Prune Belly Syndrome Flashcards
Discuss prune belly syndrome.
Prune belly syndrome is characterized by three cardinal features: bilateral undescended testicles, dilated urinary tract, and deficient abdominal wall musculature.
These manifestations place patients with prune belly syndrome at risk for testicular malignancy, infertility, urinary tract infections, and renal failure.
In addition, extra-genitourinary manifestations may also affect the gastrointestinal, orthopedic, and cardiopulmonary organ systems.
The syndrome affects males in 95% of cases with up to 29% of patients dying during the perinatal period, the majority from complications of pulmonary hypoplasia.
The complexity of these patients requires a thorough understanding of their pathophysiology by pediatric surgeons and pediatric urologists alike.
By what other names is prune-belly syndrome (PBS) known?
- Eagle-Barrett syndrome
- Abdominal musculation syndrome
- Triad syndrome
What is the incidence of PBS?
- PBS has a contemporary incidence of 3.6–3.8 per 100,000 live male births [1].
- It is a predominantly male diagnosis as <5% of those diagnosed are female [1].
What are theories regarding the embryology of the development of PBS?
- No single embryological explanation has universal acceptance and many theories overlap.
- Some theories emphasize that a severely dilated urinary tract—either from early in utero posterior urethral obstruction or an intrinsic defect in the urinary tract—leads to redundancy in the abdominal wall.
- Another theory ascribes the syndrome to a yolk sac defect.
- A final theory points to a possible defect in the lateral plate mesoderm which gives rise to the ureters, bladder, prostate, urethra and gubernaculum.
What are the major manifestations of PBS, giving rise to its alternative name of the triad syndrome?
- A deficiency of abdominal musculature leading to a wrinkled “prune- like” appearance of the abdominal wall.
- Bilateral intra-abdominal testes.
- Urinary tract dysmorphism. The urinary tract anomalies are characterized by differing degrees of renal dysplasia, hydronephrosis, dilated tortuous ureters, an enlarged bladder and a dilated prostatic urethra.
What percentage of patients with PBS are female? What major manifestations of PBS does a female with the diagnosis exhibit?
• Only 5% of PBS diagnoses are female.
• Females exhibit only deficiency of abdominal wall musculature and the
anomalous urinary tract without any gonadal abnormality [2].
Besides the triad, what are other manifestations of PBS and how common are they?
- 75% of children with PBS have non–urinary tract abnormalities [3].
- These abnormalities include respiratory (58%, e.g. pulmonary hypoplasia), cardiac (25%, e.g. patent ductus arteriosus, atrial septal defect, ventricular septal defect, tetralogy of Fallot), gastrointestinal (63%, e.g. constipation, incomplete rotation of the midgut) and musculoskeletal anomalies (65%, e.g. talipes equinovarus, scoliosis, hip dysplasia) [2, 4].
How common is prematurity in those born with PBS?
• The incidence of prematurity in the PBS population is nearly 50% [3].
What is the perinatal mortality of those born with PBS? Which factor most limits survival?
- Perinatal mortality ranges between 10–29% in contemporary studies [1].
- Perinatal mortality is directly connected to the level of prematurity and severity of pulmonary hypoplasia [2].
What is the most common urinary tract dysmorphism found in PBS?
- Hydroureteronephrosis is almost always present and most commonly bilateral [2].
- The distal ureter is usually where massive dilation occurs, however the presentation is variable.
- Hydroureteronephrosis is almost never due to obstruction within the ureter, rather, culprits include lower urinary tract obstruction (posterior urethral valves), vesicoureteral reflux, and a histologic deficiency of smooth muscle and preponderance of fibrous tissue in the ureters leading to ineffective peristalsis [3].
How common is vesicoureteral reflux in PBS?
• 75% of patients have vesicoureteral reflux with most cases being bilateral [2].
What percentage of patients with PBS have renal dysplasia?
• 50% of patients will have dysplasia in varying degrees and laterality [3].
How common is it for PBS patients to require renal replacement therapy? What are two clinical predictors for satisfactory long-term renal function in a newborn with PBS?
- 40–50% of patients will require renal replacement therapy at some point in time.
- Positive prognostic indicators include at least one normal-appearing kidney on ultrasound and a nadir serum creatinine of less than 0.7 mg/dL over their first year of life [2].
What characteristic appearance of the bladder one would expect in PBS?
- On imaging, the bladder is commonly enlarged with a volume 2 to 4 times that of expected capacity [5].
- These large bladders often demonstrate a smooth wall without trabeculation.
- The dome of the bladder is often diverticular-shaped and a patent urachus is found in 25–30% of patients [3].
- The bladder neck is commonly wide with a dilated prostatic urethra due to prostatic hypoplasia.
What type of bladder dynamics would you expect to find in a patient with PBS?
• PBS bladders are large-capacity, hypotonic bladders. Because of this, patients are often unable to adequately empty their bladder [2].
How common are urinary tract infections (UTI) in this patient population?
- Nearly 80% of patients will have at least one documented UTI. One third of these patients will develop pyelonephritis [2].
- The high likelihood of infection owes largely to the common co-morbidities of vesicoureteral reflux, ureteral urinary stasis due to ineffective peristalsis, and poor bladder emptying.
- Prophylactic antibiotics and circumcision are commonly recommended by urologists to reduce the risk of UTI.
Where are the testes usually located in patients with PBS?
• The testes typically lie intra-abdominally, bordering the dilated ureters at the level of the iliac arteries.
Histologically, is there any difference between testes in patients with PBS and those of normal, age-matched controls?
- A lower concentration of Leydig cells have been found in testicles in patients with prune belly syndrome. The number and size of seminiferous tubules are equivalent to patients without PBS [6].
- Germ cell counts, spermatogonia, and Leydig cells are essentially identical in intraabdominal testes of boys with and without PBS [3, 7].
What is the future reproductive potential in children born with PBS?
- Infertility is considered multi-factorial, largely due to cryptorchidism, retrograde ejaculation from an incompetent bladder neck, and prostatic hypoplasia.
- Prior to 1992, men with PBS were considered infertile as there had been no reported cases of paternity [8]. Since that time however, there have been several cases of men with PBS fathering children via sperm retrieval techniques and intra-cytoplasmic sperm injection.
- There have been reports of a normal pregnancy with vaginal birth in a woman with PBS [9].
What is the likelihood that patients with PBS will pass the syndrome to future offspring?
- Given the propensity of the syndrome to exist in males, occasional reported diagnosis in male siblings or cousins and the increased incidence in twins, it has been suggested that PBS has an underling genetic etiology [10].
- An exact inheritance pattern has yet to be described, and most cases are considered sporadic with patients having normal karyotypes.
- Of the few reported cases of patients with PBS fathering or mothering children reported in the literature, there have been no offspring diagnosed with PBS.
- Given what little is known at this point about the genetic basis of the syn- drome, patients should be counseled about the potential, yet still unknown, risk of transmission to their future children [8].
Are patients with PBS at an increased risk of testicular malignancy?
- There have been three cases of testis tumor reported in the literature [3].
- The risk of malignancy appears to be increased in PBS, but it is no higher than that of non-PBS patients with cryptorchidism.
- This emphasizes the importance of placement of the testes into the scrotum in a timely manner is necessary to both reduce the risk of developing malig- nancy as well as enhancing detection of potential tumors.
What area of the abdominal wall is most commonly deficient in patients with PBS?
- While rare cases have reported completely absent abdominal wall muscula- ture, the usual deficiency lies in the lower portion of the abdominal wall [11].
- The rectus muscles and internal and external obliques are less well devel- oped than in the upper abdomen and in severe cases, skin subcutaneous fat and a single fibrous layer may be all that is overlying the peritoneum [12].
Do children born with PBS commonly carry their characteristic wrinkled appearance of the abdomen into adolescence?
• As PBS children grow, adipose tissue deposits in the subcutaneous layer of the abdominal wall.
This tends to diminish the wrinkled appearance of the abdominal wall over the first year of life when their abdomen begins to take on more of a pot-bellied appearance owing to the deficiency of musculature [3].
Is there a characteristic gait associated with PBS? Are PBS children able to sit up normally?
- Walking style usually is not affected; however achievement of the walk- ing milestone may be delayed [3].
- Since the usual musculature responsible for sitting up is commonly deficient in PBS, patients tend to sit up by rolling to their sides and using their arms to push themselves up [3].
What bladder anomalies are found in prune-belly syndrome patients?
Usually the bladder in PBS patients will be massively enlarged and smooth walled without trabeculations.
Histologically muscle fibers are replaced by collagen and fibrous tissue.
This loss of muscle fibers will cause the bladder to bulge in multiple directions, which gives the appearance of a pseudodiverticulum at the bladder dome.
A patent urachus is present in 25–30% of PBS patients. In the presence of urethral atresia, a patent urachus will allow bladder emptying in utero.
The trigone is wider and the bilateral ureteral orifices are displaced more lateral than usual, which likely contributes to the presence of vesicoureteral reflux (VUR) in 75–80% of patients.
Also, the bladder neck is poorly defined and opens into a dilated, elongated prostatic urethra.
As the prostatic urethra becomes membranous, it will taper due to redundant folds of mucosa, which may give the impression of obstruction on imaging.
On urodynamic studies, PBS patients typically demonstrate a normal compliance. However, a very large bladder capacity and delay in the first sensation to void are often seen.
In addition, PBS bladders demonstrate poor detrusor contractions and incomplete emptying.
The ability to empty the bladder is variable, with around 50% of PBS patients voiding spontaneously with normal voiding pressures, normal flow rates, and low post void residual (PVR).
Regardless of the voiding pattern, all PBS patients should be closely followed because their ability to empty the bladder may change.
H&A
What ureteral anomalies are found in prune-belly syndrome patients?
As stated previously, the wide bladder trigone with bilateral ureteral orifices displaced more lateral than usual may contribute to the presence of VUR in up to 80% of patients.
The ureters are tortuous and severely dilated.
The degree of ureteral pathology can vary and has been shown to be segmental, with the distal ureter being the most affected.
Distally, the ureter has shown a greater decrease in muscle fibers and an increase in collagen and connective tissue fibers.
This causes inefficient ureteral peristalsis and the development of upper tract urinary stasis and system dilation.
This is important to identify prior to surgical reconstructions because the proximal ureter is typically a better substrate when compared with the distal ureter.
Although definite obstruction is not common, it has been reported at the ureterovesical junction (UVJ) and ureteropelvic junction (UPJ).
As the child grows and the ureters elongate, spontaneous improvement may occur in the ureteral appearance and function.
It is interesting to note that, in many cases, the flaccidity of the abdominal wall is not related to the degree of dilation of the urinary tract.
However, in a recent study, a direct correlation was observed between the flaccidity of the abdominal wall to the severity of the urinary tract dilation in which those with a severely wrinkled abdomen had marked dilation, whereas those with partial wrinkling had minimal dilation. This association continues to be studied.
H&A
What extra-genitourinary anomalies are found in prune-belly syndrome patients?
Up to 75% of children with PBS will have concomitant nongenitourinary diagnoses, with prematurity being the most common, affecting approximately 50% of newborns.
Cardiovascular, pulmonary, gastrointestinal, and musculoskeletal conditions have been described in PBS patients. The severity of these comorbidities will dictate the initial postnatal course of these patients.
Perinatal mortality (30%) is primarily related to prematurity and the degree of pulmonary hypoplasia.
Abdominal Wall
Abdominal wall muscle deficiency is one of the most distinctive features in PBS patients.
The abdomen appears wrinkled, with bulging flanks.
The degree of muscle deficiency varies, but usually the medial and inferior aspects of the abdomen are more severely involved.
The most affected muscle is the transversus abdominis.
In some cases, the musculature of the abdominal wall may be totally absent. Microscopically, it has been described as having myofilament disarray, decreased muscle fiber size, and excessive collagen accumulation.
As PBS infants and children grow, the deposition of cutaneous abdominal adipose tissue increases, which reduces wrinkles, and a potbelly appearance ensues.
Due to the lack of abdominal support, they can develop lordosis.
Gait usually is not affected but walking may be delayed, and PBS infants tend to roll on their side in order to sit up.
Due to poor lower chest wall support, PBS patients have a poor cough and are more prone to respiratory infections.
Cardiac and Pulmonary
During the neonatal period, stabilization of cardiac and pulmonary anomalies may take priority in management over the genitourinary conditions.
One-quarter of PBS patients have patent ductus arteriosus, atrial septal defect, ventricular septal defect, and tetralogy of Fallot.
Pulmonary anomalies may be seen in 60% of PBS patients. Patients can suffer from pulmonary hypoplasia due to oligohydramnios, which is caused by bladder outlet obstruction (BOO) and renal dysplasia.
Nearly half of PBS patients may need mechanical ventilation during the neonatal period.
If severe pulmonary hypoplasia is present, it can result in newborn demise.
Other associated lung anomalies may be pneumothorax or pneumomediastinum.
Reactive airway disease was found in 22% of patients in one study.
As mentioned, due to poor lower chest wall support, PBS patients have poor cough effectiveness and are more prone to respiratory infections and atelectasis.
It is important to note that PBS patients are more prone to respiratory failure from acute respiratory illness or any procedure with anesthesia.
Older patients may develop restrictive lung disease secondary to musculoskeletal abnormalities.
Gastrointestinal
Approximately 30% of PBS patients will suffer from gastrointestinal conditions.
The majority of anomalies are due to incomplete rotation of the midgut and the development of a narrow mesentery, which may lead to increased bowel mobility in the capacious abdominal cavity.
This predisposes PBS patients to developing volvulus.
In addition, intestinal atresia and stenosis have been documented. Intestinal atresias are more common than stenosis, and most atresias occur in the colon.
Omphaloceles, splenic torsion, gastroschisis, and anorectal malformations have been described.
Constipation affects 60% of PBS patients and is a lifelong problem due to the inability to generate adequate intra-abdominal pressure, which may lead to acquired megacolon.
Musculoskeletal
Musculoskeletal anomalies are present in a quarter of PBS children. The etiology of such abnormalities is suspected to be in utero compression and the sequelae of oligohydramnios.
The most common deformity is clubfoot, which is seen in 25%.
Furthermore, scoliosis (4%), hip dysplasia (5%), and pectus excavatum have been described as well.
Lower limb hypoplasia or aplasia also occurs.
Spectrum of Disease
There is a broad spectrum of severity, with some PBS patients not surviving the neonatal period to others with minimal symptoms and near normal life expectancy.
Woodard described a classification system to aid in patient management in which PBS patients are sorted into three groups.
H&A
What are the indications of abdominal wall reconstruction for prune-belly syndrome?
RECONSTRUCTION OF THE ABDOMINAL WALL
The need for abdominal wall reconstruction is reserved for those with moderate to severe degrees of abdominal wall laxity.
Those with smaller degrees of abdominal wall laxity should be observed as some improve with time.
Potential benefits to abdominoplasty include improved bladder emptying, bowel function, pulmonary efficiency, and improvement in self-esteem.
The abdominal wall reconstruction can be performed during the urinary tract reconstruction or as a separate operative intervention.
Younger patients may have difficulties with respiratory function after repair requiring postoperative mechanical ventilation in the pediatric intensive care unit.
The Ehrlich and Monfort approaches have gained popularity over the years. Many modifications to these original techniques have been described. Most techniques use a variation of fascial mobilization in a “vest over pants” configuration.
In a recent report, Smith et al. described their modification to the Monfort technique. They made five modifications that included diagnostic laparoscopy to define the topography of the abdominal wall defect, an initial midline vertical incision around the umbilicus, creation of an asymmetric central plate to compensate for the disparity in the abdominal defect, plication of the central plate to reduce redundancy and help reposition the umbilicus, and plication of focal areas of fascial weakness.
Franco et al. described a laparoscopic-assisted modification of the Monfort abdominoplasty.
A number of authors have demonstrated excellent functional and cosmetic results in PBS patients.
H&A
What are the two types of megaureter?
Megaureter describes a ureter that is abnormally dilated. The ureter can sometimes also be greatly elongated and tortuous.
In general, the two principal pathologic types of megaureter are those that are obstructed and those with massive vesicoureteral reflux.
There may be both reflux and obstruction present, as in a megaureter ending ectopically in the bladder neck or urethra, which may be obstructed during the resting state but allow reflux during voiding when the bladder neck opens.
In primary cases, the bladder is normal and the abnormality is located in the distal ureter.
In other cases, megaureter may be secondary to bladder dysfunction. For example, impressive megaureters are seen in some males with high-grade urethral obstruction from posterior urethral valves. Ablating the valves can decrease intravesical voiding pressures and allow compensation of the ureters in time.
Similarly, a neuropathic bladder secondary to meningomyelocele can cause physiologic obstruction of the bladder outlet with secondary megaureter.
Occasionally, megaureters may represent a dysmorphic anomaly without obstruction or reflux despite the dilation (nonobstructive nonrefluxing megaureters).
Megaureters may be classified on the basis of pathophysiology according to an international system proposed in 1977.
When the abnormality is intrinsic in the lower ureter, the megaureter may be a unilateral problem, especially with a typical obstructive megaureter.
These cases classically feature an abnormal terminal ureter with a variety of histologic and ultrastructural abnormalities. Some have referred to this abnormality as an adynamic segment, but it is not comparable with the adynamic segment of lower colon in Hirschsprung disease or the lower esophagus in achalasia.
The ureteral orifice often looks normal in obstructive megaureter, and true stenosis is rarely noted. Typically, a ureteral catheter can be passed through the segment without difficulty.
The ureteral orifice of a refluxing megaureter is usually abnormally dilated. Indeed, an endoscope can often be passed readily up many such megaureters.
In primary reflux, there exists an inadequate valvular mechanism related to the diameter of the ureter and the length of submucosal ureter within the bladder wall.
Ureterocele and ectopic ureter are commonly associated with megaureter, especially in the female infant with a duplex collecting system. Rarely, megaureter is the result of excessive urine flow, such as that seen in diabetes insipidus.
The prunebelly syndrome features abnormal development of the entire urinary tract including the ureters, which are typically elongated and tortuous. Microscopic section of those ureters may show decreased musculature and excessive connective tissue in the ureteral wall, often affecting the lower ureter more than the mid or upper segments.
Megaureters are typically found in two patient populations. Newborns with the problem may be identified in follow-up of antenatally detected hydronephrosis. The relative incidence of megaureter compared with other congential upper tract anomalies is higher in this group than in older children presenting with clinical problems. The most common presentation in older children is urinary tract infection, although some children may have intermittent pain from obstruction. Infants may show failure to thrive.
Whenever megaureters are encountered during urologic evaluation, the entire urinary tract must be evaluated to determine whether they are a primary or secondary problem. The kidneys and ureters are often first visualized by ultrasonography. Intravenous pyelography may be useful; however, the diagnosis of obstruction is most often made by nuclear imaging with diuretic washout.
The drainage curve after diuresis is informative, but care must be taken that the area of interest includes the entire upper system (ipsilateral kidney and ureter).
Drainage of radionuclide out of the kidney into ureter and not bladder can lead to a false-negative examination. Particularly in newborns evaluated for antenatally detected hydronephrosis, the function of the kidney should be considered in interpreting nuclear renography, and significantly decreased relative function is generally considered an indication for surgical intervention in an otherwise asymptomatic child.
Any decline in function (>10%) on serial renography is a strong indication for repair.
Antegrade pyelography performed by inserting a needle into the kidney and infusing contrast medium is occasionally helpful in defining anatomy and demonstrating differential pressures between the upper tract and bladder (Whitaker test).
Voiding cystourethrography is mandatory to determine if reflux is present and will demonstrate the ureteral anatomy if there is reflux.
In addition, in males it will demonstrate any urethral pathology.
Urodynamic study may be a valuable adjunct in assessing these cases if any bladder abnormality is suspected.
Temporary placement of an indwelling urethral catheter may also differentiate between primary and secondary megaureter on serial imaging.
Cystoscopy should define the appearance of the orifices but is generally only performed at the time of reconstructive surgery.
As noted, the orifices in obstructive megaureter usually look normal; in refluxing megaureter, they are usually widely dilated with a “gopher hole” appearance.
The advent and widespread use of intrauterine ultrasonography has allowed early diagnosis of many cases of megaureter.
Affected babies should have selective urologic investigation after birth.
Obstructive megaureters without reflux may improve spontaneously. Thus in asymptomatic infants with mild or moderate obstructive megaureter and normal relative renal function, serial observation may be considered for a period of time to see whether spontaneous improvement occurs.
If improvement is not clear cut or if the patient develops symptoms, operative repair should be performed.
Retrovesical ureteral diameter is a reasonable predictor of the likelihood of resolution of hydronephrosis or the necessity of surgical intervention in the absence of reflux. This is helpful in counseling parents.
If the primary problem is a neuropathic bladder, anticholinergic therapy and intermittent catheterization may result in improvement in ureteral dilation.
If the patient has severe urethral valves, fulgurating the valves may suffice.
Valves occur in a spectrum of severity. Thus treatment may be valve fulguration in some patients, valve fulguration followed by early megaureter repair in others, and, occasionally, valve ablation and immediate megaureter reconstruction.
In summary, indications for repair of a primary megaureter vary with the etiology.
In cases of primary obstructive megaureter, poor renal function (35% to 40% of total function by renography), a severely scarred kidney, symptoms, or decreasing function on serial studies are generally considered as clear indications for intervention.
Bilateral megaureters or one involving a solitary renal unit threaten total renal function and should be treated more aggressively.
Finally, failure to improve after a reasonable period of observation may, at times, be an acceptable indication for surgical repair.
Primary reflux into a megaureter, except in a newborn with normal renal function, is unlikely to resolve and usually will eventually require repair.
Febrile urinary tract infection is another indication for surgical correction of primary megaureter whether reflux or obstruction is present.
Secondary reflux may resolve with treatment of bladder outlet obstruction or bladder dysfunction.
Intervention, when indicated, is virtually always open repair because the results are good even when dealing with large ureters in neonates.
Endoscopic dilation followed by temporary internal stenting may result in temporary improvement in function or drainage but is unlikely to be lasting in effect.
Diversion by cutaneous ureterostomy has occasionally been used for an infected system or when renal function in a neonate is so poor that it is not clear whether repair or nephrectomy is more appropriate, or in premature infants who are symptomatic or have significantly compromised renal function. In those settings, temporary percutaneous nephrostomy drainage may be just as beneficial and less morbid.
As such, cutaneous ureterostomy is rarely necessary.
Coran
When is surgical correction for the urinary system indicated in patients with PBS?
When initial evaluation demonstrates clear obstruction, correction should be performed whether it involves ureteropelvic junction, ureterovesical junction, or bladder outlet.
Most patients have some underlying renal dysplasia, and secondary insult from obstruction should not be allowed. It is our experience that most patients with this syndrome and vesicoureteral reflux eventually undergo repair.
Classic grading systems for reflux may not apply to such patients; however, reflux in patients with prune-belly syndrome is often massive in volume.
Early observation while the patient is on daily antibiotics to prevent infection may be appropriate and allow evaluation of bladder emptying or other issues.
If reflux persists, correction is appropriate.
When patients with reflux suffer breakthrough urinary tract infection or deterioration in renal function, repair of the reflux should be undertaken.
In considering the urinary system in patients with this syndrome, it may be useful to categorize them into three groups as described by Smith and Woodard. The categories roughly correlate with the degree of renal dysplasia present.
Group I patients usually have both severe renal dysplasia and pulmonary hypoplasia and do not survive. Urologic intervention will not rescue such patients.
Group III patients generally have a mild or incomplete form of the syndrome and well-preserved renal function. Although some such patients may have impressive dilation of the urinary tract, little urologic intervention may be required if the patients do not suffer recurrent urinary tract infection.
Definite indications for intervention in group II patients with intermediate degrees of dysplasia and renal dysfunction are less clear. These patients typically have full-blown features of the syndrome and a stagnant urinary system at best. Clearly, some such patients will progress to renal insufficiency with or without surgical intervention.
So many variables are present in patients with prune-belly syndrome that it remains difficult to predict the natural history for a given patient early in presentation.
Careful surveillance is critical, and we favor surgical intervention in group Ii patients if they suffer from recurrent infection or deterioration of renal function. Those two problems often go hand in hand.
We believe that correction of obstruction or reflux, optimizing bladder emptying, and avoidance of infection may prevent renal insufficiency in some patients with marginal function or, at least, prolong the time until they suffer insufficiency.
It is clear that the scientific evidence to support such intervention, or argue against it, is largely anecdotal at this point.
Some of the most impressive megaureters are found in patients with prune-belly syndrome, and they are often then associated with other clinical problems.
The philosophy of treating selective cases by aggressive surgical reconstruction is the opposite of the “watchful waiting” espoused by others.
Surgical intervention is actually a conservative method in that it attempts to decrease stasis, urinary tract infection, and renal failure, the natural history of many patients with the syndrome.
Reconstruction is demanding and technically difficult in these patients. It also requires extensive anesthesiology expertise to maintain metabolic hemostasis in a young child undergoing major reconstruction.
This surgery should be performed in centers where surgeons concentrate on major reconstructive surgery and have access to all of the requisite back-up systems.
Coran
