery led to rescue of the patient^[9]. Initial conservative treatment with delayed surgery led to a 75% mortality rate, compared with a 20% mortality rate in patients who underwent emergency surgical intervention within 24 h of admission to the hospital^[10,11].

Our patient was at high-risk for cardiac arrest due to oxygen depletion, but eventually survived without complications. Anastomotic site healing was also potentially in jeopardy, but he recovered without leak.

In conclusion, even in the rarest of situations, such as the one described, a successful attempt to save the patient’s life can be made, even though the general condition of the patient is clinically unstable. Prompt decisionmaking and effective communication among the treating doctors and with the patient and his relatives were important factors that helped the patient’s positive outcome. Had the clinical team refused to perform surgery on this patient, or even delayed the operation because of his extremely low hemoglobin, death would likely have been assured.

REFERENCES

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• Atabek U, Spence RK, Pello M, Alexander J, Camishion R. Pancreaticoduodenectomy without homologous blood transfusion in an anemic Jehovah’s Witness. Arch Surg 1992; 127:349-351 [PMID: 1347993]
• Chigbu B, Onwere S, Kamanu C, Aluka C, Okoro O, Feyi-Waboso P, Onichakwe C. Lessons learned from the outcome of bloodless emergency laparotomies on Jehovah’s Witness women presenting in the extremis with ruptured uterus. Arch Gynecol Obstet 2009; 279: 469-472 [PMID: 18677500 DOI: 10.1007/s00404-008-0748-7]
• Namura O, Kanazawa H, Yoshiya K, Nakazawa S, Yamazaki Y. Successful surgical treatment of a ruptured abdominal aortic aneurysm without homologous blood transfusion in a Jehovah’s Witness: report of a case. Surg Today 2001; 31:912-914 [PMID: 11759889]

^*Author contributions: Padmakumar R performed surgical operation, designed the report; Pai M and Farish S contributed to the surgical operation, review of literature, data collection; Rajeev J contributed to the attending doctor for the patients (colonoscopy), editing assistance; Sanjeev T, anesthesiologist for the procedure, was responsible for the literature review; Sreevalsan TV was critical care specialist; Sheetal B and Sooraj YS were attending doctors for the patient; Rowther SS organized the report, statistics, and manuscript preparation.

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