|Ahead of print publication
Prune belly syndrome: Anesthetic challenges and management
Aparna Abhijit Bagle, Deepali Rahul Patil, Krusha Suresh Shah, Anshu Kumari
Department of Anesthesiology, Dr. D.Y. Patil Medical College and Research Centre, Dr. D. Y. Patil Vidyapeeth, Pune, Maharashtra, India
|Date of Submission||27-Jul-2021|
|Date of Decision||19-Jan-2022|
|Date of Acceptance||20-Jan-2022|
Krusha Suresh Shah,
Department of Anesthesiology, Dr. D.Y. Patil Medical College and Research Centre, Pimpri, Pune 411018, Maharashtra
Source of Support: None, Conflict of Interest: None
Prune Belly syndrome (PBS) is a triad of absence of abdominal muscles, urinary tract malformations, and undescended testes. Due to partial or complete lack of abdominal muscles and flat diaphragm, there could be difficulty in breathing and effective coughing. It can also be associated with chest wall deformities which can cause increased incidence of respiratory infections and lead to complications in the peri and postoperative period. Here, we have discussed about the anesthetic challenges in 1-month-old baby diagnosed with PBS who was posted for cystoscopy.
Keywords: Anesthetic challenges, Eagle Barrett syndrome, Prune Belly syndrome
| Introduction|| |
Prune belly syndrome (PBS) also known as Eagle Barrett syndrome or Triad syndrome is a rare disorder which presents at birth. It was first described by Frolich in 1839. The name Prune belly was given referring to the wrinkled appearance of the abdomen by Osler in 1901. With prenatal scans, PBS can be diagnosed in the second trimester of pregnancy. It is more common in males. The incidence of PBS is one in 30,000–40,000 live births with a mortality rate of 20%. Various genetic factors like 158 kb duplication at 4q22 - overlaps BMPRIB gene, duplication in intron of STIM1 gene, 67 kb duplication 202 kb downstream of NOG gene, 134 mb deletion including MYOCD gene are found to be associated with this syndrome.
It consists of a triad of partial or complete absence of abdominal muscle, failure of bilateral testes to descend into scrotum, and urinary tract malformations. There could be associated cardiovascular, musculoskeletal, gastrointestinal, respiratory, and central nervous system abnormalities,, This may necessitate surgical intervention and corrective procedures. Due to these anatomical and pathophysiological changes, patient may pose with many challenges for an anesthesiologist in the perioperative period.
There are several theories but the exact cause of this syndrome is still not known. Some studies suggest that abnormality in the development of bladder during fetal life leads to its distension and urinary retention. Due to this enlargement, there is atrophy of the abdominal musculature and cryptorchidism is due to obstruction caused by bladder enlargement. Few other studies suggest that incomplete development of abdominal musculature leads to urinary tract abnormalities. As abdominal muscles play an important role in respiration, their absence will lead to chest wall deformities. Pigeon chest, Harrison groove, flaring of rib margins are very commonly associated with this syndrome.
| Case Report|| |
A 1-month-old male baby presented with complaints of decreased micturition and decreased passage of stools with decreased appetite since birth. He was posted for cystoscopy. He was born at 34 weeks of gestation by normal vaginal delivery with a birth weight of 3 kg. The baby cried immediately after birth and was immunized up to date. On antenatal scanning at 7th month of intrauterine life, he was diagnosed with hydroureter and hydronephrosis. On preanesthetic check-up, the baby had upper respiratory tract infection for 3 days. The anterior abdominal wall lacked abdominal muscles and the skin was thin such that the intestinal loops were visible through the abdominal wall [Figure 1]. On examination, the baby was conscious and playful with a heart rate of 150 beats/min. Air entry was bilaterally equal with no added sounds. On palpation, the abdomen was doughy. On echocardiography, there was a large fenestrated secundum atrial septal defect with left to right shunt. Ultrasound of abdomen and pelvis suggested that there was marked laxity of anterior abdominal wall with bilateral hydronephrosis and bilateral dilated and tortuous ureters. On micturating cystourethrogram, there was posterior urethral dilatation which was suggestive of diverticulum. Chest X-ray showed flattening of diaphragm with flaring of ribs. There was no history of previous intensive care unit (ICU) stay, surgery, or any other comorbidities. Parents were counseled regarding the possibility of requirement of postoperative ventilatory support and ICU requirement during preoperative visit. The night before and morning before surgery the patient was nebulized with normal saline. With written informed consents from the parents, the patient was accepted under the American Society of Anesthesiology score three and taken inside the operation theater. Standard monitoring was established. Child was preoxygenated with 100% oxygen (O2) for 3 min. Injection glycopyrrolate 0.004 mg/kg intravenous (IV) was administered before induction. Induction was started with O2 plus sevoflurane, injection atracurium 0.5 mg/kg IV was given. Baby was intubated with 3.5 mm oral uncuffed endotracheal tube [Figure 2]. Anesthesia was maintained on O2, sevoflurane, and air on assisted ventilation with Jackson Rees circuit. In left lateral position, caudal block was given with 4cc of 0.25% bupivacaine using 22 G one inch needle [Figure 3]. Cystoscopy was performed with minimal blood loss. At the end of surgery after extubation child developed bronchospasm which was managed by giving 100% oxygen, asthalin puff, and maintaining airway and saturation. Baby was shifted to neonatal ICU for observation.
| Discussion|| |
PBS is associated with congenital chest deformities, pulmonary hypoplasia, or scoliosis. This can lead to pulmonary dysfunction and decreased residual capacity in the affected patients. Respiratory complications have been the leading cause of mortality in patients that present for surgical procedures. Poorly developed abdominal muscles with a flat diaphragm can lead to pooling of secretions, atelectasis, and respiratory infections. Postural drainage, chest physiotherapy, and antibiotics should be used to decrease pulmonary complications postoperatively. The most critical factor that must be watched during surgery in patients with PBS is airway pressure during abdominal wall reconstruction. Postoperatively, these patients must be monitored closely. They can easily become fatigued and may experience respiratory distress.
PBS patients can present with Potter's face, ear, and nose deformities along with micrognathia. This has been associated with oligohydramnios during antenatal period.,, Thus, airway challenges in terms of difficult mask holding and intubation are anticipated. Adequate measures of difficult intubation scenario according to the difficult airway algorithm should be kept available during the time of induction up to the postoperative period.
Renal dysfunction can occur as a result of hydronephrosis, urinary tract infections, congenital renal dysplasia, or hypoplasia. Thus, decreased renal function can lead to delayed excretion of the anesthetic agents. Thus vigilant dose calculations and titrations should be followed at all times.
Taking into consideration all the above-listed complications, regional anesthesia is preferred wherever possible. Pain relief with caudal, epidural, or peripheral nerve block may expedite recovery from anesthesia and also decrease the requirement of anesthetic agents.
| Conclusion|| |
A detailed pathophysiology of PBS should be known to prevent perioperative morbidity. A thorough preoperative assessment should be done to know the severity of the disease and associated malformations. A careful anesthetic plan should be formulated to minimize perioperative complications.
Declaration of patient consent
All the appropriate consents for sharing his/her clinical information and photographs have been obtained from the patient's parents. The parents understand that their names and initials will not be published and all attempts to conceal patient identity will be made, however, anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Nunn IN, Stephens FD. The triad syndrome: A composite anomaly of the abdominal wall, urinary system and testes. J Urol 1961;86:782-94.
Kheir AE, Ali EM, Medani SA, Maaty HS. Prune belly syndrome: A report of 15 cases from Sudan. Sudan J Paediatr 2017;17:42-8.
Boghossian NS, Sicko RJ, Giannakou A, Dimopoulos A, Caggana M, Tsai MY, et al.
Rare copy number variants identified in prune belly syndrome. Eur J Med Genet 2018;61:145-51.
Jennings RW. Prune belly syndrome. Semin Pediatr Surg 2000;9:115-20.
Wheatley JM, Stephens FD, Hutson JM. Prune-belly syndrome: Ongoing controversies regarding pathogenesis and management. Semin Pediatr Surg 1996;5:95-106.
Henderson AM, Vallis CJ, Sumner E. Anaesthesia in the prune-belly syndrome. A review of 36 cases. Anaesthesia 1987;42:54-60.
Aydoğmuş MT, Oba S, Ediz N, Berber S, Sarıkaş CM. Anaesthetic Management of laparoscopic surgery in prune-belly syndrome: report of two cases. Turk J Anaesthsiol 2013;1:41.
Bösenberg A. Anaesthesia for prune belly syndrome. South Afr J Anaesth Analg 2004;10:10-1.
Kartz J, Benumof J, Kadis L. Anesthesia and Uncommon Diseases, Pathophysiology and Clinical Correlations. 2nd
ed. Philadelphia: W. B. Saunders Co; 1981. p. 146-50.
Yoon J, Ryu J, Kim J, Jeong CY, Park SG. Anesthetic experience of a patient with Prune-belly syndrome. Korean J Anesthesiol 2014;67:S94-5.
King CR, Prescott G. Pathogenesis of the prune-belly anomalad. J Pediatr 1978;93:273-4.
[Figure 1], [Figure 2], [Figure 3]