Correspondence Address:
Dr. Sunit Kumar Gupta
Department of Anesthesiology and Critical Care, All India Institute of Medical Sciences, Jodhpur - 342 005, Rajasthan
India

Full Text



Sir,

Prune-belly syndrome is a rare congenital disease, with a characteristic triad of abdominal muscle deficiency with dried plum appearance, urinary tract abnormality, and cryptorchidism.[1],[2] These patients can have cardiovascular and musculoskeletal abnormalities and mental retardation. They may also have respiratory involvement in the form of aplasia and hypoplasia of the lungs, flaring of the ribs, and narrowing in transverse diameter of the chest leading to inability to cough, recurrent respiratory tract infections, and pneumonia.

We report an 18-day-old male patient, weighing 3 kg, a diagnosed case of prune-belly syndrome and who received anesthesia twice under our care. He had lax abdominal muscles and loose skin with dried plum appearance [Figure 1]. He also had syndactyly in bilateral lower limbs without any chest wall deformity. There was no obvious airway or facial abnormality. The patient had a history of lower respiratory tract infection and was being treated with antibiotics. He had posterior urethral valves with vesicoureteric reflux leading to megalourethra and bilateral hydroureteronephrosis. Laboratory investigations including renal function tests were within normal limits. Echocardiography did not reveal any atrial/ventricular septal defects or patent ductus arteriosus.{Figure 1}

The patient underwent cystoscopy with laryngeal mask airway (LMA) after sevoflurane induction and fentanyl 6 mcg with assisted respiration without muscle relaxant, which was uneventful. After 2 weeks, the patient was scheduled for corrective surgery. This time, we secured the airway with orotracheal uncuffed tube of size 3 mm internal diameter (ID) in view of prolonged duration of surgery, after inhalation induction with sevoflurane, fentanyl 6 mcg, and 1.5 mg atracurium. Caudal block was also given with 3 ml of 0.2% ropivacaine for pain relief. Surgery was uneventful. Postextubation, the child developed laryngospasm, which was relieved with continuous positive airway pressure and Larson maneuver. His postoperative stay in the hospital was uneventful.

The children of prune-belly syndrome have delayed developmental milestones. Vesicoureteric reflux, hydronephrosis, urinary tract infection, congenital renal dysplasia, or hypoplasia may manifest as chronic renal failure or sometimes end-stage renal disease on dialysis or renal transplant.[1] Congenital chest deformities or scoliosis lead to lung hypoplasia and pulmonary dysfunction. Congenital cardiac defects may need antibiotic prophylaxis for infective endocarditis.[2]

Preparation for anesthetic management for these patients should start in the preoperative period. Anesthesiologists need to rule out any renal and pulmonary dysfunction and cardiac abnormalities. Any preexisting chest infections should be treated with antibiotics. Communication between pediatrician, pediatric surgeon, cardiologist, and anesthesiologist is of paramount importance. For airway management, LMA should be preferred over endotracheal tube to avoid muscle relaxants and decrease chances of airway stimulation.[3],[4] Regional anesthesia should be preferred, wherever feasible.[3],[4] Regional techniques for pain relief such as caudal, epidural, or peripheral nerve block may expedite recovery from anesthesia. The use of short-acting anesthetics such as sevoflurane, atracurium, and remifentanil can also hasten recovery from anesthesia. Judicious fluid administration and possible route of elimination of anesthetics should be kept in mind. Due precautions should be taken for laryngospasm and bronchospasm.[3],[4] Poorly developed abdominal wall muscles along with flat diaphragm lead to pooling of secretions and atelectasis. Hence, postural drainage, chest physiotherapy, and antibiotics can be used to reduce postoperative pulmonary complications. Anesthesia plan in patients with prune-belly syndrome should make provisions for the associated anomalies of the chest wall, lungs, cardiovascular system, central nervous system, and renal and other organs and judicious use of muscle relaxants.

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Conflicts of interest

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References