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LETTERS TO EDITOR
Year : 2019  |  Volume : 20  |  Issue : 1  |  Page : 47-49
 

Prune-belly syndrome: Anesthetic implications and management


Department of Anesthesiology and Critical Care, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India

Date of Web Publication6-May-2019

Correspondence Address:
Dr. Sunit Kumar Gupta
Department of Anesthesiology and Critical Care, All India Institute of Medical Sciences, Jodhpur - 342 005, Rajasthan
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/TheIAForum.TheIAForum_2_19

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How to cite this article:
Goyal S, Gupta SK, Kothari N, Bhatia P, Kumar M. Prune-belly syndrome: Anesthetic implications and management. Indian Anaesth Forum 2019;20:47-9

How to cite this URL:
Goyal S, Gupta SK, Kothari N, Bhatia P, Kumar M. Prune-belly syndrome: Anesthetic implications and management. Indian Anaesth Forum [serial online] 2019 [cited 2019 Aug 26];20:47-9. Available from: http://www.theiaforum.org/text.asp?2019/20/1/47/257678




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: Potbelly abdomen with vesicostomy scar

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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.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Holder JP. Pathophysiologic and anesthetic correlations of the Prune-Belly syndrome. AANA J 1989;57:137-41.  Back to cited text no. 1
    
2.
Jennings RW. Prune Belly syndrome. Semin Pediatr Surg 2000;9:115-20.  Back to cited text no. 2
    
3.
Strand WR. Initial management of complex pediatric disorders: Prunebelly syndrome, posterior urethral valves. Urol Clin North Am 2004;31:399-415, vii.  Back to cited text no. 3
    
4.
Bariş S, Karakaya D, Ustün E, Tür A, Rizalar R. Complicated airway management in a child with Prune-Belly syndrome. Paediatr Anaesth 2001;11:501-4.  Back to cited text no. 4
    


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