Pelvic Osteotomies for Cloacal Exstrophy
Main Text
Table of Contents
Cloacal exstrophy is part of a wide-ranging spectrum of rare congenital abnormalities resulting from the same embryological defect. Conditions include bladder exstrophy, epispadias, cloacal exstrophy, omphalocele, and more. Mortality due to complications with cloacal exstrophy was historically significant as it is among the most severe of these abnormalities. However, advancements in reconstructive surgery have improved the survival of patients. Pelvic osteotomy is typically indicated in cloacal exstrophy as it normally presents with widely separated pubic bones that require approximation as part of abdominal wall closure.
The omphalocele, exstrophy of the cloaca, imperforate anus, and spinal defects (OEIS) complex is rare, affecting 1 in 200,000 to 400,000 pregnancies. There may be a spectrum of defects ranging from penile epispadias, through bladder and cloacal exstrophy.1
In nearly all cloacal exstrophy patients, spinal cord or vertebral abnormalities are seen. Orthopedic deformities also include a widely separated pubic symphysis and varying degrees of limb abnormalities.2 Pelvic osteotomy as seen in this case is indicated to remedy pubic deformities as part of the treatment course for cloacal exstrophy.
The patient was one of two twins. The other twin presented with no cloacal or congenital abnormalities. Prenatal ultrasound detected cloacal exstrophy with associated myelomeningocele.
Prenatal ultrasound findings can be suggestive of defects of the exstrophy-epispadias complex. Cloacal exstrophy will be visible upon birth in the delivery room. Patients are often preterm. As many as 65% of patients will present with clubfoot, and as many as 80% of patients have vertebral abnormalities. Myelodysplasia is very commonly seen in cloacal exstrophy patients.3
Omphaloceles are present in nearly all cloacal exstrophy patients. The bladder will be open and separated into two halves that flank the exposed interior of the cecum. Exposed bladder plate with a wide diastasis of the pubic rami can be seen.
Early diagnosis of the OIES complex is now possible using prenatal ultrasound. A prolapsed ileal segment unique to cloacal exstrophy may be visualized as an “elephant trunk like” mass on ultrasound. Major diagnostic criteria include non-visualization of the bladder, a large midline infraumbilical anterior wall defect, cystic anterior wall structure, omphalocele, and myelomeningocele.4 Minor diagnostic criteria include lower extremity defects, renal anomalies, ascites, widened pubic arches, narrow thorax, hydrocephalus, and single umbilical artery.4 CT or MRI can be used to obtain more detailed imaging prior to surgery to determine the best surgical plan.4
Anterior oblique osteotomy was the technique used in this case. Traditionally, osteotomies have been posterior, transverse, or a combination of the two. However, anterior has been shown to be more favorable. Posterior iliac osteotomy is an extensive procedure requiring rotating the patient from the prone to supine position intraoperatively whereas an anterior osteotomy can be performed with the patient in supine position throughout the procedure. Anterior osteotomy was associated with a closer reduction of the symphyseal diastasis and lower rates of recurrence of separation in pubic diastasis.5,6 The anterior oblique iliac osteotomy requires only a modest dissection of tissue allowing for less blood loss and a lower risk of infection and vascular and neurologic lesions.7
Other techniques of reducing pubic diastasis include the oblique iliac wing osteotomy or a pubic ramotomy. The oblique iliac wing osteotomy shows promise as a technique but has not been widely tested at this time. Pubic ramotomy has been shown to inadequately restore pelvic osseous relations, except in female newborns, and is therefore not widely used.3,7
Modern treatment of cloacal exstrophy will involve an anterior pelvic osteotomy facilitating the success of urogenital reconstructive surgery. This involves reduction of the pubic diastasis to restore normal bladder, urethra, and pelvic floor muscle anatomy.7 The goals of the modern treatment of cloacal exstrophy include secure abdominal and bladder closure, preservation of renal function, achievement of satisfactory continence, functional genitalia, and prevention of short bowel syndrome.8, 9
Severe pulmonary hypoplasia has been noted as a contraindication.10 In cases where there is an extremely wide pubic diastasis of >6 cm or if the patient has previously failed a primary closure, osteotomy may be more effective if performed prior to a secondary procedure for abdominal wall closure.8
Pubic bone approximation is a requirement for a successful bladder and abdominal wall closure for patients with cloacal exstrophy, thereby necessitating a pelvic osteotomy. Pelvic osteotomy allows for a deeper placement of the bladder in the pelvis and reduces abdominal wall tension.11 Most patients who do not undergo osteotomy at the time of cloacal closure require a secondary closure.8
A key point in successful cloacal exstrophy closure is to have a tension-free approximation of the widely separated public bones and anterior abdominal wall. To maintain closure and prevent said tension from altering the bladder neck and neourethral closure, secure pelvic ring closure, and postoperative traction or external fixation is needed regardless of the method of the osteotomy. Prevention of shearing forces from affecting those areas allows the bladder to remain deeper in the pelvis behind the anterior pelvic closure and lowers the risk of dehiscence of the wound and extrusion of the bladder.8
The anterior osteotomy approach has several advantages over the traditional approaches, including better approximation and mobility of the pubic rami at the time of closure and no requirement for turning the patient during the operation. Adjunctive posterior osteotomy can be included as needed from the anterior approach for cases of prior failed closure or extreme diastasis of >6 cm.3, 8 These cases may also benefit from a staged procedure rather than completing the osteotomy at the same time as the exstrophy closure. The patient’s pelvis would be brought together with slow stretching of the pelvic soft tissue and lead to fewer complications and less blood loss at the time of closure.11
Anterior osteotomy may be preferred over posterior in cases of severe lumbosacral dysraphism due to the proximity of incision to that of the often present myelomeningocele defect.3 Lumbar dysraphism has a high incidence rate in cloacal exstrophy patients, and most patients will require multiple later surgical procedures to achieve adequate bladder capacity and continence. While continence is part of the end goal of treatment, a direct and definitive relationship between pubic symphyseal closure and continence has not been established as the structures related to continence continue to evolve over time postosteotomy and lead to the need for subsequent procedures.4, 5
Complications can include dehiscence, bladder prolapse, and ventral herniation. Firm and tension-free closure is important to mitigate the odds of these complications.8 Femoral nerve palsies are another potential complication but are usually transient. Avoiding this can be facilitated by carefully elevating the superior gluteal nerve and vessels during the procedure. Infections are always a risk in surgical procedures and can promote dehiscence postosteotomy, so patients require monitoring of the wound site especially around any wires, sutures, or external fixators used to secure the pubic symphysis.
Recurrence of pubic diastasis separation is a common concern postosteotomy and occurs in up to 87% of patients who receive a combined pelvis osteotomy at <6 months of age and up to 71% in patients >6 months, necessitating a repeat closure. Lower rates are seen in anterior osteotomy cases versus posterior osteotomy.6 Increased rates of successful closure were seen in patients where 6–8 weeks of Buck’s traction with an external fixator was used postosteotomy.12 Older children tend to see lower rates of recurrent dehiscence, have increased bone density that allows for better pin fixation, and are closer to the adult anatomy of the ischiopubic segment as the pelvis grows.
Most patients will have good continence and be able to return to the same level of physical activity postoperatively. Normal gait and restoration of strength are seen except in cases of superior gluteal nerve injury. A long-term study found that exstrophy patients who did not have an osteotomy have higher relative joint force and stress levels along with increased degenerative changes in the hips on radiology.9, 13
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Nothing to disclose.
The patient referred to in this video article has given their informed consent to be filmed and is aware that information and images will be published online.
Citations
1. Smith NM, Chambers HM, Furness ME, Haan EA. The OEIS complex (omphalocele-exstrophy-imperforate anus-spinal defects): recurrence in sibs. J Med Genet. 1992;29(10):730-732. doi:10.1136/jmg.29.10.730
2. Mitchell ME, Plaire C. Management of Cloacal Exstrophy. In: Zderic SA, Canning DA, Carr MC, Snyder HMcC, eds. Pediatric Gender Assignment: A Critical Reappraisal. Springer US; 2002:267-273. doi:10.1007/978-1-4615-0621-8_16
3. Nhan DT, Sponseller PD. Bilateral Anterior Innominate Osteotomy for Bladder Exstrophy. JBJS Essent Surg Tech. 2019;9(1). doi:10.2106/JBJS.ST.18.00018
4. Woo LL, Thomas JC, Brock JW. Cloacal exstrophy: A comprehensive review of an uncommon problem. J Pediatr Urol. 2010;6(2):102-111. doi:10.1016/j.jpurol.2009.09.011
5. Jones D, Parkinson S, Hosalkar HS. Oblique pelvic osteotomy in the exstrophy/epispadias complex. J Bone Joint Surg Br. 2006;88-B(6):799-806. doi:10.1302/0301-620X.88B6.17712
6. Satsuma S, Kobayashi D, Yoshiya S, Kurosaka M. Comparison of posterior and anterior pelvic osteotomy for bladder exstrophy complex. J Pediatr Orthop B. 2006;15(2):141-146. doi:10.1097/01.bpb.0000191873.61635.10
7. Giordano M, Di Lazzaro A, Guzzanti V, et al. Oblique pelvic osteotomy in the treatment of bladder exstrophy in neonates. J Pediatr Orthop B. 2019;28(3):207-213. doi:10.1097/BPB.0000000000000614
8. Ben-Chaim J, Peppas DS, Sponseller PD, Jeffs RD, Gearhart JP*. Applications of Osteotomy in the Cloacal Exstrophy Patient. J Urol. 1995;154(2):865-867. doi:10.1016/S0022-5347(01)67187-8
9. Sponseller PD, Jani MM, Jeffs RD, Gearhart JP. Anterior innominate osteotomy in repair of bladder exstrophy. J Bone Joint Surg Am. 2001;83(2):184-193. doi:10.2106/00004623-200102000-00005
10. Modern treatment of cloacal exstrophy. J Pediatr Surg. 1991;26(4):444-450. doi:10.1016/0022-3468(91)90993-4
11. Inouye BM, Tourchi A, Di Carlo HN, et al. Safety and efficacy of staged pelvic osteotomies in the modern treatment of cloacal exstrophy. J Pediatr Urol. 2014;10(6):1244-1248. doi:10.1016/j.jpurol.2014.06.018
12. Baird AD, Sponseller PD, Gearhart JP. The place of pelvic osteotomy in the modern era of bladder exstrophy reconstruction. J Pediatr Urol. 2005;1(1):31-36. doi:10.1016/j.jpurol.2004.09.001
13. Purves JT, Gearhart JP. Pelvic Osteotomy in the Modern Treatment of the Exstrophy-Epispadias Complex. EAU-EBU Update Ser. 2007;5(5):188-196. doi:10.1016/j.eeus.2007.07.002
Procedure Outline
Table of Contents
- Salter Incision: One finger width below iliac crest, 1-2 cm proximal to ASIS
- Elevate External Oblique Muscle: Iliac crest exposed from ASIS to 1/2-3/4 the length
- Dissect Sartorius/Tensor Fascia Lata Interval: Protect and medially reflect lateral femoral cutaneous nerve
- Split Iliac Crest Apophysis
- Expose Inner & Outer Table of Ilium: Sub-periosteal dissection on medial and lateral surface
- Expose Sciatic Notch
- Cut from Crest to Sciatic Notch
Transcription
INTRODUCTION
Good morning. I'm Puru Gholve. I’m one of the pediatric orthopedic surgeons. Today I'm helping the urological team to close cloacal exstrophy. My part is pelvic osteotomy which will assist in closure of the cloacal osteotomy - cloacal exstrophy sorry. There are multiple and different types of pelvic osteotomies that are described to assist urological team to close the cloacal exstro - exstrophy. There is anterior oblique iliac osteotomy and that is the osteotomy that will be done today.
The approach used for the osteotomy is the sawdust incision which extends one fingerbreadth going along the along the iliac crest and going about 1 to 2 centimeter proximal to the anterior superior iliac spine. The incision deepens to the underlying tissues. The superior skin flap is retracted medially and the insertion of the external oblique muscle on the iliac crest apophysis is elevated and the iliac crest is exposed right from the anterior superior iliac spine going almost 1/2 to 3/4 of the crest. Anteriorly, plane is developed between the Sartorius and tensor fascia lata. The fascia in between the Sartorius and tensor fascia is dissected. The lateral femoral cutaneous nerve tie is protected, and that is kept with the Sartorius medially. This interval is deepened. The deep part of the rectus femoris is identified, and in this kid - and in general for bladder exstrophy - we don't need any further dissection to expose the hip, but the prow - PROW - the prow of the ilium that is the area between the anterior superior iliac spine and the anterior inferior iliac spine will be exposed. So dissection is just beneath the anterior superior iliac spine going up to the anterior inferior iliac spine, dissecting the muscle. The iliac crest apophysis is split along its length with the number 15 blade. When the splitting is done, it’s done with the sawing motion. Once the the iliac crest apophysis, with a thumb this split apophysis can be pushed medially and laterally. The medial half goes medially, the lateral half comes laterally, and then a subperiosteal dissection is carried on till the sciatic notch. This exposes the inner and outer table of the ilium. Once this is done, in small kids, we adjust with a scissor, about one to two centimeter behind anterior superior iliac spine. The pelvis can be cut right, starting from the iliac crest going towards the notch. The bone should be cut right up to the sciatic notch. Also, a light amount of skin traction or bucks traction is placed so that the osteotomy fragment does not migrant proximally.
Today, this will be the procedure that will be done on this kid, and we will talk through the steps once the procedure is being done.
These children tend to have very largely widely spaced pubic rami. That’s the ring of the pelvis. You can actually feel them here where my two thumbs are, and what we will do is create pelvic osteotomy to allow sort of inward hinging of the - of the pelvis so that we can close the pelvic ring. That is a crucial step in this operation because if you do not close a pelvic ring without tension, that increases the chances of dehiscence of the wound and extrusion of the bladder.
CHAPTER 1
Something like this. So they’re making incision along the iliac crest, starting - just medial to the ASIs. So the planes are going to be very very small here. I want to go just more medial with the incision Knife please. And then go back also. Slowly increasing the incision. Sem, please, sem. Put this. Let’s move the sem posterior a little. You also. So you can see now there's some muscle change here. That should be Sartorius to start up immediately because it's externally rotated spine. Your tensor is going to be more on this side like this. So I will confirm this is Sartorius - TFL. Far from it. Okay, agree - that's your TFL. Sartorius is more in the front. Yeah, yeah. I just want to get little exposure here like this. So you can come this way and hold it like this. So I’m going to elevate a little bit of this stuff here. Okay. It will appear, once you - that's Sartorius on that side. Let’s elevate the external oblique fascia before we go in there.
So once we find that place, it's not hard. Let's do little posterior side dissection here. We are taping this, right? So we are done incision along the - just beneath the iliac crest. Right now I'm elevating the external oblique muscles - muscle - from the top of the iliac crest. So I - this is the anterior superior iliac spine and the crest is going all the way back here, so I'm on the top of the crest here. Important to know, the TFL and Sartorius muscles. So this is the Sartorius muscle go medially - this muscle - and from here backward, this is the tensor fascia lata. So we are going to go through the tensor fascia lata into the start of the start of this TFL interval. Right now, we want to do little more exposure of the iliac crest. Just trying to go as back as possible here. Thank you. If you want to zoom in, I can show you. This is the exposed iliac crest. So this is the top of the iliac crest. Apophysis are exposed and this is anterior superior iliac spine and the crest is exposed up to here.
I’m happy with the exposure here, right? So far yeah. Let’s go to the TFL side now. Sartorius is in front of you. That's Satorius. Can you hold this retractor for a minute? Good, so we are opening from here now. So I can see the fibers of TFL here. I'm just pushing these fibers laterally. I want to be sure that I don't - this is the end over here.
So we’ve exposed the Sartorius TFL interval, and we can see the rectus femoris into the depth of it. Our next step is to cut the iliac crest. You want to keep your finger on the crest. Looking for the crest... So the crest has been cut little bit on the inside - that should not make any difference. So we are elevating the iliac crest. Might want to just spackle Ray-Tec here. The superior dissection will just keep on oozing on you otherwise. Normally, I’m able to just shove it in full Ray-Tecs here. You can see how much it’s going in here. Let’s do the anterior dissection here now. Where are the ASI’s? This is the ASI here - we’re going to connect this dot here. You are good - you're not going to the lateral decubitus nerve, which is more medial. Can you hold hold this one for a minute?
CHAPTER 2
I’m usually not able to position it - that's the hardest part. Okay.
Hard to get... the baby’s too small. I’m quite sure that it is here - let’s see if we can see it from this side. It's harder from outside to inside - inside to outside is the way to do it. I can take the suction in my hand for a minute.
So, granted you can peek in, the right angle snap is passing beneath the sciatic notch. You can see starting from the inside of the sciatic notch and coming of the outer ilium. Now we are going to do the osteotomy. Osteotomy is about a centimeter behind the ASIs. Now the last part of the osteotomy I do with the - intuitively. You are holding it, right? You can see that tough structure that needs to be released. There’s the deep periosteum. So I’m going to cut it out ultimately. But I don’t want it to come out - I want it to straight come out so everything should be cut there.
He’s really moving now. Yes. There’s nothing that can hold this. I think the hole is so small - it's impossible to get a pin in there. Irrigation please.
CHAPTER 3
So for description, just try to cut this vine. Otherwise, it's hard to close. The first one goes around it, so can you go through the apophysis? Alright, the closure looks good. I wouldn’t do any nylon on that - it’s good.
CHAPTER 4
Probably more like here, huh? Do you mind running some problem on this side? The sac is coming all the way to the incision. I’m going to try to avoid it as much as possible. I think it goes like back here - yeah. It’s a mobile window though. The crest is here.This is the top. You'll see on the inside. You can see this is the top of the crest - that is the inside. Knife please.
That’s the ASIs - you can't go beyond that. That's Satorius, right? Yeah. Just go slow and steady - I’m just afraid for the - can I get pull please? Get me a skin knife first - got to go a little posterior. You’re really close to the pouch - yeah. That’s - that might be sac. Yeah - that might be sac there. I will be able to dissect around it anyways.
That should be fine, right? Now want to divide up here - expose the… I don’t think we should be going more than this, right? There’s little elevation here. That’s good. Let’s go to the TFL now. I might have taken on a little Sartorius there looks like, right? Yeah, looks like it.
Can you put the other side of the retractor in there? Yeah that's good. I’m going to delve out of the plane right now. That’s fine - that’s the ASI. This is - I’m not going to expose too much of the crest this time. We’re starting to do minimal as much as possible, right? I’m a little outside - that’s why it bled. Let’s get out of this plane here for TFL. Can I get a Freer please? Pick ups please. There’s the TFL okay, that here and that one there, okay? You got to get the fat off. That’s good. Please just to me here. Come in here and bring double. Come in there and double. Can I let go? This one - this one - this one. Come in there double. That’s good. Very good. Ray-Tecs please - just plunge it down - yeah, just back in there.
So let me make cut a little posterior here, otherwise we won’t be able to see anything. You can keep the bone wax away from it - I’m just going to use the Freer. You don't go out there - you really can't retract stuff. Freer back please. A little bit tight on this side, but I had anticipated that. Let’s see what we can do on this side here. Let’s see if we can get to it from this side. Just leave it like this here. This is not subperiosteal - it is not subperiosteal. Okay. Start right there? See that’s a subperiosteal plane - one single misstep and it will cause bleeding. With kid again falling too much in the supination - that’s the problem. You want to hold the pelvis - that is the right thing you are doing, holding the pelvis. What happened to the sciatic notch here all the way. Good ahead. So you can hold this one here. Come alongside now - I need a right angle snap. I’ll hold this one - you can hold the pelvis for a minute. I have not gone on this side yet - we got there - no I didn’t. Okay got this like this - keep the leg like this down.
So what’s happening is some of this periosteum is still hinged to here, but there’s no subperiosteal dissection. This the outer table - see here? If you take this thing down, that will help. But this tissue was blocking us. Okay. I should be able to see here now - so might help if you just pull back a little bit. Can I get another right angle snap please? I think we are there - it’s just... we are falling into the crest here. That’s it - that’s the sciatic notch. We should start to see a little bit better now. Stop here - you - you are there. So pull back a little bit. Turn towards me - no the other way around. Yeah - now - it just sticks into the soft tissues. We don’t - don’t want the osteotomy to be too much in the front though. Then we can get our exposure though, right? How many excuses... Yeah - you’re fine. Yeah, that’s the part. He’s in there now. Just hold it. That’s good - that’s hinged - that’s hinged.
We can just start the osteotomy - I can see here. Yeah, there I am. Where? Just touching your tip. I don’t know - I don’t see you. Hold this there for a minute - don’t push it - come back to me. Now you can come out with the Freer. Just the tip of the periosteum not allowing you to move.
Pick up. You are... that fragment - let me hold it. Yep. Don’t take too much of it - just a fair part. I’m going to take a little more from here - this part. More? You can close it.