Case history:
A 40 year old underprivileged conjoined twin (Mr. A and Mr. B) (Pygopagus) presented to local hospital (200 km distant from our center, which is a tertiary health care facility) with high grade fever (102 degrees), worsening dyspnea, cough and severe fatigue for past three days. The patient’s primary treatment was delayed due to transportation difficulties during the lockdown, as well as shame and reluctance on the part of family members to accompany them. The patients were managed by telemedicine video call from our center. As they presented primarily with respiratory symptoms at the time of second wave of COVID-19 pandemic, a presumptive diagnosis of COVID-19 infection was made. Nasopharyngeal swabs of both the patients were sent for testing of SARS CoV-2 virus. Both of them had positive reports with CT (Cycle threshold) value of 13 and 12 respectively. The conjoined twins (Patient A) were already taking treatment from neurosurgery outpatient department of our center for dorsal spine tuberculosis with paraparesis for past 1 year. Mr. A was on antitubercular medications for past 1 year. He was provided with custom fitted kyphotic brace made from a cast on his cervico – dorsal spine. They were kept on a regular follow-up through telemedicine due to ongoing Covid-19 pandemic and they were embarrassed to travel large distances for medical appointments. They also used to avoid crowded places since everyone would look at them as if they were aliens. Therefore, video consultations were provided and routine liver function test were shared on our whatsapp group. They did not have any other significant medical histories. However, they had a very poor hygiene due to their low socioeconomic status (Figure 1. A-C) .
After discussion with the treating team at local hospital, it was advised to shift them to our tertiary health center. On arrival, patient was immediately shifted to ICU on two combined beds. They were connected to high flow nasal cannula (60 litres/min), and were maintaining an oxygen saturation of 80- 84 % which was not adequate. Arterial blood gas analysis was done and it was observed that PaO2/FiO2 ratio of both patients (Mr. A and Mr. B) were between 70-80. They had metabolic acidosis with high lactate and low bicarbonate (Table 1). As they were not maintaining adequate oxygen saturation, had very high respiratory rate along with worsening of ventilatory parameters, they were intubated and taken to mechanical ventilation. Intubation was done for each of them in lateral position. Two separate mechanical ventilators (Hamilton-medical) were kept and VCV (volume controlled ventilation) mode with FiO2 80 to 90 % and PEEP 6 to target a saturation of 94% was initiated. Deep sedation targeting Richmond Agitation Sedation Scale of -4 was initiated using titrated doses of fentanyl at the rate of 25 to 150 mic/hour and midazolam infusion at the rate of 1-3 mg/hour. But as persistent asynchrony was present with desaturation, muscle relaxant infusion with vecuronium 2 mg/hr was initiated. The airway tubing and intravenous lines of each patient were color coded to avoid confusion and drug errors. We could not place central neck line in them as it was quite difficult to insert due to their position. Instead, peripherally inserted central line (PICC) was placed. To confirm absence of cross circulation between them via the sacral venous plexus, atropine was given to patient A, he developed tachycardia but patient B had normal pulse rate ensuring absence of any cross circulation4.After confirmation of absence of any cross-circulation, anti-covid medications were started as per their individual estimated body weight.
Bedside Chest X ray was done which showed multiple infiltrates, linear and ground glass opacities in both the lungs with consolidation in right lung of patient A. Patient B had consolidation in bilateral lung fields, suggestive of severe ARDS (Figure 2 A and B). His biochemical parameters were suggestive of cytokine storm. There was significant leukocytosis with neutrophillia. His renal function and serum electrolytes were normal. CRP and ferritin was highly elevated suggestive of inflammatory cycle. Procalcitonin and D-dimer were markedly raised suggesting ongoing septicemia and thrombosis. However, liver function and lipid profile were normal. Lactate dehydrogenase (LDH) and Interleukin-6 (IL-6) were significantly raised in both the patients suggestive of severe covid-19 infection. Cardiac profile, myocardial Creatinine phospho kinase (CPK-MB) and Troponin-I were normal. Electrocardiogram showed normal sinus rhythm. Echocardiogram didn’t show any valvular malfunction or defect.
Inj. methyl prednisolone (pulse dose) 500 mg once in a day were given to both of them separately for three consecutive days. Low molecular weight heparin was also started to both the patients at a dose of 60 mg twice a day along with intravenous injections of piperacillin and tazobactum (4.5 gm) thrice a day. Injection toclizumab was also given to both of them. For next three days, both of them were kept on maximum ventilator support (FiO2 – 100%, PCV mode, Pi-30, PEEP – 18 cmH2O). We changed the mode to pressure control mode to prevent any barotrauma arising out of increasing airway pressures. Over the period, the compliance gradually decreased with increasing plateau pressure (around 40-50) (Figure 3A). However, despite providing ventilatory support their oxygen saturation was not adequate (SPO2- 85 %). However due to conjoint sacroiliac junction, they would always maintain obliquity in their posture (lateral position). It was nearly impossible to provide them ventilation in prone position in spite of being in moderate to severe ARDS. Gradually their saturation level dropped down from 85% to 30-40% in next three days (Figure 3B) with 100 % FiO2 and PCV Mode, Pi 38 and 18 PEEP and unfortunately they suffered cardiac arrest. Patient B expired on day 3 of admission and patient A expired on day 4 of admission after 14 hours of gap. The hemodynamic parameters were stable until the last day of treatment before patient A expired.