A rare case of pelvic bone duplication.

Iliac bone malformations are rare and result from early disturbance of the genetic and epigenetic processes that come together to form the pelvic girdle. We report the case of a 5-month-old boy found to have a duplication of the ilium and describe the likely causes of this very rare malformation.

Transsacral Osseous Corridor Anatomy Is More Amenable To Screw Insertion In Males: A Biomorphometric Analysis of 280 Pelves.

BACKGROUND: Percutaneous iliosacral screw placement is the standard procedure for fixation of posterior pelvic ring lesions, although a transsacral screw path is being used more frequently in recent years owing to increased fracture-fixation strength and better ability to fix central and bilateral sacral fractures. However, biomorphometric data for the osseous corridors are limited. Because placement of these screws in a safe and effective manner is crucial to using transsacral screws, we sought to address precise sacral anatomy in more detail to look for anatomic variation in the general population. QUESTIONS/PURPOSES: We asked: (1) What proportion of healthy pelvis specimens have no transsacral corridor at the level of the S1 vertebra owing to sacral dysmorphism? (2) If there is no safe diameter for screw placement in the transsacral S1 corridor, is an increased and thus safe diameter of the transsacral S2 corridor expected? (3) Are there sex-specific differences in sacral anatomy and are these correlated with known anthropometric parameters? METHODS: CT scans of pelves of 280 healthy patients acquired exclusively for medical indications such as polytrauma (20%), CT angiography (70%), and other reasons (10%), were segmented manually. Using an advanced CT-based image analysis system, the mean shape of all segmented pelves was generated and functioned as a template. On this template, the cylindric transsacral osseous corridor at the level of the S1 and S2 vertebrae was determined manually. Each pelvis then was registered to the template using a free-form registration algorithm to measure the maximum screw corridor diameters on each specimen semiautomatically. RESULTS: Thirty of 280 pelves (11%) had no transsacral S1 corridor owing to sacral dysmorphism. The average of maximum cylindrical diameters of the S1 corridor for the remaining 250 pelves was 12.8 mm (95% CI, 12.1-13.5 mm). A transverse corridor for S2 was found in 279 of 280 pelves, with an average of maximum cylindrical diameter of 11.6 mm (95% CI, 11.3-11.9 mm). Decreasing transsacral S1 corridor diameters are correlated with increasing transsacral S2 corridor diameters (R value for females, -0.260, p < 0.01; for males, -0.311, p < 0.001). Female specimens were more likely to have sacral dysmorphism (defined as a pelvis without a transsacral osseous corridor at the level of the S1 vertebra) than were male specimens (females, 16%; males, 7%; p < 0.003). Furthermore female pelves had smaller-corridor diameters than did male pelves (females versus males for S1: 11.7 mm [95% CI, 10.6-12.8 mm] versus 13.5 mm [95% CI, 12.6-14.4 mm], p < 0.01; and for S2: 10.6 mm [95% CI, 10.1-11.1 mm] versus 12.2 mm [95% CI, 11.8-12.6 mm ], p < 0.0001). CONCLUSIONS: Narrow corridors and highly individual, sex-dependent variance of morphologic features of the sacrum make transsacral implant placement technically demanding. Individual preoperative axial-slice CT scan analyses and orthogonal coronal and sagittal reformations are recommended to determine the prevalence of sufficient-sized osseous corridors on both levels for safe screw placements, especially in female patients, owing to their smaller corridor diameters and higher rate of sacral dysmorphism.

The surgical outcome and the surgical strategy of percutaneous endoscopic discectomy for recurrent disk herniation.

STUDY DESIGN: A retrospective analysis. OBJECTIVE: To present the surgical outcome of percutaneous endoscopic discectomy (PED) for recurrent herniated intervertebral disk disease (HIVD) and to suggest a surgical strategy. SUMMARY OF BACKGROUND DATA: Revision discectomy is technically demanding because of the scar tissue, unclear anatomic planes, and retraumatization to the posterior structures. Although open microdiscectomy is a standard method, endoscopic techniques have emerged as a surgical alternative with comparable results. PED was performed with either the transforaminal (PETD) or the interlaminar approach (PEID). Previous reports have shown the surgical outcomes of PETD or PEID for recurrent HIVD, but the application of each approach was not addressed clearly. METHODS: Consecutive 26 patients (M:F=16:10, mean age 53.1±12.4 y), who underwent PED for recurrent HIVD, were enrolled. The previous operation was an open discectomy in 22, a PETD in 2, and a PEID in 2 patients. PETD was considered preferentially, if it was feasible (n=11), because of the scar tissue formed by the previous operation. PEID was chosen (n=15) because of a high iliac crest (8), high canal compromise (3), high-grade inferior migration (2), and narrow neural foramen (2). All patients were followed up for 19.3±11.3 months. RESULTS: In all patients, the recurrent disk material was removed successfully, and conversion to an open surgery was not necessary. Postoperative magnetic resonance imaging revealed that the ruptured disk was removed successfully in all cases. A favorable outcome (excellent or good outcome by MacNab's criteria) was achieved in 21 patients (81%). Re-recurrence occurred in 2 patients at 6 and 12 months postoperatively. Risk factors for an unfavorable outcome were not found in the present study (P>0.05). CONCLUSIONS: The relevant utilization of updated surgical techniques may be helpful in overcoming the difficulty of revision surgery.

The pelvic digit anomaly in a patient with multiple fractures: does it mimic the fracture?

Pelvic digit is a rare congenital anomaly where bone develops in the soft tissue adjacent to normal skeletal bone. Pelvic digits are most often associated with the ilium but may also pseudoarticulate with other pelvic bones or the abdominal wall. Its importance lies in its differentiation from acquired abnormalities due to trauma such as myositis ossificans and avulsion injuries of pelvis. In this article, we present a case of pelvic digit with multiple fractures. To avoid unnecessary investigation methods and treatment, this entity should be kept inmindwhen an atypical bone structure is noted around the pelvis.

Anterior inferior iliac spine deformity as an extra-articular source for hip impingement: a series of 10 patients treated with arthroscopic decompression.

PURPOSE: To describe an arthroscopic technique for decompression of a prominent anterior inferior iliac spine (AIIS) leading to extra-articular hip impingement and to provide short-term outcome after this procedure. METHODS: We retrospectively reviewed office charts, imaging studies, operative reports, arthroscopic images, preoperative and postoperative hip flexion range of motion, and preoperative and postoperative modified Harris Hip Scores in a consecutive series of 10 male patients who had arthroscopic decompression of symptomatic AIIS deformities leading to extra-articular hip impingement. The procedure was performed through standard anterolateral and mid-anterior hip arthroscopy portals that were also used to explore the joint and address concomitant intra-articular pathologies. RESULTS: The mean age was 24.9 years, with 8 of 10 patients aged younger than 30 years. In 9 patients, an anterior cam lesion was identified and decompressed before the AIIS decompression. The mean follow-up time was 14.7 months (range, 6 to 26 months). Hip flexion range of motion improved from 99° ± 7° before surgery to 117° ± 8° after surgery (P < .001). The modified Harris Hip Score improved from 64 ± 18 before surgery to 98 ± 2 at latest follow-up after surgery (P < .001). CONCLUSIONS: Arthroscopic decompression of a symptomatic AIIS deformity is a reproducible procedure that can provide excellent outcomes at short-term follow-up. As opposed to using an open approach for decompressing a prominent AIIS, an arthroscopic approach may be of particular value in patients with mixed intra- and extra-articular sources of hip dysfunction, because it enables the surgeon to address all pathologies with a single arthroscopic procedure. LEVEL OF EVIDENCE: Level IV, therapeutic case series.

Risser sign: the value of the lateral spinal radiograph to assess the excursion of the iliac apophysis.

The course of the ossification of the iliac apophysis is considered in adolescent patients with idiopathic scoliosis, under the name of the Risser sign, to determine the remaining spinal growth. Although the iliac crest develops in the three-dimensional space as a complex structure, the iliac apophysis ossification has been assessed only on a one plane frontal spinal radiograph. This study points out the usefulness of the lateral radiograph for the visualization of the whole iliac crest, especially the posterior region which otherwise cannot be observed. Two young female pelvis specimen were examined with anatomical measurements and radiography. Lateral spinal radiographs of 201 girls were analyzed for the iliac apophysis excursion. The measures of the width of the iliac bone beneath the iliac crest revealed one anterior and one posterior thick regions, coupled with an intermediate thin region. The regions of the maximal thickness corresponded to the earliest appearance of the apophysis ossification (Risser 1), while the thin part of the iliac bone corresponded to late appearance of the apophysis ossification (Risser 3-4). The ossification of the posterior part of the crest was best visualized with the lateral radiograph, which was exclusive in showing the posterior superior iliac spine region. On the frontal spinal radiograph the end of the course of the apophysis (Risser 3-4) is usually searched at the level of the sacroiliac joint, while in reality this point was found to be situated more caudal, and accessible for observation on the lateral radiograph.

Accuracy of sacroiliac screw placement with and without intraoperative navigation and clinical application of the sacral dysmorphism score.

INTRODUCTION: Percutaneously-placed sacroiliac (SI) screws are currently the gold-standard fixation technique for fixation of the posterior pelvic ring. The relatively high prevalence of sacral dysmorphism in the general population introduces a high risk of cortical breach with resultant neurovascular damage. This study was performed to compare the accuracy of SI screw placement with and without the use of intraoperative navigation, as well as to externally validate the sacral dysmorphism score in a trauma patient cohort. PATIENTS AND METHODS: All trauma patients who underwent sacroiliac screw fixation for pelvic fractures at a level 1 trauma centre over a 6 year period were identified. True axial and coronal sacral reconstructions were obtained from their pre-operative CT scans and assessed qualitatively and quantitatively for sacral dysmorphism - a sacral dysmorphism score was calculated by two independent assessors. Post-operative CT scans were then analysed for breaches and correlated with the hospital medical records to check for any clinical sequelae. RESULTS: 68 screws were inserted in 36 patients, most sustaining injuries from road traffic accidents (50%) or falls from height (36.1%). There was a male preponderance (83.3%) with the majority of the screws inserted percutaneously (86.1%). Intraoperative navigation was used in 47.2% of the patient cohort. 30.6% of the cohort were found to have dysmorphic sacra. The mean sacral dysmorphism scores were not significantly different between navigated and non-navigated groups. Three cortical breaches occurred, two in patients with sacral dysmorphism scores >70 and occurring despite the use of intraoperative navigation. There was no significant difference in the rates of breach between navigated and non-navigated groups. None of the breaches resulted in any clinically observable neurovascular deficit. CONCLUSION: The sacral dysmorphism score can be clinically applied to a cohort of trauma patients with pelvic fractures. In patients with highly dysmorphic sacra, reflected by high sacral dysmorphism scores, intraoperative navigation is not in itself sufficient to prevent cortical breaches. In such patients it would be prudent to consider instrumentation of the lower sacral corridors instead.

Use of the S3 Corridor for Iliosacral Fixation in a Dysmorphic Sacrum: A Case Report.

CASE: The S1 and S2 corridors are the typical osseous pathways for iliosacral screw fixation of posterior pelvic ring fractures. In dysmorphic sacra, the S1 screw trajectory is often different from that in normal sacra. We present a case of iliosacral screw placement in the third sacral segment for fixation of a complex lateral compression type-3 pelvic fracture in a patient with a dysmorphic sacrum. CONCLUSION: In patients with dysmorphic sacra and unstable posterior pelvic ring fractures or dislocations, the S3 corridor may be a feasible osseous fixation pathway that can be used in a manner equivalent to the S2 corridor in a normal sacrum.

Nail-Patella Syndrome: clinical and molecular data in 55 families raising the hypothesis of a genetic heterogeneity.

Nail-Patella Syndrome (NPS) is a rare autosomal dominant condition comprising nail and skeletal anomalies. Skeletal features include dysplastic patellae and iliac horns, as well as scapula and elbow dysplasia. Nephropathy and glaucoma or intra-ocular hypertension can sometimes be present. NPS is due to variants affecting function in LMX1B, which encodes a LIM-homeodomain protein critical for limb, kidney and eye development. We describe the phenotype and the molecular data of 55 index patients and their 39 relatives presenting with typical NPS. We identified 38 different LMX1B anomalies, 19 of which were not reported before. In our series, 9% of families are not carriers of a LMX1B genomic alteration after extensive study of the coding and non-coding regions of the gene. One of the families showed no linkage to the LMX1B locus, raising the hypothesis of a genetic heterogeneity.

Arthroscopic findings in the knee in nail-patella syndrome: a case report.

Nail-patella syndrome is a rare genetic disorder that can cause significant morbidity in several organs, including the musculoskeletal system. This case report describes our arthroscopic findings in the knees of an adolescent with bilateral hypoplastic and subluxed patellae. Our findings suggest that early intervention should be considered whenever this syndrome is recognized. This may modify some of the long-term joint problems seen in adults with nail-patella syndrome.

Fixation of bilateral pelvic osteotomies with external fixator in exstrophy bladder complex.

OBJECTIVE: To present the early results of pelvic osteotomies performed for repair of exstrophy bladder. METHODS: Five cases of exstrophy bladder were treated with closure following bilateral iliac osteotomies. Three patients underwent closure of pubic symphysis diastasis by use of external fixator, one by screws and cerclage wires, and one by use of K-wires and suture. The patients were followed up by the pediatric urologist and orthopedic surgeon. RESULTS: All patients achieved a closure of diastasis and a tension free repair after the index surgery. The average follow-up was 3.6 years with range of 4 months to 6 years. All osteotomies healed within two months and had closure of the diastasis, except one which had a partial failure with loss of 50% correction. No patient had any wound dehiscence or breakdown of the bladder repair. Preoperative mean diastasis of symphysis pubis was 6 cm (range; 4.5 cm to 7 cm) and post operative mean diastasis was 3.5 cm with the range of 2.5 cm to 4 cm at 12 months follow up. All patients achieved urinary continence post operatively and were passing urine per-urethra with satisfactory urinary control as followed-up with the pediatric urologists. CONCLUSION: Bilateral iliac osteotomies and use of external fixator in our series was found to be helpful in achieving a tension free closure and preventing dehiscence of the repair.

Impact of anomalous vertebral segmentation on measurements of bone mineral density.

In anteroposterior (AP) bone mineral density (BMD) measurements of the lumbar spine (LS), the presence of ribs is used to identify vertebra T12. Similarly, in lateral LS-BMD measurements, the position of the iliac crest is used to identify the lumbar vertebrae. The aim of this study was to determine the impact of variations in spinal segmentation and iliac crest position on BMD measurements. In 375 women (ages 50-85 years) radiographs were taken of the thoracic and lumbar spine, as well as AP measurement of LS-BMD, by dual-energy x-ray absorptiometry (DXA). In 121 subjects lateral decubitus LS-BMD was also measured. Anomalous spinal segmentation was found in 16.5%, and L1 would have been incorrectly identified on the AP-DXA image in 13%. The change in BMC and BMD between adjacent vertebrae was greater in the upper than the lower lumbar spine. Misidentification of L1 for T12 resulted in underestimation of the bone mineral content in grams (BMC) of L1 by a mean of 11.5 +/- 14.4% (SD; range -33.5 to 33.5%). For the usual region of interest, L2-4, the BMC (g) was underestimated by 8.4 +/- 8.7% (range -1.5 to 29.2%), with the BMD (g/cm2) underestimated by 3.6 +/- 4.8% (range -5.4 to 11.6%). The position of the iliac crest on the lateral decubitus DXA scans would have led to misidentification of either L2 or L4 for L3 in 15 cases (12%). This resulted in the BMD of L3 being underestimated by 2.7 +/- 19.4% (range -242.4 to 34.6%).(ABSTRACT TRUNCATED AT 250 WORDS)

Simpson-Golabi-Behmel syndrome: congenital diaphragmatic hernia and radiologic findings in two patients and follow-up of a previously reported case.

This report suggests the association of congenital diaphragmatic hernia in Simpson-Golabi-Behmel syndrome by describing two unrelated males with this malformation. One male was the maternal half-nephew of our previously reported 8-year-old boy with this syndrome. Review of the skeletal roentgenograms of these 2 affected males, and those of the previously reported 8-year-old, documents flare of the iliac wings, narrow sacroiliac notches, and the presence of two carpal ossification centers as a newborn ("advanced bone age"). We also report the follow-up of the 8-year-old boy, now 16 years old, who continues to have significant overgrowth and speech, dental, developmental, and adjustment problems.

Sacral agenesis.

Twenty-three patients with sacral agenesis evaluated at Newington Children's Hospital between 1959 and 1977 were classified according to patterns of morphologic deficiency in the bones and articulations. Motor deficit in these patients corresponded, within one level, to vertebral loss; sensory deficit did not correspond. The major orthopaedic problems often associated with sacral agenesis are spinopelvic instability, scoliosis, myelomeningocele, hip dislocation and contracture, knee contracture, and foot deformity. These can be controlled or corrected by proper orthopaedic management.

Anterior innominate osteotomy in repair of bladder exstrophy.

BACKGROUND: Classic bladder exstrophy is a developmental defect presenting at birth with a wide pubic separation and an exposed bladder; cloacal exstrophy involves, in addition, intestinal prolapse. Reconstruction requires several surgical procedures. The use of anterior iliac osteotomies in this process has not been reviewed in a large series. METHODS: We reviewed the results of eighty-six anterior innominate osteotomies performed in conjunction with genitourinary repair of classic and cloacal bladder exstrophy in eighty-two patients. Clinical outcome measures were successful bladder closure, achievement of continence, and maintenance of a normal gait. Radiographs of the pelvis were reviewed, and the pubic intersymphyseal diastasis (a measure of the reduction in tension on the anterior closure) was measured preoperatively and at three time-points postoperatively. Children with classic exstrophy who had undergone osteotomy and bladder neck reconstruction but not bladder augmentation were divided into four groups on the basis of the degree of continence. In addition, children with classic exstrophy were stratified according to age at the time of the osteotomy. The mean postoperative percent reduction in the amount of the original diastasis was determined for all groups. RESULTS: Children with classic exstrophy and those with cloacal exstrophy had correction of the diastasis after the osteotomy, with greater correction in those with classic exstrophy, presumably because of better bone quality. Daytime continence was achieved with anterior osteotomy and bladder neck reconstruction in 74% of the children for whom continence was a goal. However, no difference in the symphyseal diastasis or in the percentage of pubic reduction was detected among the four continence groups. Children who were older at the time of the osteotomy maintained better correction over time. Wound dehiscence or bladder prolapse occurred in 4% of the patients who had osteotomy and primary closure, and the only important complication of the osteotomies was transient palsy of the left femoral nerve in seven children. CONCLUSIONS: Anterior innominate osteotomy is an effective part of reconstructive repair of bladder exstrophy. The primary goals of the osteotomy are to reduce the tension in the closed bladder and the lower abdominal wall and to promote continence by restoring the sling of the pelvic floor muscles. These goals can be achieved in the majority of patients.

Accessory caudal axial and pelvic ribs.

Accessory caudal ribs are reported as an extremely curious anomaly in five patients. Once the fracture of this rib was a source of pains after injury. The different shapes of the ribs are documented in this clinical survey which is the most extensive in the present literature. Anomalous ribs arise due to inappropriate segmentation during the embryonal development of the axial skeleton.

A case with spondylo-metaphyseal dysplasia type A4.

We present a case with spondylo-metaphyseal dysplasia type A4 characterized by ovoid vertebral bodies with anterior tongue-like deformities, widened irregular and sclerotic metaphyseal changes, short iliac wings, slightly short long bones and short tubular bones of the hands with irregular metaphyses. She also had bipartite trochlea and irregular patellar margins, which have not been described in spondylo-metaphyseal dysplasia types to date.

Congenital deficiency of the fibula with ipsilateral iliac horn and absence of the kidney.

Congenital deficiency of the fibula is sometimes accompanied by femoral hypoplasia, genu valgum, patellar a/hypoplasia or dislocation, tibial bowing, foot deformity, and toe deficiency in the affected limb. 'Iliac horns' are bony projections extending posterolaterally from the ilium and considered to be pathognomonic of nail-patella syndrome. We report a 5-year-old Japanese girl with congenital complete deficiency of the left fibula, ipsilateral iliac horn and absence of the left kidney.

Positive personal identity of skeletonized remains using abdominal and pelvic radiographs.

Premortem and postmortem radiographs of the chest and abdomen are often available for comparison and provide a basis for making or rejecting an identification. The case reported here exemplifies the way that individualizing features, such as contours of bony elements, skeletal anomalies, and radiodensities and radiolucencies, are used in establishing personal identity.