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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.

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., (Copyright © 2018 Elsevier Ltd. All rights reserved.)

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.

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.

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.

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.

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.

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., (Copyright © 2012 Arthroscopy Association of North America. Published by Elsevier Inc. All rights reserved.)

A rare case of congenital entrapment of the left ureter in an iliac bone canal causing left side hydroureteronephrosis is reported. The patient represented a case of congenital ureteral entrapment in a reversed C-shaped iliac bone canal that was missed before his laparoscopy. The ureter also had a retro-iliac course. The patient was successfully managed by laparoscopic ureteroureterostomy. Interestingly, in our experience, laparoscopy provided a minimally invasive milieu for both diagnosis and correction of this rare cause of obstructive uropathy., (Copyright © 2012 Elsevier Inc. All rights reserved.)

The anatomy of the inferior vena cava (IVC) and common, external and internal iliac veins (CIV, EIV, IIV respectively) was examined in 59 embalmed adult human cadavers of Caucasian origin. In the present study, we focus our attention on the drainage patterns of the ascending lumbar and iliolumbar veins (ALV, ILV) when there are variations in the major pelvic veins by highlighting and describing the variations themselves. The above patterns, when no variations exist, have already been reported. Among the 59 cadavers, nine (9/59, 15.3%) presented anomalies of the major pelvic veins, as follows: anomalous drainage of both EIV and IIV in 8.5% of the specimens (5/59, Type I); variations of the IVC in 3.4% (2/59, Type II); duplication of the IIV (1/59=1.7%, Type III); anastomotic branch between the right IIV and the left CIV (1/59=1.7%, Type IV). ALV drained either into the CIV (8/18 sides, 44.4%) or in EIV (5/18 sides, 27.8%), while absence of ILV was the most frequent pattern (8/18 sides, 44.4%). A common trunk, joining the two veins, was observed in 5/18 sides (27.8%). Those drainage patterns were in accordance with the ones discovered in our previous study. In Type I the two veins always drained into the EIV. Knowledge of the surgical anatomy of all studied veins may prevent injury to these veins during operations in the retroperitoneal space and pelvis or malposition of venous catheters placed from the groin., (Copyright © 2012 Elsevier GmbH. All rights reserved.)

Patients with Type 1 Diabetes Mellitus (DM) have markedly increased risk of fracture, but little is known about abnormalities in bone microarchitecture or remodeling properties that might give insight into the pathogenesis of skeletal fragility in these patients. We report here a case-control study comparing bone histomorphometric and micro-CT results from iliac biopsies in 18 otherwise healthy subjects with Type 1 Diabetes Mellitus with those from healthy age- and sex-matched non-diabetic control subjects. Five of the diabetics had histories of low-trauma fracture. Transilial bone biopsies were obtained after tetracycline labeling. The biopsy specimens were fixed, embedded, and scanned using a desktop μCT at 16 μm resolution. They were then sectioned and quantitative histomorphometry was performed as previously described by Recker et al. [1]. Two sections, >250 μm apart, were read from the central part of each biopsy. Overall there were no significant differences between diabetics and controls in histomorphometric or micro-CT measurements. However, fracturing diabetics had structural and dynamic trends different from nonfracturing diabetics by both methods of analysis. In conclusion, Type 1 Diabetes Mellitus does not result in abnormalities in bone histomorphometric or micro-CT variables in the absence of manifest complications from the diabetes. However, diabetics suffering fractures may have defects in their skeletal microarchitecture that may underlie the presence of excess skeletal fragility., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Background: Bladder extrophy malformation includes outward rotation of the innominate bones, pubic symphysis diastasis and acetabular retrovertion. We have corrected the pelvic deformation during the reconstruction of the bladder neck and the urethra in older children., Methods: From 1/01/2002 to 1/01/2006 in the Department of Pediatric Urology and the Department of Pediatric Orthopedics in Katowice 4 children were operated on for bladder extrophy. The age of the patients ranged from 2.3 to 4.1 years; an average of 3.6 years. In the patients with bladder extrophy we performed the urological reconstruction and simultaneously the bilateral Salter's osteotomy was done., Results: The follow up was 52 months (48-84).The consolidation of the osteotomy was obtained in all patients after 62 days (48-72) with the correction of the retroversion of the hips and symphysis to 2.4 cm (0.9-5.8)., Conclusion: The innominate Salter's osteotomy is helpful in the bladder neck reconstruction and allows to achieve the proper anatomy of the pelvic ring.

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.

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.

Study Design: A 7-year-old ambulatory girl with complete bilateral agenesis of the ilium is reported., Objective: To document the clinical evolution and the spino-pelvic balance of a girl with bilateral agenesis of the ilium., Summary of Background Data: Scapuloiliac dysostosis is a rare focal skeletal dysplasia consisting in hypoplasia of ilium and scapula., Methods: A 7-year-old girl with a short stature was referred to our clinic at 35 months of age for an asymptomatic lumbar hyperlordosis. She presented a waddling gait, and normal range of motion of her upper and lower limbs. The neurologic examination was within normal limits., Results: Initial radiographs at 35 months of age showed complete bilateral agenesis of the ilium with preservation of the pubis, ischium, and sacrum, associated with a bilateral high hip dislocation. The lumbar lordosis and sacral slope were 89 degrees and 84 degrees, respectively. A radiographic skeletal survey also showed hypoplasia of both scapulae. At 7 years old, the girl was fully ambulatory, with a waddling gait and had no pain. The sacral slope and lumbar lordosis have increased to 97 degrees and 97 degrees, respectively. There was no evidence of spondylolisthesis. So far, the patient has required no treatment for her condition., Conclusion: This article reports a unique case of scapuloiliac dysostosis with complete bilateral agenesis of the ilium in an ambulatory girl. This case indicates that a functional gait is possible with bilateral congenital absence of the ilium, provided that there is adequate muscular support and normal neurologic function.

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.

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.

The shoulder and pelvic girdles represent the proximal bones of the appendicular skeleton that connect the anterior and posterior limbs to the body trunk. Although the limb is a well-known model in developmental biology, the genetic mechanisms controlling the development of the more proximal elements of the appendicular skeleton are still unknown. The knock-out of Pax1 has shown that this gene is involved in patterning the acromion, while the expression pattern candidates Hoxc6 as a gene involved in scapula development. Surprisingly, we have found that scapula and ilium do not develop in Emx2 knock-out mice. In the homozygous mutants, developmental abnormalities of the brain cortex, the most anterior structure of the primary axis of the body, are associated with important defects of the girdles, the more proximal elements of the secondary axis. These abnormalities suggest that the molecular mechanisms patterning the more proximal elements of the limb axis are different from those patterning the rest of appendicular skeleton. While Hox genes specify the different segments of the more distal part of the appendicular skeleton forming the limb, Emx2 is concerned with the more proximal elements constituting the girdles., (Copyright 2001 Academic Press.)

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.

Nail-patella syndrome is a rare hereditary (autosomal dominant) disorder, also called hereditary osteo-onychodysplasia and Fong's syndrome. Its incidence is 4.5 per million population in the United States. Patients have a characteristic tetrad of pathologic symptoms including fingernail dysplasia, hypoplastic or absent patellas, radial head dislocation, and iliac horns. Soft-tissue changes and renal dysplasia have also been associated with the syndrome. Iliac horns are bilateral accessory outgrowths consisting of cortex and medulla continuous with the iliac bone. They are located at the site of attachment of the gluteus medius muscles and project posterolaterally. These smooth bony outgrowths are asymptomatic, frequently palpable, and, because they have no effect on gait, they need not be treated. Iliac horns are the pathognomonic feature of Nail-patella syndrome; that is, they occur in approximately 80% of cases and are observed only in this condition. Four patients (two female, two male) with Nail-patella syndrome have been examined in the authors' department: three family members, including a 37-year-old woman, her 18-year-old son, and her 15-year-old daughter, and an unrelated 26-year-old man. All patients, regardless of age or sex, had similar pelvic findings on their bone scans. Although whole-body scans were obtained in all patients, significant scintigraphic findings were observed only in the pelvis in all the patients examined. This is most likely the result of the mild nature of the related deformities, which do not yield scintigraphically detectable osteoblastic changes. A representative image showing these independent ossification on a Tc-99m MDP bone scan is presented. A conventional pelvic radiographic image of the same patient's pelvis is presented for comparison.

The morphology of the iliac bone was assessed at the end of growth on AP x-rays of the pelvis in 21 children who had previously undergone unilateral pelvic osteotomy. The nonoperated side was used as a reference. There were 13 girls and 8 boys. Age at operation varied from 12 months to 12 years with a mean of 3 years and 10 months. The patients were distributed in 2 groups depending on their age at operation: before age 5 (group A, 16 cases) or after age 5 (group B, 5 cases). The mean age at follow-up was 15 years and 2 months (range 11-19 years). The end of pelvic growth was established by Risser stage IV. Distinct hypoplasia of the ilium due to premature growth arrest was observed in 16 cases: 12 in group A and 4 in group B. Other changes in the morphology of the ilium were noted, e.a. increased height of the ilium which was noted in 12 cases. The cosmetic prejudice was however minor, as compared with the radiological changes. In the authors' opinion, the alar hypoplasia was related to growth disturbances due to repeated splitting of the iliac apophysis. To prevent this complication the authors recommend avoiding the use of an electrocautery to incise the iliac apophysis and cutting the Kirschner wires so that their proximal ends lie within the subcutaneous fat, in order to avoid repeated splitting of the apophysis at the time of hardware removal.

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.

Purpose: To determine whether the axial pelvic profile is morphologically different in fetuses with Down syndrome from those with a normal karyotype., Materials and Methods: Pelvic images were selected from ultrasound studies in 27 fetuses with trisomy 21 and in 135 fetuses with a normal karyotype. An observer blinded to study results measured the angle formed by the convergence of lines drawn tangent to the wing of the ilium. This angle was measured prospectively in 20 normal fetuses by four independent observers to estimate variability., Results: The iliac bones could be assessed in 19 fetuses with trisomy 21 and in 87 fetuses with a normal karyotype. Between 15 and 20 weeks of gestation, the mean iliac angle was 60 degrees in normal fetuses and 75 degrees in fetuses with Down syndrome (P < .001). Intra- and interobserver correlation coefficients were .70 and .62, respectively. The greatest variability in results was among fetuses (estimated variance, 72.5); smaller variance was seen with repeat measurements in the same fetus (34.7) and with measurements by different observers (9.1)., Conclusion: The mean iliac angle in fetuses with Down syndrome is larger than that in fetuses without Down syndrome and may aid in weighing the risks of trisomy 21 against the risks of performing amniocentesis.

Dysplasia epiphysealis hemimelica is a rare developmental abnormality involving aberrant epiphyseal cartilage growth. This is the first known case report describing dysplasia epiphysealis hemimelica arising from the sacroiliac joint. The operative technique described through an indirect computed tomography guided approach limited the exposure and potential morbidity involving the sacroiliac joint.

Hereditary osteo-onychodysplasia, also known as the nail-patella syndrome (NPS), consists of a clinical tetrad of nail dysplasia, hypoplastic or absent patella, radial head dislocation, and iliac horns. Bilateral posterior iliac horns are observed only in NPS and present in >80% of patients. We describe the appearance, location, and structure of iliac horns by CT, MRI, and 3-dimensional CT reconstruction.

Nail-patella syndrome is an uncommon inherited disorder characterized by nail dysplasia, iliac horns, and knee and elbow abnormalities. We report a patient with nail-patella syndrome in whom calvicular horn, shoulder girdle dysplasias, and pelvic abnormalities were demonstrated, along with the classical tetrad.

We present a case of oculoauriculovertebral spectrum (hemifacial microsomia, Goldenhar's syndrome) found shortly after birth with right iliac hypoplasia, absent right pubic and ischial ossification centres and posterior urethral valves, which have not previously been described in this spectrum.

Bladder exstrophy and cloacal exstrophy present challenging reconstructive problems. Traditionally, bilateral posterior iliac osteotomy has been performed in the majority of patients undergoing primary closure for these abnormalities. Recently, 2 anterior osteotomy approaches have been proposed: 1) incision of the ilium transversely above the acetabulum (transverse osteotomy) and 2) incision of the superior ramus of the pubis (superior ramus osteotomy). We devised a new anterior mid-iliac diagonal osteotomy that provides a more functional pelvic closure. To provide a fair comparison of the procedures we developed a pelvic exstrophy model based on data from a 3-dimensional computerized tomogram of a 3-year-old patient with classical bladder exstrophy. The different osteotomies were then performed on the model and the results were compared. Our new anterior mid-iliac diagonal osteotomy correction allowed the best surgical approach, provided the best correction of the flattened ilia bony deformities, was performed in the thinnest area of marrow bone and resulted in the best pelvic symmetry.

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.

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)

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.

Of all the bony anomalies of the pelvis, unilateral developmental disturbances of the body of the iliac bone and the lateral parts of the os sacrum mainly affect the statics of the vertebral column. After a short description of the normal development of the os coxae and os sacrum, an attempt is made to systematize these anomalies and explain them from a developmental point of view. This is achieved by studying anatomical specimens and clinical cases. Primary disturbances concerning the growing of the cartilaginous epiphysis itself or secondary disturbances, which result from unilateral muscular palsy, can be responsible for retarded development of the body of the iliac bone. Unilateral dysplasia of the lateral part of the os sacrum could be based on disturbances of the precartilage. This is also thought to be the reason for asymmetrical formations of so-called transitional vertebrae.

Four familiar cases of the Nail-patella syndrome are reported in this article. All the patients displayed clinical and radiographic changes characteristic of this syndrome. All of them had elongated and sharp-pointed postero-inferior iliac prominences. This bony abnormality has not been previously described in this syndrome.

2 cases of a peculiar unilateral protuberance near the iliac bone with intraspinal abnormalities are reported. 8 cases have been described in the literature with this characteristic congenital association; they indicate the frequency of an occult spinal dysraphism which must be proved by myelography. In each case, surgical treatment of bone abnormality or of intraspinal lesions, or of both, should be discussed.

An anomaly of the pelvis consisting of a unilateral iliac horn is presented. It appeared as an isolated bony malformation which was not associated with signs, symptoms, or stigmata of hereditary onycho-osteodysplasia.

Congenital bone anomalies are occasionally located adjacent to deep lipomas. These bone dysplasias may take the form of localized overgrowth or osseous malformation. Two patients with iliac anomalies and spinal dysraphism and one with a hypertrophied rib and vertebra associated with lipomas are described. As both the lipomas and osseous anomalies tend to lie in the same sensory nerve distribution, there may be a common embryonic neural dysfunction affecting formation and subsequent growth of these structures.

Five cases of a rare developmental anomaly involving extraskeletal bone formation are described--four in the pelvis and one arising from the 12th rib. Three of these bony growths, called "Pelvic digits" had well developed "joints" or pseudoarticulations. The similarity of this anomaly to posttraumatic myositis ossificans and avulsion injuries of the pelvis (rectus femoris) is pointed out and a differential diagnosis offered.

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.