Repeat pelvic osteotomy in cloacal exstrophy: applications and outcomes

PurposePelvic osteotomies have been shown to enhance success rates for classic exstrophy patients when closed primarily or secondarily after initial failure. Primary closure of cloacal exstrophy also benefits from osteotomy but this has yet to be shown for re-closure of cloacal exstrophy failures. This study looks at the applications, complications, and long-term success rates in this very select group of patients.MethodsWe extracted from an institutionally approved exstrophy database 15 patients who had undergone repeat pelvic osteotomy and analyzed patient history, complications and orthopedic outcomes.ResultsAll patients who underwent reclosure at our institution remain closed. Major complications were seen in two patients and minor complications in four patients. Urinary continence was achieved in 10 patients with augmentation and continent stoma formation, urinary diversion was performed in two patients and three patients await a continence procedure.ConclusionsRepeat pelvic osteotomy in cloacal exstrophy is successful and the complication rate is low. Pelvic osteotomy is associated with enhanced success rates of primary and secondary closure with better cosmesis of the abdominal wall and genitalia. Intrasymphyseal plates along with gradual reduction of the extreme diastasis utitlizing an external fixation device can be beneficial prior to further genitourinary surgery.     

Safety and efficacy of staged pelvic osteotomies in the modern treatment of cloacal exstrophy

Introduction and objectiveStaged pelvic osteotomy (SPO) prior to bladder closure has been shown to be a safe and effective method for achieving pubic approximation in cloacal exstrophy (CE) patients with extreme diastasis. However, SPO outcomes have never been compared to those for combined pelvic osteotomy (CPO) at the time of closure in CE patients.MethodsA prospectively maintained database of 1208 exstrophy–epispadias complex patients was reviewed for CE patients treated with pelvic osteotomies. Inclusion criteria were osteotomy at the authors' institution and closure within two months of osteotomy. After inclusion, patients were separated into four groups depending on osteotomy procedure (SPO vs. CPO) and whether their osteotomy occurred with primary closure or re-closure. Patient demographics, closure history, pre-operative diastasis measurement, most recent post-operative diastasis measurement, and outcomes were recorded and compared by chi-squared tests and ANOVA.ResultsAmong 116 CE patients reviewed, 46 met inclusion criteria. With primary closure or re-closure, 27 had SPO and 19 had CPO. No SPO re-closure patients had previous osteotomy; 4 CPO re-closure patients had a previous osteotomy with closure. Median time between osteotomy and closure in SPO patients was 14 days. Median follow-up after SPO and CPO were 4 and 11 years, respectively. SPO significantly reduced the pre-operative diastasis compared to CPO on most recent diastasis measurement (3.5 cm vs. 0.4 cm, p = 0.003). There were no significant differences in the overall complication rate, or the rates of each specific complication, between the SPO and CPO groups. No patients had wound dehiscence or prolapse. One CPO patient was able to intermittently catheterize per urethra while all other patients required continent urinary diversion to achieve continence.ConclusionsTo the authors' knowledge, this is the first study comparing SPO and CPO outcomes in CE patients. SPO reduces pre-operative diastasis more than CPO, and does not appear to incur increased rates of complication, closure failure, or incontinence. Due to its apparent safety and greater efficacy, SPO should be considered in all CE patients with extreme diastases undergoing primary closure or re-closure.     

Comparison of Musculoskeletal and Urological Functional outcomes in Patients with Bladder Exstrophy undergoing repair with and without osteotomy

PurposeIt is controversial whether osteotomy by restoring a more normal pelvic anatomy might improve the final outcome of bladder exstrophy (BE) repairs. We compared the functional orthopaedic and urological outcome in BE patients treated with and without osteotomy.Material and MethodsOrthopaedic and urological outcome was compared in 8 BE patients treated with osteotomy and 6 BE patients treated without osteotomy. Orthopaedic evaluation included an assessment of pubic bones dissymmetry, bending of the spine, presence of Trendelemburg or Thomas sign, and presence of out-toeing. Pubic diastasis was ruled out with a plain x-ray of the pelvis. A Pediatric Orthopedic Society of North America (POSNA) questionnaire was administered to every child or his/her caregiver to assess functional outcome. Urological evaluation included an assessment of contemporary continence status and required continence surgery.ResultsAll patients presented a pubic diastasis. This was in median 49 (24 – 66) mm in patients treated without osteotomy and 42 (25 – 101) mm in those treated with osteotomy (p = 0.3). There was no difference either in the orthopaedic outcome or in any features of the POSNA questionnaire. Nor was there a difference in the final continence rate or in the number of additional continence procedures required.ConclusionsAlthough osteotmy is an essential step in the treatment of many BE patients in order to achieve a tension-free closure of the bladder and the abdominal wall, our preliminary RESULTS suggest that it does not improve the eventual orthopaedic or urological outcome of BE.     

Bilateral anterior pubic osteotomy in bladder exstrophy repair: report of increasing success

 Bilateral posterior iliac osteotomy is performed in most patients undergoing primary closure of an exstrophic bladder; the aims are to facilitate abdominal-wall closure, prevent postoperative wound dehiscene, and possibly, to achieve better urinary control in older age. A new technique, anterior pelvic osteotomy of the superior pubic ramus, seems to obtain tension-free symphysis approximation safely and quickly. We report our initial experience with this osteotomy. Five neonates, four males and one female from 1 to 4 days old, all underwent closure surgery for bladder exstrophy (BE) and subsequent bilateral osteotomy of the superior pubic ramus (SPRO). Postoperatively, Bryant's traction was applied. Tension-free, complete approximation of the symphysis and uncomplicated healing were achieved in all five cases without palsy of the obturator nerve or postoperative hemorrhage. Follow-up revealed partial rediastasis with a stable anterior pelvic ring. Tension-free closure and immobilization are important factors in both initial and subsequent closure of BE. Several osteotomy techniques are currently in use. SPRO presents numerous advantages, namely, ease and rapidity, minimal blood loss, and no requirement for an extra skin incision or need to turn the patient on the operating table. A certain degree of rediastasis with growth was subsequently observed: although undesirable, this complication is common to all osteotomy techniques. We believe that SPRO is a valid and uncomplicated method to facilitate BE closure. Accepted: 14 April 2000     

Comparison of musculoskeletal and urological functional outcomes in patients with bladder exstrophy undergoing repair with and without osteotomy

It is controversial as to whether osteotomy, by restoring a more normal pelvic anatomy, might improve the final outcome of bladder exstrophy (BE) repairs. We compared the functional orthopaedic and urological outcomes in BE patients treated with and without osteotomy. Orthopaedic and urological outcomes were compared in eight BE patients treated with osteotomy and six BE patients treated without osteotomy. Orthopaedic evaluation included an assessment of pubic bones dissymmetry, bending of the spine, presence of Trendelenburg or Thomas sign, and presence of out-toeing. Pubic diastasis was ruled out on a plain X-ray of the pelvis. A Pediatric Orthopedic Society of North America (POSNA) questionnaire was administered to every child or his/her caregiver to assess functional outcome. Urological evaluation included an assessment of required continence surgeries and of contemporary continence status. All patients presented a pubic diastasis. This was in median 49 (24-66) mm in patients treated without osteotomy and 42 (25-101) mm in those treated with osteotomy (p = 0.3). There was no difference either in the orthopaedic outcome or in any features of the POSNA questionnaire between groups. Neither was there a difference in the final continence rate nor in the number of additional continence procedures required. Although osteotomy is an essential step in the treatment of many BE patients in order to achieve a tension-free closure of the abdominal wall and bladder, our preliminary results suggest that it does not improve the eventual orthopaedic or urological outcomes of BE.     

The role of osteotomy in surgical repair of bladder exstrophy

Classic bladder exstrophy (CBE) patients are born with a pubic diastasis that increases steadily with age from a mean value of 4 cm at birth to a mean of 8 cm at age 10, compared with a mean normal width of the pubic symphysis of 0.6 cm at all ages. The width of the sacrum and length of the posterior (iliac) segment of the pelvis in CBE patients are normal; however, the anterior (ischiopubic) segment of the pelvis is a mean 30% shorter and both the anterior and posterior segments are externally rotated compared to controls. The main role of osteotomy in treatment of CBE appears to be to relax tension on the bladder and repaired abdominal wall during wound-healing. Anterior innominate osteotomy with optional posterior vertical iliac osteotomy presents several advantages over the prior conventional technique of posterior iliac osteotomy. These include (a) less intraoperative blood loss, (b) better apposition and mobility of the pubic rami at the time of closure, (c) allowance for placement of an external fixator under direct vision, (d) allowance for secure external fixation in children over 6 months old, and (e) no requirement to turn the patient during the operation.     

The use of pelvic osteotomy in cloacal exstrophy

Cloacal exstrophy is the most severe congenital malformation within the exstrophy spectrum. Its successful treatment requires a dedicated multidisciplinary exstrophy team that includes a pediatric orthopedic surgeon familiar with the exstrophy complex. In 1995, Ben-Chaim et al (J Urol 1995;154:865-7) reported an 89% complication rate in those cloacal exstrophy patients closed primarily without a pelvic osteotomy and a 17% complication rate in those closed with an osteotomy. Therefore, the use of pelvic osteotomy is a well-established method of obtaining a successful cloacal exstrophy closure. This article reviews the different options available for pelvic osteotomy and stabilization of the pubic symphysis in patients with cloacal exstrophy.     

Porcine acellular dermal matrix for delayed abdominal wall closure after pediatric liver transplantation

Children are one of the groups with the highest mortality rate on the waiting list for LT. Primary closure of the abdominal wall is often impossible in the pediatric population, due to a size mismatch between a large graft and a small recipient. We present a retrospective cohort study of six pediatric patients, who underwent delayed abdominal wall closure with a biological mesh after LT, and in whom early closure was impossible. A non‐cross‐linked porcine‐derived acellular dermal matrix (Strattice^? Reconstructive Tissue Matrix; LifeCell Corp, Bridgewater, NJ, USA) was used in all of the cases of the series. After a mean follow‐up of 26 months (21–32 months), all patients were asymptomatic, with a functional abdominal wall after physical examination. Non‐cross‐linked porcine‐derived acellular dermal matrix (Strattice^?) is a good alternative for delayed abdominal wall closure after pediatric LT. Randomized controlled trials are necessary to determine the best moment and the best technique for abdominal wall closure.     

Penile ischemic injury in the exstrophy/epispadias spectrum: New insights and possible mechanisms

ObjectivePartial or complete penile loss following bladder exstrophy and/or epispadias repair has been reported in the literature progressively more frequently.Patients and methodsThe authors report new cases of penile injury following bladder exstrophy and/or epispadias repair referred to their centers and not previously published. They review the literature on this subject and offer an explanation as to the likely mechanism for the penile injury and recommendations to avoid this complication.ResultsSeven new cases of partial or complete penile loss following bladder exstrophy or epispadias repair have been recently referred to the authors’ institutions. Twenty-one patients have previously been reported in the literature. Altogether, 24 cases occurred after bladder exstrophy closure: 23 after complete primary repair of exstrophy (Mitchell repair) and one after first-stage radical soft-tissue mobilization (Kelly repair). Nineteen of 24 patients did not have a pelvic osteotomy at the time of primary closure. Four cases occurred after epispadias repair: two following the second-stage radical soft-tissue mobilization (Kelly repair) and two following penile disassembly epispadias repair (Mitchell repair).ConclusionExstrophy closure combined with epispadias repair can be followed by ischemic penile injury, particularly when osteotomy is not performed. Compression of the pudendal vessels after pubic apposition and/or direct injury to the pudendal vessels play an important role in the pathogenesis of this complication.     

Combination of tissue expansion and porcine mesh for secondary abdominal wall closure after pediatric liver transplantation

Lafosse A, de Magnee C, Brunati A, Bayet B, Vanwijck R, Manzanares J, Reding R. Combination of tissue expansion and porcine mesh for secondary abdominal wall closure after pediatric liver transplantation. Abstract: We report the case of a two and a half yr boy hospitalized in our Pediatric Transplantation Unit for portal vein thrombosis following liver transplantation. After performing a meso‐Rex shunt, abdominal wall closure was impossible without compressing the portal flow. A combination of two techniques was used to perform the reconstruction of the muscular fasciae and skin layers. The association of tissue expanders and porcine mesh (Surgisis^®) allowed complete abdominal wall closure with good functional and esthetic results. Use of both techniques is a useful alternative for difficult abdominal closure after liver pediatric transplantation.     

Bony abnormalities in classic bladder exstrophy: The urologist’s perspective

IntroductionAs the primary practitioner managing patients with classic bladder exstrophy (CBE), it is incumbent upon the pediatric urologist to understand the associated orthopedic anomalies and their management.MethodsA Pubmed search was performed with the keyword exstrophy. Resulting literature pertaining to orthopedics and published references were reviewed.ResultsAnatomic changes to the bony pelvis include outward rotation, acetabular retroversion with compensatory femoral anteversion, anterior pubic shortening, and pubic diastasis. Imaging options have improved, which impacts surgical planning. Surgical approach, including type of osteotomy and method of pubic approximation, is evolving. Most centers employ immobilization after surgery, with external fixation, Bryant’s traction, Buck’s traction, and spica casting being the most common methods. Orthopedic complications range from minor pin-site infections to neurologic and vascular compromise. Most experts agree osteotomy aids bladder closure beyond 72 h of life, but effect on continence remains controversial. Although no significant orthopedic benefit has been expounded, it may be too early to appreciate improvement in frequency or severity of osteoarthritis or hip dysplasia.ConclusionWhile orthopedic surgeons remain vital to managing exstrophy patients, knowledge of the anatomy, imaging, surgical approaches, and immobilization enable effective communication with parents and other physicians, improving care for these complicated patients.     

Surgery in Legg-Calve-Perthes disease

A 10-year period of surgical management of Legg-Calve-Pethes disease (LCP) is reviewed. A total of 40 hips were operated on in 36 of 213 patients with LCP using four different techniques: femoral varus osteotomy, Salter's innominate osteotomy, Le Coeur's triple osteotomy, and Chiari's pelvic osteotomy. The choice of each technique was based on the clinical and roentgenographic anomalies present in each case. The benefits and disadvantages of these techniques in LCP are discussed. Overall results were judged as good in 65% of cases, fair in 23%, and poor in 12%. Offprint requests to: H. Bensahel at the above address     

Le Coeur骨盆截骨术在DDH治疗中的应用

目的介绍一种可用于治疗发育性髋关节发育不良（developmental dysplasia of the hip，DDH）的Le Coeur骨盆三联截骨术。方法本组9人10髋，平均手术年龄8岁7个月（6岁5个月～12岁11个月）。手术由股骨近端的短缩、内翻或／和旋转截骨与骨盆三联截骨两个部分组成。骨盆截骨经二个切口完成：内收肌入路截断耻骨上下支，髋前外侧入路截开髂骨；将耻骨上下支向后、内推移并使断端重叠，将截开的髂骨远端向下翻压、向前旋转；自体股骨干骨块或异体骨块嵌入髂骨截面作支撑，2～3枚克氏钢针固定；术后髋人字形模具固定6～8周。结果7人有保守复位史，3人有手术复位史。手术后及短期随访的X线片上的髋臼指数（acetabular index，AI）或Sharp角，Wiberg中心边缘角（center edge angle，CE），髋臼股骨头指数（acetabular-head-index，AHI）均有明显改善，闭孔的形态大小可基本恢复对称。结论Le Coeur骨盆三联截骨术操作简单，风险小，能使股骨头获得良好的覆盖，适合于大龄儿童的髋臼发育不良或半脱位。     

Aesthetic,urological, orthopaedic and functional outcomes in complex bladder exstrophy-epispadias's management

Background:

Postoperative complications are related to the surgical procedures, of failures of initial bladder closure and influence the urological, aesthetical and orthopaedic outcomes.

Materials and Methods:

We reviewed four patients who underwent complex bladder exstrophy-epispadias repair over a period of 14 years. The outcomes of treatment were assessed using, aesthetic, urological and orthopaedic examination data. Orthopaedic complications were explored by a radiography of the pelvis.

Results:

Out of four patients who underwent bladder exstrophy surgical management, aesthetic, functional outcomes and complications in the short and long follow-up were achieved in three patients. The first patient is a male and had a good penis aspect. He has a normal erection during micturition with a good jet miction. He has a moderate urinary incontinence, which requires diaper. In the erection, his penis-measures 4 cm long and 3 cm as circumference. The second patient was a female. She had an unsightly appearance of the female external genitalia with bipartite clitoris. Urinary continence could not be assessed; she did not have the age of cleanness yet. The third patient had a significant urinary leakage due to the failure of the epispadias repair. He has a limp, a pelvic obliquity, varus and internal rotation of the femoral head. He has an inequality of limbs length. Pelvis radiograph shows the right osteotomy through the ilium bone, the left osteotomy through the hip joint at the acetabular roof.

Conclusion:

When, the epispadias repair is performed contemporary to initial bladder closure, its success is decisive for urinary continence. In the female, surgical revision is required after the initial bladder closure for an aesthetic appearance to the external genitalia. Innominate osteotomy must be performed with brilliancy amplifier to avoid osteotomy through to the hip joint to prevent inequality in leg length.     

Gastroschisis and omphalocele

The survival rate of patients with abdominal wall defects has gradually improved with the advances in the investigation and treatment modalities. The present paper reviews the results of various treatment modalities and also analyses the long term results in these patients. A meta-analysis was performed via a medline search of English written clinical studies containing the text words “abdominal wall defects”, gastroschisis and “omphalocele or exomphalos” from 1953 to 1998. The present consensus on operative management of abdominal wall defect is to provide primary closure, if it can be achieved without haemodynamic or respiratory compromise. Patients with primary closure on analysis were found to have better survival rates, reduced risk of sepsis and overall, a shorter hospital stay. However, resumptions of oral feeds, duration of total parenteral nutrition (usually lasting 10–15 days) and ventilatory sgpport required postoperatively did not significantly differ in the primary and silo technique. Long term outcome of these patients is generally good, but they have high incidence of GER (40–50%) for which they should be on regular follow up.     

Staged approach for abdominal wound closure following combined liver and intestinal transplantation from living donors in pediatric patients

Abstract:  Primary closure of the abdominal wall after combined liver and intestine transplantation from a living donor into a pediatric patient is usually not possible, because of the size of the donor organ, graft edema, and preexisting scars or stomas of the abdominal wall. Closure under tension may lead to abdominal compartment syndrome with vascular compromise and necrosis of the transplanted organ. We describe our experience of abdominal wound closure after liver and intestinal transplant in the pediatric patient using a staged approach. From February 2003 to June 2006, we managed five pediatric liver and intestinal living donor transplant recipients. Because of the large post-transplantation abdominal wall defect, a staged technique of abdominal wound closure was utilized. Initially, an absorbable Polygalactin mesh was sutured around the layer of the defect. As soon as adequate granulation tissue was formed over the mesh a STSG was applied. From the wound stand point all five patients were managed successfully with staged wound closure after transplantation. Granulation tissue filled and covered the mesh within 7.6 wk. A STSG was then used to cover the defect. All infants recovered well and none had a significant wound complication in the immediate post-operative period following STSG. At a mean follow-up of 24 months only one patient developed an entero-cutaneous fistula five months post-transplant. Staged abdominal wall coverage with the use of Polygalactin mesh followed by STSG is a simple and effective technique. A closed wound is achieved in a timely fashion with protection of the viscera. Residual ventral hernia will need to be managed in the future with one of several reconstructive techniques.     

Patchplasty with expanded polytetrafluoroethylene and skinplasty for the closure of large congenital abdominal wall defects

A combined technique of patchplasty with expanded polytetrafluoroethylene (PTFE) and a special skinplasty is described for congenital abdominal wall defects where the abdominal cavity needs enlargement but skin closure is primarily possible. The patchplasty consists of suturing two half-moon-shaped, expanded PTFE leaves to the sides of the muscular abdominal wall, then approximating them under observation of intra-abdominal pressure by monitoring five parameters (urinary bladder pressure, central venous pressure, transcutaneous oxygen saturation, ventilation pressure, and mean arterial pressure). The skin is incised in a horizontal manner according to the tension lines. However, after closure, regional necrosis of the skin in the mid-portion above the incision is often observed due to lack of sufficient subcutaneous tissue at this site. Therefore, a skinplasty is done as a preventive measure, resecting this potential site of necrosis. The final appearance of the skin is an inverted T-shape. The surgical and monitoring techniques are described and illustrated. Correspondence to: A. Rokitansky     

骨盆三联截骨术治疗Perthes病的研究进展

Perthes病即儿童股骨头缺血性坏死.目前Perthes病治疗理念是"包容治疗",包容治疗的术式较多,常用的包括股骨近端内翻截骨术、骨盆Salter截骨术及骨盆三联截骨术,骨盆三联截骨术具有不影响股骨头颈干角及下肢力线、不增加髋关节压力、不改变髋臼形状等特点.各种三联截骨术入路及截骨方式不同、手术术式多,并且三联截骨...     

The outcome of newborns with abdominal wall defects according to the method of abdominal closure: the experience of a single center

Recent reports suggest that the technique of abdominal closure in neonates with anterior abdominal wall defects (AWD) correlates with the outcome. The aim of this study is to analyze factors related to mortality and morbidity, according to the technique of abdominal closure of these neonates. Retrospective analysis of charts from 76 consecutive neonates with AWD treated in a single institution. They were divided according to the type of abdominal wall closure: group I: primary closure, group II: silo followed by primary closure and group III: silo followed by polypropylene mesh. Outcome was analyzed separately for neonates with gastroschisis and omphalocele. There were 13 deaths (17.1%). Mortality for neonates with isolated defects was 9.6%. Mortality rate was similar in all groups for either neonates with gastroschisis or omphalocele. Postoperative complications were not significantly different among groups except for a prolonged time of hospitalization in group III. Mortality rate is not correlated with the type of abdominal closure. Neonates with primary closure or with other methods of abdominal wall closure had similar rate of postoperative complications. Neonates with mesh closure of the abdomen have prolonged hospitalization. The use of a polypropylene mesh is a good alternative for neonates whose primary closure or closure after silo placement is not possible.     

Addressing extreme size mismatch in pediatric intestinal transplantation: Outcomes of intestinal length reduction

Background

Bench liver reduction, with or without intestinal length reduction (LR) (coupled with delayed closure and abdominal wall prostheses), has been a strategy adopted by our program for small children due to the limited availability of size-matched donors. This report describes the short, medium, and long-term outcomes of this graft reduction strategy.

Methods

A single-center, retrospective analysis of children that underwent intestinal transplantation (April 1993 to December 2020) was performed. Patients were grouped according to whether they received an intestinal graft of full length (FL) or following LR.

Results

Overall, 105 intestinal transplants were performed. The LR group (n = 10) was younger (14.5 months vs. 40.0 months, p = .012) and smaller (8.7 kg vs. 13.0 kg, p = .032) compared to the FL group (n = 95). Similar abdominal closure rates were achieved after LR, without any increase in abdominal compartment syndrome (1/10 vs. 7/95, p = .806). The 90-day graft and patient survival were similar (9/10, 90% vs. 83/95, 86%; p = .810). Medium and long-term graft survival at 1 year (8/10, 80% vs. 65/90, 71%; p = .599), and 5 years (5/10, 50% vs. 42/84, 50%; p = 1.00) was similar.

Conclusion

LR of intestinal grafts appears to be a safe strategy for infants and small children requiring intestinal transplantation. This technique should be considered in the situation of significant size mismatch of intestine containing grafts.