Anaesthesia for a paediatric patient with Rosai–Dorfman disease

Abstract

Editor—Rosai–Dorfman disease is a histiocytic proliferative idiopathic disorder1Rosai J Dorfman RF Sinus histiocytosis with massive lymphadenopathy: a newly recognized benign clinicopathological entity.Arch Pathol. 1969; 87: 63-70PubMed Google Scholar characterized by the presence of sinus histiocytosis with lymphadenopathy, and benign haematopoietic and fibrous proliferation in the head and neck. Extranodal involvement is rare but may occasionally affect the central nervous system.2Bhandari A Patel PR Patel MP Extranodal Rosai–Dorfman disease with multiple spinal lesions: a rare presentation.Surg Neurol. 2006; 65: 308-311Abstract Full Text Full Text PDF PubMed Scopus (32) Google Scholar Prednisolone is used to control the disease,3Valladares Otero G Pérez Ferrer A Gredilla Díaz E et al.Perioperative respiratory complications in pediatric patients with mediastinal masses: a report of 2 cases.Rev Esp Anestesiol Reanim. 2006; 53: 657-660PubMed Google Scholar but surgery is indicated to relieve symptoms of compression or for biopsy. Possible anaesthetic problems include acute airway obstruction, superior cava vena syndrome (SCVS), cardiac or mediastinal compression of the pulmonary artery, acute pulmonary oedema, or cardiopulmonary collapse. An inhalation technique may be elective with maintenance of spontaneous ventilation, avoiding the use of neuromuscular blocking agents. We report the case of a patient with Rosai–Dorfman disease undergoing surgery for excision of a preauricular adenopathy. A 21-month-old (13 kg) child required removal and biopsy of right preauricular lymphadenopathy under general anaesthesia. She had been diagnosed with Rosai–Dorfman disease at 15 months old (positive for S-100 protein), but was not on regular treatment. Preoperative evaluation showed cervical lymphadenopathy and firm, painless preauricular nodes. Previous tomographic study identified several paratracheal nodes with tracheal compression and airway displacement. The patient was premedicated with atropine 0.01 mg kg−1. Anaesthetic induction was performed by tidal breathing of sevoflurane 7% in a mixture of oxygen and air (inspired oxygen fraction, 40%) resulting in uneventful tracheal intubation after 5 min with SPO2 92%. Anaesthesia was maintained with spontaneous ventilation through Mapleson C system with mixed O2/air 40/60% and sevoflurane 0.9 MAC. Dexamethasone 0.15 mg kg−1 and metamizol 30 mg kg−1 were given. Surgery proceeded uneventfully, and the trachea was extubated on awakening. In the post-anaesthesia care unit, the patient developed one episode of dyspnoea with desaturation, due to partial airway obstruction, which required manual ventilation with O2 100%, but not tracheal intubation. Her stay in ICU for the first 24 h was uneventful. It is essential, in preoperative evaluation of Rosai–Dorfman disease, to establish the degree of organ involvement. Classical clinical presentation, along with painless cervical lymphadenopathy, associates with nasal obstruction, adenoidal hypertrophy, fever, neutrophilia, and elevated erythrocyte sedimentation rate, with polyclonal hypergammaglobulinaemia. The diagnosis requires histopathological and immunohistochemical confirmation. Assessment of the respiratory system should include checking for extrinsic airway compression (in X-ray studies) and obstructive or restrictive pattern on lung function tests, reflecting the presence of mediastinal masses.3Valladares Otero G Pérez Ferrer A Gredilla Díaz E et al.Perioperative respiratory complications in pediatric patients with mediastinal masses: a report of 2 cases.Rev Esp Anestesiol Reanim. 2006; 53: 657-660PubMed Google Scholar Anxiolytic premedication should be avoided, and i.v. access using the lower limb is preferred in SCVS. Neuromuscular blocking agents may produce airway collapse and should be avoided. Inhalation anaesthesia with spontaneous ventilation is strongly recommended. An LMA may be a useful option. Thoracic epidural block is an option due to its advantages over i.v. analgesia.4Soliman LM Mossad EB Thoracic epidural catheter in the management of a child with an anterior mediastinal mass: a case report and literature review.Paediatr Anaesth. 2006; 16: 200-205Crossref PubMed Scopus (12) Google Scholar Adequate preoperative evaluation of potential cardiovascular or respiratory obstructive symptoms is essential in these patients. None declared.