Pseudohypoparathyroidism: report on a family with four affected sisters.

A family consisting of a mother, a father with probable pseudopseudohypoparathyroidism (PPHP), two normal daughters, and four daughters with pseudohypoparathyroidism (PHP) have been observed for more than 15 years at North Carolina Memorial Hospital (NCMH). The studies performed on family members included (1) roentgenographic examinations of the chest, skull, hands, and soft tissues; (2) serum calcium, phosphorus, and immunoreactive parathyroid hormone measurements; (3) urinary cyclic adenosine 3'5'-monophosphate determinations following parathyroid injection; and (4) HLA and blood-type determinations. We review the genetic aspects of PHP. The findings in this family suggest an autosomal dominant mode of transmission in PHP.

Prolonged and functional survival with the asplenia syndrome.

Asplenia syndrome is a rare congenital complex of splenic agenesis, cardiac malformation, and malposition of the abdominal viscera. Prolonged functional survival is very uncommon, with death usually caused by severe infection or congestive heart failure. A 21-year-old man with typical asplenia syndrome who is currently asymptomatic and in his third year of college is described.

Clivus epidural hematoma: a case report.

An acute traumatic epidural hematoma extending from the odontoid process to the dorsum sella is described. The mechanism for the formation of an extradural hematoma in this unusual location seems to be related to age and a severe hyperflexion injury.

Posterior cervical arthrodesis and stabilization with a lateral mass plate. Clinical and computed tomographic evaluation of lateral mass screw placement and associated complications.

STUDY DESIGN: A prospective study evaluating screw position and associated complications in 21 consecutive patients treated with a plate and screw fixation system applied to the lateral masses of the cervical spine. OBJECTIVES: To determine the clinical safety of lateral mass screws by determining their anatomic location and clinical complications in a consecutive patient series. SUMMARY OF BACKGROUND DATA: Lateral mass plating has been advocated for procedures in which wiring techniques cannot be used, especially in instances in which the posterior elements are deficient. METHODS: The first 21 consecutive patients who underwent posterior cervical arthrodesis and lateral mass plating with a single fixation system were reviewed prospectively. Computed tomography scans taken after surgery were reviewed independently by an orthopedic spinal surgeon and by a radiologist to evaluate screw tip position. Clinical and radiographic outcome was assessed at each visit after surgery. RESULTS: Ten of 164 (6.1%) lateral mass screws were malpositioned in six patients. Three symptomatic patients underwent four additional operative procedures to remove or replace the malpositioned screws. All patients had radiographic union, and no patient developed mechanical implant failure requiring removal of instrumentation. Radiographic evaluation noted that 17% of the screws were in the central axial zone of the lateral mass on computed tomography. CONCLUSIONS: Lateral mass plating was associated with no vertebral artery or spinal cord injury. There was a 1.8%-per-screw risk of radiculopathy, which corresponds with published cadaveric studies. Radicular symptoms improved with screw removal in each case. The advantages of segmental fixation achieved with lateral mass plates and screws must be weighed against the risk of radiculopathy.

Outcome analysis of noninstrumented anterior cervical discectomy and interbody fusion in 348 patients.

STUDY DESIGN: Twenty-one outcome and outcome-relevant variables (fusion and patient satisfaction) were evaluated in a subset of 348 of 514 patients operated on by one surgeon during a 22-year period, using Cloward's anterior cervical discectomy and dowel interbody fusion. Minimum patient follow-up was 2 years; average length of follow-up was 5.2 years. This retrospective analysis is accompanied by a comprehensive review of the literature (1975-1996) of noninstrumented anterior cervical fusions, excluding fibular grafts alone in the interbody space. OBJECTIVES: To provide data on outcome (with regard to patient satisfaction and radiologically supported fusion) and risks of noninstrumented anterior cervical discectomy and fusion for intractable cervical nerve root and spinal cord compression symptoms at single or multiple levels, using the results from a single surgeon. METHODS: Three experienced spine radiologists determined fusion rates in one to five levels in 348 patients on the basis of the results of plain film analysis. Patient self-assessment was used to determine degree of patient satisfaction and other related variables. From a comprehensive review of the literature, 43 clinical reports meeting requirements for comparison of findings with those in the current study were selected from more than 1600 reports. RESULTS: The mean fusion rate for 348 patients in the current study ranged from 75% (multilevel) to 88% (one level; n = 202). The overall fusion rate was 83%. The persistent complication rate was 0.1%, and patient self-assessments showed that 78% were satisfied with the outcome and that 83% returned to work. The overall fusion rate for 2037 patients evaluated in the comprehensive literature review is 92%. CONCLUSIONS: Results of this study indicate that better outcome in noninstrumented anterior cervical discectomy and fusion is associated with solid fusion, fewer fused levels, nonsmoking patients, higher education levels, and absence of secondary economic gain. There was no correlation between fusion status and bone graft source or use of cervical collar.

Diagnostic spinal injection procedures.

The most important determinant of successful treatment in all spinal disorders is accurate diagnosis. Diagnostic injection procedures, such as facet blocks, selective nerve root blocks, and discography can aid in accurately diagnosing the source of a patient's pain.

Relationship of the pelvic angle to the sacral angle: measurement of clinical reliability and validity.

There is a need to better document the reliability and validity of assessment measures used in physical therapy. Studies documenting the reliability of measurement of the pelvic angle and its relationship to sacral motion are presently inconclusive. The purpose of this study was twofold. First, we wanted to determine the reliability and validity of a goniometric measurement of the pelvic angle. We also wanted to test the hypothesis that there is a relationship between the pelvic angle and the sacral angle. Intertester and intratester reliability of goniometric pelvic angle measurements of 23 healthy young adults were examined using three different raters. Radiographic measurements of the pelvic and sacral angle using two raters and goniometric measurement of the pelvic angle using a single rater were taken from 15 patients with low back pain who had been referred for X-rays. Intraclass correlation coefficients (ICCs) of intratester reliability for goniometric measurements of the pelvic angle were .93, .96, and .96. The intertester reliability was .95. The ICCs for intratester reliability for radiological measurements were .92 and .95 for the sacral angle and .98 for both measurements of the pelvic angle. Intertester reliability coefficients were .86 and .88, respectively. The Pearson correlation coefficients for the goniometric and radiological measurements of the pelvic angle were .85 and .68. A comparison of the radiological and goniometric measurements of the pelvic angle with the sacral angle demonstrated low average correlations of .43 and .58, respectively. The results indicate a high level of correlation between and within testers for goniometric measurements of the pelvic angle but only a fair correlation between goniometric and radiological measurements of the pelvic angle.(ABSTRACT TRUNCATED AT 250 WORDS)

Location of intrauterine devices: evaluation by computed tomography.

PIP: A 21-year old white multiparous woman who had undergone a Copper-7 IUD insertion 6 days before hospital admission for abdominal pain reported she could not locate her IUD string. A slight bleeding from the cervical os was noted upon pelvic examination, and no IUD string was found. Ultrasonic B-mode scanning was performed, but the pathognomonic signs of an IUD (e.g., shadowing or entrance-exit echoes) were not seen. A digital scout view, obtained with a GE CT/T 8000 scanner showed the IUD within the pelvis. Laparoscopy was performed to remove the IUD, and the patient recovered uneventfully. The previous definitive procedure for locating IUD position was hysterosalpingography (HSG) but this technique is now used only when the sonographic and plain radiographic findings are equivocal. CT is a much easier, faster, and less invasive procedure than HSG and just as definitive; it also has the advantage of lower patient irradiation, with about 2 rad (0.02 Gy) skin dose and about 0.2 rad (0.002 Gy) gonadal dose for a single section. Fluoroscopy during HSG may deliver 0.5-1.0 rad/minute (0.005-0.01 Gy/minute) to the gonads. Patient irradiation may be minimized by a digital scout view of the pelvis. It is possible to perform the entire examination with only 1 axial scan, as in this patient, by accurately aligning the scanner.^ieng

Venous air embolism.

This case report describes a new radiologic finding, that of an air-fluid level in the pulmonary artery. This is pathognomonic of venous air embolism when the patient has a chest film made in the upright position.

Application of magnetic resonance imaging and computerized tomography as an adjunct to the surgical management of soft tissue sarcomas.

Magnetic resonance imaging (MRI) and computed tomography (CT) scans of 53 evaluable patients with biopsy-proven soft tissue sarcomas were reviewed and compared with operative results to ascertain the accuracy for each imaging modality to predict resectability. Location of soft part sarcomas included: abdomino-pelvic (3), retroperitoneal (7), extremity (35), and other anatomic sites (8). MRI was observed to have greater accuracy than CT to preoperatively predict resectability (96.2% vs. 75.5%, respectively, p = 0.0034) following three-dimensional, multiplanar evaluation. Further, MRI was judged to have superior sensitivity to CT (95.6% vs. 73.3%, respectively, p = 0.006) and equivalent specificity (100% vs. 87.5%, respectively, p = 0.125). MRI represents a sophisticated diagnostic imaging technique to differentiate normal tissue from soft tissue sarcomas with superior contrast resolution in multiplanar imaging. MRI is considered to be the imaging modality of choice for these tumors with the advantage of not exposing the patient to ionizing irradiation or intravenous contrast agents to delineate contiguous structures.