Accepted Manuscript A worst case scenario - an undiagnosed, ruptured AVM managed with unconventional ways Dr Ishu Bishnoi, Mch (Neurosurgery), Dr Vikram Saini, MS (General surgery), Dr Pallavi Chugh, MD (Anesthesia), Dr Sheikhoo Bishnoi, MD (Psychiatry), Dr Geetika Duggal, MD (Anesthesia), Dr Anamika Adib, MBBS PII: S1878-8750(18)32114-4 DOI: 10.1016/j.wneu.2018.09.068 Reference: WNEU 10273 To appear in: World Neurosurgery Received Date: 22 July 2018 Revised Date: 8 September 2018 Accepted Date: 11 September 2018 Please cite this article as: Bishnoi DI, Saini DV, Chugh DP, Bishnoi DS, Duggal DG, Adib DA, A worst case scenario - an undiagnosed, ruptured AVM managed with unconventional ways, World Neurosurgery (2018), doi: https://doi.org/10.1016/j.wneu.2018.09.068. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. ACCEPTED MANUSCRIPT Title – A worst case scenario - an undiagnosed, ruptured AVM managed with unconventional ways Authors 1) Dr Ishu Bishnoi, Mch (Neurosurgery). Assistant Professor, Neurosurgery, RI PT Maharaja Agrasen Medical College, Agroha. ishubishnoi@gmail.com, 07042539159 2) Dr Vikram Saini, MS (General surgery). 2nd year Postgraduate resident, surgery, Maharaja Agrasen Medical College, Agroha. SC General Vikramsaini2006@hotmail.com , 09467577996 Anesthesia, Maharaja M AN U 3) Dr Pallavi Chugh, MD (Anesthesia). 2nd year Postgraduate resident, Agrasen Medical College, Agroha. Drpallavi_mehta@yahoo.com 09996887550 4) Dr Sheikhoo Bishnoi, MD (Psychiatry). Assistant Professor, Psychiatry, 08510084003 TE D Maharaja Agrasen Medical College, Agroha. Dr.sheikhoo@gmail.com 5) Dr Geetika Duggal, MD (Anesthesia). Professor, Anesthesia, Maharaja Medical EP Agrasen College, Agroha. drgeetikaduggal@gmail.com, AC C 08930200806 6) Dr Anamika Adib, MBBS. Junior resident, Neurosurgery, Maharaja Agrasen Medical College, Agroha. Anamika.adib2411@gmail.com , 08708886149 Keywords AVM; clip; hematoma Abbreviations list • AVM – Arteriovenous malformation • GCS – Glasgow coma score ACCEPTED MANUSCRIPT NCCT – Non-contrast computed tomography AC C EP TE D M AN U SC RI PT • ACCEPTED MANUSCRIPT Abstract Introduction – Treatment of AVMs is, nowadays, performed at specialized neurosurgical centers, which are well equipped with microscope with green filter, RI PT neurosurgical cath-lab and experienced neurosurgeons team. The patients diagnosed with AVMs at smaller hospitals should be referred to such hospitals. But what happens when a neurosurgeon has to face the unavoidable worst case scenario, i.e., emergency unplanned surgical excision of undiagnosed, ruptured AVM with large SC hematoma. This case report is about management of such case. Case report - A 26 M AN U years male was brought to emergency with episodes of seizures and sudden loss of consciousness. His GCS score was E1V1M3. CT head revealed right occipital hematoma with mass effect and herniation. Suspected diagnosis was spontaneous tumor or hypertensive bleed. He had right side anisocoria. Emergency surgery was done. Intraoperatively, it was found to be ruptured AVM. After explaining about non- TE D availability of conventional instruments (microscope, clips, ICG) to relatives, we planned for excision of AVM with available resources. Results and conclusion - EP Postoperatively patient recovered satisfactorily with right side vision loss. He underwent cranioplasty after 6 weeks. In such case, being patience, following basic AC C steps, transfusion of blood and lateral thinking about using available resources can help you in satisfactory management of patient. ACCEPTED MANUSCRIPT Title – A worst case scenario - an undiagnosed, ruptured AVM managed with unconventional ways Abstract Introduction – Treatment of AVMs is, nowadays, performed at specialized RI PT neurosurgical centers, which are well equipped with microscope with green filter, neurosurgical cath-lab and experienced neurosurgeons team. The patients diagnosed with AVMs at smaller hospitals should be referred to such hospitals. But what SC happens when a neurosurgeon has to face the unavoidable worst case scenario, i.e., emergency unplanned surgical excision of undiagnosed, ruptured AVM with large M AN U hematoma. This case report is about management of such case. Case report - A 26 years male was brought to emergency with episodes of seizures and sudden loss of consciousness. His GCS score was E1V1M3. CT head revealed right occipital hematoma with mass effect and herniation. Suspected diagnosis was spontaneous TE D tumor or hypertensive bleed. He had right side anisocoria. Emergency surgery was done. Intraoperatively, it was found to be ruptured AVM. After explaining about nonavailability of conventional instruments (microscope, clips, ICG) to relatives, we EP planned for excision of AVM with available resources. Results and conclusion - AC C Postoperatively patient recovered satisfactorily with right side vision loss. He underwent cranioplasty after 6 weeks. In such case, being patience, following basic steps, transfusion of blood and lateral thinking about using available resources can help you in satisfactory management of patient. ACCEPTED MANUSCRIPT Introduction Treatment of AVMs is, nowadays, performed at specialized neurosurgical centers, which are well equipped with microscope with green filter, neurosurgical cath-lab and 1,2 The patients diagnosed with AVMs at smaller RI PT experienced neurosurgeons team. hospitals, should be referred to such hospitals. But what happens when a neurosurgeon has to face the unavoidable worst case scenario, i.e., emergency M AN U This case report is about management of such case. SC unplanned surgical excision of undiagnosed, ruptured AVM with large hematoma. Case Report A 26 years male was brought with two episodes of seizures, followed by headache, vomiting and sudden loss of consciousness. He was brought around 4 hours later after TE D loss of consciousness. There was past history of intermittent headache and left side focal seizures. On examination, he was in deep coma, having labored breathing. The GCS score was E1V1M3. His right pupil was dilated/non-reactive and left was EP normal size/reactive (anisocoria). The airway was immediately secured by intubation. He was sedated and paralyzed to stop further progression of cerebral edema. NCCT AC C head (Fig 1) revealed right occipital hematoma with mass effect and right side tentorial herniation. The suspected diagnosis was spontaneous tumor or hypertensive bleed. Due to rapid deterioration, urgent decompressive craniotomy and hematoma evacuation was planned. Procedure – Right side large parieto-occipital craniotomy was made. The dura was very tense. After trying measures like head end elevation, mannitol 300ml bolus, furosemide 40mg and hyperventilation, dural tension decreased little bit. As dura was ACCEPTED MANUSCRIPT incised, the brain started bulging out. It was showing tortuous bright red colored vessels over parieto-occipital region suggestive of ruptured AVM. It was a nightmarish situation, as we did not have any preoperative knowledge of feeders, venous drainage, nidus anatomy and proper instruments (non-sticky bipolar cautery, RI PT clips, clip applicator, neurosurgical microscope with green filter, Indocyanine dye and surgical glue). The situation was worsening further as the brain was bulging too much with no pulsations. The idea of large hemispheric decompressive craniotomy might SC have saved us from this situation. But due to this sudden anxious situation, it never came to us. However, we tried leaving dura as such and approximating skin edges to M AN U cover brain surface, so that patient can be referred to higher center, which was fully equipped. The skin was not approximating. The most nearby center was around 4-5 hours drive away and his attendants were not affording it. After discussing every worst possibility, we planned hematoma evacuation and if AVM bled, surgical TE D excision would be attempted. The relatives were explained everything. Informed consent was taken again. EP Before beginning procedure, 2 adult units of whole blood were released and 1-gram trenexamic acid was given intravenously. The blood bank was informed about need of AC C 3-5 units of “O+” whole blood, 4-6 units platelets and 4-6 units fresh frozen plasma (FFP). The hematoma was evacuated via transgyral approach at maximum bulge site. After some time, profuse bright red bleeding started, most probably due to release of pressure or injury to nidus. Due to ongoing blood loss, the plan was changed and it was decided to control feeders first. Blood transfusion was started after around 500ml blood loss. Circumferential resection of cortex was done around tortuous vessels (Fig 2). There were two large feeders at base, which were not getting controlled by bipolar cauterization. We came up with a plan of ligating them with Ligaclip-200 (cystic duct ACCEPTED MANUSCRIPT clips), using laparoscopic cystic duct clip applicator (Fig 3a). Due to clip applicator’s long arm, it became very difficult to clip the feeder. There was no fine control and main surgeon had tremors in hand due to anxiety and long arm instrument without support. After trying for some time, we came with idea of “two hand technique” (Fig RI PT 3b). The assistant was asked to hold handle and apply clip when chief surgeon gave “yes” nod, while chief surgeon held the distal end of arm in his right hand and guided it at right place. The left hand held suction to clear blood and retract surrounding SC brain parenchyma. It helped. But the bleeding was still going on and around 2-liter blood was lost. The patient developed hypotension (systolic blood pressure dropped M AN U to 70mm Hg). This stopped ongoing blood, loss, cleared surgical field and helped in controlling feeders. The visible portion of nidus was excised then. The nidus was thus excised in pieces due to inexperience, no knowledge about its exact size and location. The rest hematoma was evacuated after it. The brain became lax and duroplasty was TE D done using galea. Patient was further transfused 3 units of whole blood, 4 units of fresh frozen plasma and 3 platelets. The anesthetist maintained SBP (systolic BP) around 100mm Hg (except single episode of hypotension) throughout surgery. The EP bone flap was placed in subcutaneous plane of abdominal wall. AC C Postoperatively, patient was kept sedated and paralyzed on Propofol and Vecuronium. Postoperative CT head (Fig 4a) was done after 12 hours, showed significant decompression of hematoma and relieved herniation. Patient GCS was E3VTM5 on day 2 and by day 3, he was following commands, with GCS E3VTM6. He was extubated on day 5 after regaining full consciousness. Gradually he recovered satisfactorily but with some visual deficit (left side homonymous hemianopia, macular sparing). Postoperative CT angiography (Fig 4b) confirmed near total excision of nidus with single abnormal communication of feeder from right posterior ACCEPTED MANUSCRIPT cerebral artery and superficial cortical vein draining into superior sagittal sinus. The choice of excision or embolization of small residue was given, but it was refused due to non-affordability and risk of complications of surgery/embolization. RI PT Follow up - He underwent cranioplasty after 6 weeks (Fig 4c). Currently, he is on regular follow up for last 12 months and doing fine. Repeat CT angiography brain has been planned after 2 years. SC Discussion M AN U Micro-neurosurgical excision of nidus has advanced a lot in last two decades. This is because of development of good diagnostic tools, anatomic knowledge and advances 1 in surgery. Now days, surgical excision of AVM is performed in sequential steps: (1) a large craniotomy is made to obtain adequate exposure to the AVM, including its TE D arterial feeders and venous out flow; (2) after opening dura, first find, isolate and block (clip or coagulate) its arterial feeders; (3) the nidus is resected circumferentially from the adjacent brain parenchyma leaving venous drainage; 4) in the end, venous 1-5 While operating any kind of AVM, these EP drainage is blocked and nidus is resected. basic steps must be followed to minimize surgical complications. That’s why before AC C executing surgical excision, the operating surgeon must thoroughly evaluate AVM anatomy, its relation with brain (eloquent and non-eloquent area, and neurovascular structure) and check surgical equipment. Unplanned surgery can result into disaster. There are two reports about early surgical management (<8hours), without much 3,4 planning, of acutely ruptured AVM presenting with mass effects. Benifla M et al found good outcome in 3 children, who presented with ruptured AVM and mass ACCEPTED MANUSCRIPT 3 3 effect. They used microsurgical technique and followed basic steps. Parvesi G et al reviewed 27 cases of ruptured AVM, who underwent surgical excision and decompression within 6 days (21 patients on first day) and found good outcome in 23 patients.4 Both studies have concluded that single stage excision of ruptured AVM RI PT with mass effect results in good outcome.3,4 These published studies are from those hospitals, which are well equipped. Our case was unique in the sense that it was done at a preliminary center (no microscope, no clips, no glue, no ICG dye facility), by an SC inexperienced surgeon (chief surgeon had assisted only three AVM excision during M AN U training program), with limited resources; keeping one aim in mind i.e. save patient’s life. There was large hematoma causing tentorial herniation. Due to inexperience of seeing sudden ruptured AVM, we never considered it as a possibility. We suspected it to be a TE D tumor, drug abuse or hypertensive bleed. During surgery, it was found to be an AVM. As explained earlier, the conditions were not favorable to refer patient at fully equipped center. The excision was begun following basic excision steps. 1-5 This EP gruesome condition necessitated alternate methods to control blood loss. That’s why AC C we came with idea of using cystic duct clips to control feeders and then “two hand clip technique” to control tremors, while applying clips over large feeders. The anesthetist also plays a vital role in such situation. Induced hypotension and adequate replacement of blood & blood products helped in achieving hemostasis and stability of vitals. Conclusion An undiagnosed spontaneous parenchymal bleed can be due to AVM rupture. The ACCEPTED MANUSCRIPT treating neurosurgeon should keep it as a differential diagnosis and be prepared to face such disastrous condition after doing craniotomy. In a peripheral hospital or limited resources operation theatre, the right way of handling such situation, is to keep your mind calm, use alternate methods like Liga clips/sutures, proceed gradually and RI PT meticulously, follow basic rules of AVM surgery (catch feeders first, then veins), wide resection to control feeders at a safe distance from nidus even if at eloquent area AC C EP TE D M AN U SC location (first goal should be control of bleeders rather than eloquent region). ACCEPTED MANUSCRIPT Acknowledgements – We are grateful to staff Mamta Bishnoi, who assisted the case. Conflict of interests - Nil References - RI PT 1. Derdeyn CP, Zipfel GJ, Albuquerque FC, Cooke DL, Feldmann E, Sheehan JP, Torner JC; American Heart Association Stroke Council. Management of Brain Arteriovenous Malformation: A Scientific Statement for Healthcare SC Professionals from the American Heart Association/American Stroke Association. Stroke 2017;48:e200-224. doi: 10.1161/STR.0000000000000134. M AN U 2. Yamada S, Takagi Y, Nozaki K, Kikuta K, Hashimoto N. Risk factors for subsequent hemorrhage in patients with cerebral arteriovenous malformations. J Neurosurg. 2007;107:965–972. 3. Benifla M, Shelef I, Melamed I, Merkin V, Barrelly R, Cohen A. Urgent removal of ruptured cerebral arteriovenous malformations in children. Harefuah 2010;149:148- 152. TE D 4. Pavesi G, Rustemi O, Berlucchi S, Frigo AC, Gerunda V, Scienza R. Acute surgical removal of low-grade (Spetzler-Martin I-II) bleeding arteriovenous malformations. Surg Neurol. 2009;72:662-667. AC C EP 5. Toyoda K, Anderson CS, Mayer SA. New Insights in Intracerebral Hemorrhage. Front Neurol Neurosci 2016;37:166-181. ACCEPTED MANUSCRIPT Figure 1a - NCCT head, axial cuts, is depicting right parieto-occipital hematoma, reaching till ipsilateral occipital horn and compressing it with ipsilateral transtentorial herniation, 1b – single axial cut, NCCT head is depicting hematoma with midline shift of 12 mm (a). RI PT Figure 2a – Intraoperative image showing edematous brain (red arrow) bulging from “inverted U shaped dural flap” with tortous vessels, 2b – closer view of edematous clear due to hypotension. SC brain with bright red coloured tortous vessel (black arrows), draining veins, 2c - after some nidus excision, the colour of vessels became less bright and little bit blue, draining veins (black arrows), 2d – after excision, a cavity was formed (arrow), but small size of nidus was still present (white dot). It was excised when field became Figure 3a – Laparoscopic cystic duct clip applicator, 3b - “two hand technique” of M AN U clip application over large arterial feeders. Figure 4a – Postoperative NCCT head showing decompressed brain, evacuated hematoma and small residual blood, 4b – CT angiography brain (basal cuts – axial, coronal and sagittal) showing no residual AVM nidus, 4c – 3D CT angiography showing normal architecture of arteries along with clip artifact, 4d – 3D NCCT head, AC C EP TE D after cranioplasty, showing good reconstruction of skull AC C EP TE D M AN U SC RI PT ACCEPTED MANUSCRIPT AC C EP TE D M AN U SC RI PT ACCEPTED MANUSCRIPT AC C EP TE D M AN U SC RI PT ACCEPTED MANUSCRIPT AC C EP TE D M AN U SC RI PT ACCEPTED MANUSCRIPT ACCEPTED MANUSCRIPT Highlights • AVM surgery should be performed in experienced centers with a high case volume. RI PT • Undiagnosed, ruptured AVM can present as hematoma with mass effect, which needs urgent decompressive surgery. AC C EP TE D M AN U alternate ways to save patient’s life. SC • Facing such situation, at an underdeveloped hospital, necessitates