Volume 2 - Issue 10
Dr. Vasanthi Vidyasagaran*
Department of Anaesthesiology, Kauvery Hospital, Chennai, Tamilnadu, India
An 18-year-old boy with facial deformity, presented for cosmetic correction. There was also a huge convoluted mass arising from the scalp, covering the lateral aspect of his forehead. He was diagnosed as a case of Cutis Verticis Gyrata, though his features were not very typical of the disease.
His left ear was also enlarged. On examination, he had irregular teeth and a protruding maxilla. There was significant restriction of neck movements. Difficult airway in terms of bag mask ventilation as well as intubation was anticipated.
On auscultation of the chest, normal vesicular breath sounds were heard on the right side, but diminished breath sounds on the left side. Palpation of the spine confirmed a right kyphoscoliosis along with thickened skin all over the back on the left hemithorax.
The diminished breath sounds were probably a result of the diffuse swelling over his thorax, as well as the kyphoscoliosis. His lung function tests confirmed restrictive lung disease. He was given chest physiotherapy, with deep breathing exercise to improve lung function for one week prior to surgery.
His preoperative oxygen saturation at room air was 93%. After adequate premedication with inj. Tramadol 50 mg, inj. Glycopyrrolate 0.2 mg IM, Dexamethasone 8 mg, Ranitidine and Ondansetron, IV he was wheeled into the operating theatre. Anaesthesia was induced with Propofol 120 mg and Suxamethonium 75 mgs, and he was intubated Size 7 oral RAE tube.
Laryngoscopy was difficult due to limited neck extension and protruding teeth. Baseline ventilator pressures were noted and intraoperative lung compliance and pressures were constantly monitored to avoid barotrauma. Oxygen saturation of 95-96% on inspired oxygen concentration of 50% was accepted. Neuromuscular monitoring was used to guide dosage of muscle relaxants, as residual neuromuscular blockade at end of surgery may have serious consequences in these patients.
Surgery involving excision of tumour on forehead and scalp, ear reconstruction and cheek repair was done in approximately 5 hours. He was appropriately hydrated and monitored. The patient was disconnected from the ventilator and manually ventilated intermittently to keep a check on lung compliance. At the end of the procedure, patient was reversed and extubated making sure that he was fully conscious, and his respiratory efforts were adequate. Post extubation, saturation dropped to 90% which improved with supplemental oxygen. Patient recovered uneventfully.
Now we have a patient who has a potentially difficult airway where securing the airway may be a problem, and restrictive lung disease. (upper and lower airway involved).
He also had a rare disease, and the surgical procedure was expected to be difficult. Surgeons from various specialities were involved and the procedure lasted for over four hours. Intense monitoring was required throughout the procedure.
Cutis verticis gyrata typically affects the vertex and occipital region, but some forms can involve the entire scalp. The cause is unknown in primary cases, but it could have a genetic or endocrine factor. Secondary cases may be due to inflammatory dermatoses. It is common among mentally retarded patients.
Restrictive pulmonary diseases are characterized by decreased lung compliance. Lung volumes are typically reduced, with preservation of normal expiratory flow rates. Thus, both Forced Expiratory Volume in 1s (FEV1) and forced vital capacity (FVC) are reduced, but the FEV1/FVC ratio is normal.
There may be intrinsic lung diseases, such as pulmonary fibrosis related to rheumatoid arthritis or asbestosis, or extrinsic, caused by kyphoscoliosis or obesity. The key feature is impaired oxygenation at alveolar level.
Management of such patients must be well planned at all levels.
Postoperative outcomes in these patients are largely influenced by careful planning and systematic management of perioperative care.
A 16-year-old girl was being evaluated for bi-maxillary osteotomy.
She was withdrawn and was not very communicative. It was obvious that she had productive cough as she was coughing even while trying to talk for a few minutes.
On examination, she was thin built. Chest auscultation revealed bilateral coarse breath sounds typical of bronchiectasis. Her heart sounds were heard on the right side. Cardiac and respiratory investigations were requested. Diagnosis of Kartageners syndrome was made and physician consultation was advised. Preoperative optimization was done with postural drainage of secretions, antibiotics, mucolytic, and bronchodilators. Surgery was scheduled 10 days later. Patient insisted on having surgery done, despite the risks involved.
Safe anaesthesia was planned and administered. Short acting anaesthetic agents were used. Airway was secured with nasal cuffed endotracheal tube. Ventilation strategy was modified to allow for prolonged expiration. Care was taken not to hyperventilate, as patient's baseline end tidal CO2 values was around 50 mmHg. Humidified gases were used. Tracheal toileting was done intermittently to ensure no clogging of mucous plugs. Prior to extubation, tracheal toileting was done again, reversal administered, regular breathing pattern ensured and then extubated.
Key points to highlight in this case are:
A young unmarried girl aged 19 years presented to emergency room around 11 PM with severe right iliac fossa pain, worsening over last few hours. She looked very pale, afebrile and had tachycardia, pulse rate - 130/minute, Hb was 7gms%. Ultrasound was not available in that place, but, the surgeon was sure of his diagnosis as acute appendicitis requiring emergency surgery.
The Anaesthetist who examined the patient raised doubts about it being a ruptured ectopic pregnancy, due to patient's clinical presentation with severe tachycardia and generalised pallor, with no rise in temperature. Hence, blood grouping and typing was done and four units' blood made available. Questioning the patient in the presence of family can be tricky, and she refused to answer any questions. Urine pregnancy test could not be done as a kit was not available. She required emergency laparotomy anyway, and wheeled into emergency theatre...
General anaesthesia was induced. An obstetrician was alerted, and she was on her way, whilst the general surgeon started the laparotomy. There was hemoperitoneum. It was indeed a ruptured ectopic! The surgeon clamped the bleeding fallopian tube. Intravenous fluids one litre, followed by 2 units packed red cells were administered. The obstetrician joined in soon and secured haemostasis. Other fallopian tube and ovary looked healthy and were left behind. Patient recovered well.
When there is an emergency, a quick overall assessment of the patient is important. As much as one should focus on the main complaint, there should be no rigid diagnosis, especially in a case of acute abdomen. This will aid further plan of action.
As an anaesthetist, we cater to all surgical specialities which is a unique feature and advantage of our speciality. The anaesthetist could diagnose the condition as she was experienced in obstetric anaesthesia and seen more number of ruptured ectopic than this young surgeon. The anaesthetist was prepared with adequate blood even before start of anaesthesia and that saved the patient who was already quite anaemic.
This highlights the importance of good communication and rapport between surgeon and anaesthetist. We do come across situations like these, where diagnosis and surgical decision including timing of surgery have to be discussed and appropriate decisions be made. The patient is the most important member of the team and any difference of opinion should be sorted out only after the patient is safely shifted out of theatre.