A Novel Technique for Correction of Unilateral Craniofacial Fibrous Dysplasia using Multiplanar Sequential Cutting Guides

 

Fibrous dysplasia is a benign bone lesion characterised by replacement of normal bone with immature fibro-osseous connective tissue.

 

The craniofacial region is one of the most affected sites. Usually, craniofacial fibrous dysplasia presents as a unilateral painless swelling. But, sometimes the lesion can involve vital structures, such as the optic nerve and the auditory canal, causing functional deficits. The presence, or absence, of functional deficits, skeletal maturation and the age of the patient significantly affect the course of its management.

 

The surgical management of craniofacial fibrous dysplasia in the absence of functional deficits ranges from the use of conservative techniques to radical resection of the lesion. Conservative techniques include debulking and contouring of the diseased bone to best match the contralateral side. However, this technique has limitations as there is no definitive way to evaluate the exact bone mass that needs
to be removed to attain facial symmetry.

 

Craniofacial resection is a radical approach that offers higher precision. With the help of virtual surgical planning and mirroring technique, we can determine the amount of excess bone that needs to be shaved off by mirroring the normal side onto the affected side in case of unilateral craniofacial fibrous dysplasia. This pre-operative plan can be executed intraoperatively either by using CT-guided navigation during bone contouring, or by designing 3D-printed passive surgical guides that help determine the limit of resection.

 

Both these techniques have their inherent limitations, and are subject to intra-operative errors and inaccuracies.

In this case study, we discuss this new method of computer-guided contouring for unilateral craniofacial fibrous dysplasia. We describe the new technique that uses sequential cutting guides to perform osteotomies in multiple planes to match the anatomical contours of the unaffected contralateral side.

 

Case Study

 

A 28-year-old male patient presented with a left-sided facial swelling since birth that was gradually increasing in size.

 

There was no history of pain, ulceration, or any associated functional deficit. On examination, a diffuse, bony swelling was found involving the left cheek and extending inferiorly till the mandible. CT revealed left-sided
fibrous dysplasia involving the zygoma and hemi-mandible.

 

Post investigation, debulking and recontouring of the left zygoma was planned to improve the facial aesthetic, and mandibular recontouring was planned for stage 2 of the surgery.

 

Pre-operative virtual surgical planning

 

High-resolution CT scan of the skull was obtained with a slice thickness of 0.6mm to 0.75mm. This DICOM (Digital Imaging and Communications in Medicine) data was then transferred into a segmentation software – inPrint 3.0 | Materialise. The region of interest was segmented and converted into workable stereolithographic models.

 

The cutting guides were designed using a free and open-source software - Blender 2.7. We created a mid-sagittal plane, and the 3D model of the skull was cut into two equal halves to separate the disease side from the normal side. The normal side was mirrored onto the disease side, and the amount and the extent of resection needed was evaluated. Based on this evaluation, multiplanar sequential cutting guides were designed. These guides, along with the model of the skull, were printed in 3D using fused deposition modelling. All these items were sterilised using ethylene oxide a day before the surgery.

 

Surgical steps

 

A bicoronal approach with left sided preauricular extension along with left upper gingivo-buccal sulcus approach was used to expose the diseased bone. 3D printed cutting guides were applied one after the other and multiplanar osteotomies were performed using a sagittal saw to remove the excess bone. Any sharp bony margins were smoothened with a bur. A calvarial bone graft was harvested to reconstruct the excised zygomatic arch and was fixed at its new position with a long spanning titanium plate. Then, midface soft tissue suspension was done with prolene sutures. Ensuring haemostasis, wound closure was performed in layers. The post-operative course was uneventful with optimal clinical outcome. One-year post-operative photograph of the patient shows excellent facial symmetry.

 

Dr Sanjay Mahendru
Director
Department of Plastic, Aesthetic and Reconstructive Surgery,
Medanta - Gurugram

 

Dr Rahul Jain
Consultant
Department of Plastic, Aesthetic and Reconstructive Surgery,
Medanta - Gurugram

 

 

Cardiac Myxoma Presented as Paraplegia

 

Myxomas are the most common primary cardiac tumours. Majority are found in the left atrium and are attached to the interatrial septum. Clinical features of a myxoma encompass a triad of arterial embolism, obstruction of intracardiac blood flow, and constitutional signs, including fatigue, fever, weight loss, and elevated CRP.

 

Embolism affects 30%-40% patients with myxoma, with cerebral arteries being the most common destination followed by the coronary, renal, mesenteric, and peripheral arteries. Symptoms related to peripheral embolism are experienced in 2%-15% cases. Cases of mesenteric embolism are extremely rare, and only a few case reports are available.

 

Case Study

 

A 24-year-old male presented to the Emergency with pain in the back and lower limbs a day after suddenly falling at home because of bilateral lower limb weakness. He was evaluated at other centres and referred to Medanta Hospital, Lukcnow, 24 hours later after he had lost all sensation below the umbilicus – 0/5 motor power in the right lower limb and 1/5 motor power in the left lower limb, cold peripheries, and bowel-bladder incontinence. He was also experiencing dyspnoea since the fall.

The investigations revealed high leucocyte count with raised neutrophils; the renal function was within the normal range. His bedside 2D ECHO showed myxomatous frond-like mass in the left atrium with attachment to interatrial septum moving to and fro to the left ventricle via the mitral valve. While the right and the left heart function was normal, the CT angiogram showed ill-defined heterogeneously enhancing hypodense lesion within the left atrium extending to the left ventricle, suggestive of a myxoma.

 

CT angiogram suggested intraluminal thrombus causing near-total occlusion of the infrarenal abdominal aorta and bilateral proximal common iliac arteries with the length of the segment being about 8cm. The scan also showed extension of the thrombus to the origination point of the left renal artery with infarcts in left kidney, and segmental infarct in right renal artery.

Another finding was the complete occlusion of the distal superior mesenteric artery and at the origin of the inferior mesenteric artery causing infarcts in the spleen. The CT angiogram also revealed a thrombus causing total occlusion of bilateral anterior tibial arteries and peroneal arteries.

 

We arrived at a diagnosis of left atrial myxoma with acute bilateral lower limb ischemia. The patient was started on intravenous heparin sodium and taken for emergency surgery.

Intra-operative transesophageal echocardiogram (TEE) confirmed the finding. Hence, we first removed myxomatous tissue from bilateral femoral arteries and the aorta by performing a proximal and distal embolectomy (via bilateral femoral access) to minimise ischemia time. Patient was then placed on cardiopulmonary bypass, and we removed the frond-like left atrial myxoma attached to the interatrial septum which measured about 5x5 cm.

 

In the post-operative period, the patient gradually recovered from right lower limb palsy and his sensory function was restored over the next two days. A residual foot drop remained requiring a splint. Post-operative day 1, left lower limb sensory and motor power was recovered. Over the next two-three days, patient’s elevated creatinine level normalised with adequate urine output. Total leukocyte count also normalised on day one after the operation. He complained of mild abdominal pain with faecal incontinence from which he recovered in a couple of days. Post-operative day four, the patient tolerated the feed well, passed stool and urine normally, and was mobilised. He was discharged on day five on Acitrom.

 

During the post-operative follow up after a week, the patient did not complain of any symptoms. He was mobile, with significant improvement in the right-sided foot drop.

The patient had an unremarkable recovery even though he was presented to us much after the golden period of six hours had passed. This case emphasises the importance of early referral and timely intervention that can save patients’ limb and life.

 

Dr Gauranga Majumdar
Director - Cardiothoracic and Vascular Surgery
Cardiac Surgery, Heart Institute
Medanta - Lucknow

 

Acute Aortic Dissection in a Patient with COVID-19 Pneumonia Infection

 

Acute aortic dissection is one of the few cardiac emergencies that require urgent medical attention. In this condition, blood traverses within layers of the aortic wall and creates a false lumen. Depending on the site and the extent of involvement of the aorta, dissection is divided into Stanford Type A (involving ascending aorta) and Stanford Type B
(involving descending thoracic or thoracoabdominal aorta distal to the left subclavian artery).

This classification plays an important role in deciding the course of management. Acute Type A aortic dissection requires emergency surgical intervention as 50% patients die within the first two days if not treated surgically. However, when an aortic dissection is detected early and treated promptly the chance of survival improves significantly.

 

Case Study

A 45-year-old female suffering from hypertension, and diabetes – on irregular medication – was presented to the Emergency with cough and chest pain radiating to her back since that morning. Patient was evaluated thoroughly, and all routine investigations were done immediately. Her ECHO test result showed mild concentric left ventricular hypertrophy (LVH), mild aortic regurgitation (AR) with a flap in the ascending aorta, partially thrombosed false lumen and mild to moderate pericardial effusion.

 

The investigations were suggestive of a dilated ascending aorta, showing an aortic aneurysm of 4.2cm. Further, the patient underwent a CT angiography that revealed she was suffering from acute Type A aortic dissection with partially thrombosed false lumen in the arch, and a descending thoracic aneurysm (DTA). All branches were from the true lumen.

 

The case was further complicated by the fact that the patient was found COVID-19-positive. Her chest CT scan showed evidence of mild Covid pneumonitis, and her CO-RAD score was 5. But she was maintaining acceptable oxygen saturation on nasal prongs with a flow rate of 2 lit/min and her routine investigations were also within normal limits.

 

Hence, she was taken for emergency surgery following strict Covid protocols. Under local anaesthesia, right radial artery (20G) and left femoral artery (16G) were cannulated for monitoring. General anaesthesia was induced later. Then, an 8.5F sheath was placed along with a triple-lumen central venous catheter through the right internal jugular vein. First, the axillary artery was exposed for arterial return followed by a sternotomy. During the surgery, a haemorrhagic pericardial effusion, a dilated and dissected ascending aorta, along with a haematoma and a tear was observed. However, there was no tear noticed in the arch. We reconstructed the aortic root with resuspension of the aortic valve at the sinotubular junction. A 24mm interposition dacron graft was anastomosed. The ascending aorta was replaced with hemiarch under hypothermic circulatory arrest, and antegrade cerebral perfusion was done after reconstructing the arch.

 

During the surgery, 5 units of packed red blood cells (PRBC) and 4 units of random donor platelet concentrate (RDPC) were transfused.

 

Post operatively, the patient was shifted to negative-suction isolation room in the ICU. On the post-op day, she did not require inotropic support and was passing adequate urine. Hence, she was weaned off the ventilator on the same day.

 

Pre-operatively, her C-reactive protein (CRP) and lactate dehydrogenase (LDH) were markedly raised with mildly deranged liver function test (LFT) and serum ferritin; D-dimer was within normal range. Arterial blood gas (ABG) was showing PO2 of 58 mmHg with SPO2 93% on 2 litres of supplemental oxygen through nasal cannula. On post-op
day four, chest CT scan was repeated, and it did not show
any further deterioration; the CRP level had also started
to normalise.

 

Patient stayed in the ICU for six days and was shifted to ward on day seven after the surgery. Post-operative ECHO showed normal LV function with aortic conduit in situ. She was discharged from the hospital on post-op day 10 in a stable condition. Perioperatively, she was put on dexamethasone that was later tapered down and stopped after her first follow-up visit to the OPD.

A month after her discharge, the patient is doing well.

 

Dr Sanjay Kumar
Director - CTVS
Cardiac Surgery, Heart Institute
Medanta - Patna

 

Dr Rajeev Ranjan
Director - Cardiovascular Anesthesia and CTVS ICU
Institute of Critical Care and Anaesthesiology
Medanta - Patna

 

 

Total Body Irradiation via Tomotherapy

 

Total body irradiation (TBI) is a radiotherapy technique used to deliver radiation to the entire body. It is mostly used in conjunction with chemotherapy as a preparative regimen for haematopoietic stem cell transplant (HSCT). It has proven benefits for eradicating residual malignant or genetically disordered cells for ablating haematopoietic stem cells, and for immunosuppression to reduce the risk of graft rejection.

 

TBI provides a uniform dose of radiation to the whole body, penetrating areas such as the central nervous system (CNS) and testes, where traditional chemotherapy is ineffective. Additionally, it allows tailoring of therapy with the ability to shield or boost the dose to certain regions as necessary.

 

The optimal regimen depends on a range of clinical variables, including patient age, disease, and type of HSCT. With competing goals of disease eradication and avoidance of toxicity, the most accepted total dose of TBI for myeloablative HSCT is 12-15 Gy delivered in 6 to 12 fractions over three to five days. Patients with comorbidities and elderly cohort, not suitable for myeloablative regimen, are treated with non-myeloablative regimen of 2-4 Gy in one session. The above fractionation results in increased survival for about 10% patients in addition to chemotherapy.

TBI is primarily limited by the toxicity to critical organs, especially lung, eyes, heart, liver, and kidneys. Multiple techniques are used to deliver TBI - standing or lateral - with each technique presenting its own pros and cons, such as standing for a long time during treatment without moving.

 

The introduction of helical tomotherapy offers a new potential to address both these issues. Helical tomotherapy helps conform the dose to very specific areas of the body, sparing critical organs. It also helps give treatment to the patient in supine position, which is much more comfortable. Gives us the capability to allow marrow-only or marrow-plus nodal irradiation so that higher dose can be delivered while sparing critical structure, thus resulting in high cure rates, longer life expectancy and less morbidity.

 

Medanta, Gurugram, started the TBI program in 2012 using the ‘Moving Junction Technique’ with 4 MV photon beam, SSD (source to skin distance) ranging 125-135cm, field size 40cmx40cm three or four splits, AP/PA (antero-posterior) beams.

 

The major challenges included dose inhomogeneity, organ shielding, specific site dose escalation (brain, testes, mediastinal nodes etc.), difficulties during anaesthesia for non-cooperative or paediatric patients and many other operational issues.

 

After commissioning our Tomotherapy-H machine in 2016 we have migrated all our TBI treatments on it as it addresses all the challenges faced. Any dose escalation or shielding, if needed, can be done in one treatment plan and delivered during the same setup. Till date, we have treated, more than 175 patients using the Tomotherapy-H machine.

 

Dr Tejinder Kataria
Chairperson
Division of Radiation Oncology, Cancer Institute
Medanta - Gurugram

 

Dr Deepak Gupta
Senior Consultant
Division of Radiation Oncology, Cancer Institute
Medanta - Gurugram

 

Medanta Brings World-class, Specialised Women and Childcare Facilities to Lucknow

 

Launches Institute of Obstetrics and Gynaecology, Fetal Medicine, Infertility and Gynaecological Onology, and Division of Neonatology

 

Eminent high-risk obstetrics and gynaecology expert with over 30 years of experience Dr Neelam Vinay has been appointed as the Director of the Institute of Obstetrics and Gynaecology, Fetal Medicine, Infertility and Gynaecological Oncology, and the Division of Neonatology at Medanta-Lucknow. With this, world-class, affordable, comprehensive, healthcare for woman and children has been brought under one roof in the city.

 

Women of all ages - right from the onset of puberty with menarche to pregnancy to menopause and beyond - will get round-the-clock access to comprehensive treatment and state-of-the-art infrastructure and facilities here. The hospital is equipped to provide complete obstetrics care, handle pregnancies, including high-risk cases, and health complications of newborn children.

 

“We specialise in treating all gynecological ailments, including disorders such as irregular menstruation, problems faced by adolescent girls, tumours, uterine fibroids, and uterine prolapse. We have specialised clinics for gynaecological cancers such as ovarian, endometrial and cervical cancers. Soon, we will also launch an IVF facility for infertility treatment. Women in Lucknow will not have to go outside the city for any gynecological needs,” Dr Neelam Vinay said.  The hospital has advanced ICU and HDU (high dependency unit) for critically ill women supported by a specialised, 24x7 blood bank.

 

The institute will not only specialise in caring for pregnant women, offering all the specialised ultrasound scans, but will also be medically and technologically equipped to diagnose and treat complex fetal problems, and invasive fetal procedures.

Dr  Aakash Pandita, Senior Consultant, Division of Neonatology, who will head the Neonatal services, said that the Level-3 NICU with neonatal transport facilities is a first-of-its-kind in the state.

 

The NICU at Medanta - Lucknow will offer all the latest facilities required to treat the sickest of babies. Some of these facilities will be available for the first time in the city, such as volume-targeted ventilation, high-frequency ventilation, point-of-care ultrasound, echocardiography, therapeutic hypothermia, inhaled nitric oxide and total parenteral nutrition. It will also promote developmental supportive care to newborn babies such as day-night light cycling and facilities for kangaroo mother care.

 

“The world-class Level-3 NICU will care for extremely small preterm babies weighing less than 1 kg. It will offer transport incubators, doubled-wall incubators, and non-invasive ventilation techniques such as non-invasive mechanical ventilation (NIMV), continuous positive airway pressure (CPAP) and heated humidified high-flow nasal cannula  (HHHFNC),” Dr Pandita said, adding that the Division will also do non-invasive surfactant technique SURE (surfactant without endotracheal tube intubation), which he is credited with starting in India.

 

The neonatal team will be supported by ancillary care providers from paediatric cardiology, paediatric gastroenterology, haematology, cardiac thoracic surgeons and CTVS.

 

Dr Neelam Vinay
Director

Obstetrics and Gynaecology, Fetal Medicine, Reproductive, Medicine and Gynaecological Oncology, Lucknow

 

Gynaecologist with over 30 years of experience in high-risk obstetrics, advanced laparoscopic and hysteroscopic surgeries, advanced management of infertility and transvaginal sonography. She also specialises in handling complex gynaecological surgeries, including gynaecological oncology.

 

Dr Aakash Pandita
Senior Consultant

Neonatology, Lucknow

Paediatrician with expertise in high-risk neonatal, perinatal and paediatric care. He is trained in evidence-based neonatology with over 10 years  of experience in Level-3 NICU care and management of sick neonates.
He has extensive experience in handling babies weighing less than 1,000 grams.

 

Dr Pratibha Dhiman
Senior Consultant

Medical and Haemato Oncology, Gurugram

Medical and Haemato Oncologist with extensive experience in treating both benign and malignant haematological diseases. She also has expertise in performing bone marrow transplants, including haploidentical and matched unrelated transplants. She has performed successful transplants for leukemia, lymphoma, multiple myeloma, and multiple sclerosis.