Atlantoaxial rotatory displacement in children.

AIM: To correlate the Pang and Lee class with the clinical course in a consecutive series of patients presenting with painful torticollis. METHODS: Forty-seven dynamic rotational computed tomography (CT) scans in 35 patients were classified into one of the five types defined by Pang and Li, including types I (atlantoaxial rotatopry fixation), II ("pathologic stickiness" without crossover of C1 on C2), III ("pathologic stickiness" with crossover of C1 on C2), IV (normal or muscular torticollis), and V (diagnostic grey zone). The Pang and Li class was then compared with the radiologist's report, which was graded abnormal, diagnosis of rotatory subluxation or fixation, or non-diagnostic. Medical records were reviewed and the clinical course was compared among the five sub-types. RESULTS: We reviewed 47 CT scans in 35 patients, and the majority were performed without sedation. The average age was 7.7 years (4-14 years old) and associated conditions included minor trauma (20%), surgical procedures around the head and neck (29%), and Grisels syndrome (20%). Twenty-six percent of our studies fell within the pathologic spectrum (5% type 1 or rotatory fixation, 21% types 2 and 3 or rotatory subluxation), while 45% were classified as muscular torticollis (45%) and 28% fell within the diagnostic grey zone. Seven radiologists interpreted these studies, and their interpretation was discordant in 45% of cases. Clinical resolution occurred in 27 of 29 cases for which follow-up was available. One of two patients with fixed rotatory subluxation required a C1-C2 arthrodesis. CONCLUSION: The Pang and Li classification characterizes a spectrum of abnormalities in rotation to facilitate communication, although the indications for dynamic CT scan should be further defined.

Right- versus left-sided congenital diaphragmatic hernia: a comparative outcomes analysis.

PURPOSE: Right-sided congenital diaphragmatic hernia (R-CDH) occurs in up to 25% of all CDH cases, but has been less widely studied compared to left-sided defects. We sought to compare characteristics and outcomes of left- versus right-sided defects in a large cohort of CDH patients who underwent standardized treatment and follow-up at a single institution. METHODS: We retrospectively reviewed charts of all CDH patients in our pulmonary hypoplasia program from January 2002 through December 2014. Categorical variables were analyzed by Fisher's exact test and continuous variables by Mann-Whitney t-test (p≤0.05). RESULTS: A total of 330 CDH patients were treated more than the 12-year study period, with 274 (83%) left-sided and 56 (17%) right-sided cases identified. Specific pulmonary morbidities were associated with R-CDH, with increased duration of nitric oxide therapy, increased requirement for tracheostomy, increased requirement for supplemental oxygen at the time of NICU discharge, and increased chronic pulmonary hypertension with requirement for long-term Sildenafil therapy. CONCLUSIONS: In this series, R-CDH was not associated with increased mortality, but was associated with increased requirement for pulmonary vasodilatory therapy and requirement for tracheostomy. The high incidence of pulmonary complications indicates increased severity of pulmonary hypoplasia in R-CDH, supporting a role for delivery in tertiary centers with expertise in CDH management.

Identifying expectant parents at risk for psychological distress in response to a confirmed fetal abnormality.

The aim of the study was to determine the incidence of psychological distress among expectant women carrying fetuses with prenatal diagnosed abnormalities and their partners. A 2-year retrospective medical chart review was completed of 1032 expectant mothers carrying fetuses with a confirmed anomaly, and 788 expectant fathers, who completed the CFDT Mental Health Screening Tool. Furthermore, 19.3 % of women and 13.1 % of men reported significant post-traumatic stress symptoms, and 14 % of men and 23 % of women scored positive for a major depressive disorder. Higher risk was noted among expectant parents of younger age and minority racial/ethnic status, and women with post-college level education and current or prior use of antidepressant medications. Heightened distress was noted within fetal diagnostic subgroups including neck masses, sacrococcygeal teratomas, neurological defects, and miscellaneous diagnoses. Incorporating screening tools into prenatal practice can help clinicians better identify the potential risk for psychological distress among expectant parents within high-risk fetal settings.

Chorioamniotic membrane separation and preterm premature rupture of membranes complicating in utero myelomeningocele repair.

BACKGROUND: Since the results of the Management of Myelomeningocele Study were published, maternal-fetal surgery for the in utero treatment of spina bifida has become accepted as a standard of care alternative. Despite promise with fetal management of myelomeningocele repair, there are significant complications to consider. Chorioamniotic membrane separation and preterm premature rupture of membranes are known complications of invasive fetal procedures. Despite their relative frequency associated with fetal procedures, few data exist regarding risk factors that may be attributed to their occurrence or the natural history of pregnancies that are affected with chorionic membrane separation or preterm premature rupture of membranes related to the procedure. OBJECTIVE: The objective of this study was to review chorioamniotic membrane separation and preterm premature rupture of membranes in a cohort of patients undergoing fetal management of myelomeningocele repair including identification of risk factors and outcomes. STUDY DESIGN: This was a retrospective review of patients undergoing fetal management of myelomeningocele repair and subsequent delivery from January 2011 through December 2013 at 1 institution. Patients were identified through the institutional fetal management of myelomeningocele repair database and chart review was performed. Perioperative factors and outcomes among patients with chorioamniotic membrane separation and preterm premature rupture of membranes were compared to those without. Risk factors associated with the development of chorioamniotic membrane separation and preterm premature rupture of membranes were determined. RESULTS: A total of 88 patients underwent fetal management of myelomeningocele repair and subsequently delivered during the study period. In all, 21 patients (23.9%) were diagnosed with chorioamniotic membrane separation by ultrasound and preterm premature rupture of membranes occurred in 27 (30.7%). Among the chorioamniotic membrane separation patients, 10 (47.6%) were diagnosed with global chorioamniotic membrane separation and 11 (52.4%) with local chorioamniotic membrane separation. Earlier gestational age at the time of fetal surgery was a significant risk factor for the development of chorioamniotic membrane separation (P = .01) and preterm premature rupture of membranes (P < 0.0001). Chorioamniotic membrane separation was significantly associated with preterm premature rupture of membranes (59.1% vs 21.2%, P = .008) and earlier gestational age at delivery (32.1 ± 4.2 vs 34.4 ± 3.5 weeks, P = .01). The average number of days from chorioamniotic membrane separation to preterm premature rupture of membranes was 11.0 ± 10.1 and from chorioamniotic membrane separation to delivery was 31.0 ± 22.5. The mean time interval between fetal management of myelomeningocele repair and preterm premature rupture of membranes was 47.9 days. Mean latency period from preterm premature rupture of membranes to delivery was 25 days. Gestational age at delivery was significantly lower in patients with preterm premature rupture of membranes (31.6 ± 3.4 vs 34.9 ± 3.5 weeks, P = .0001). Using logistic regression analysis, nulliparity, gestational age at fetal management of myelomeningocele repair, and membrane separation remained significant risk factors for preterm premature rupture of membranes. CONCLUSION: Chorioamniotic membrane separation after fetal management of myelomeningocele repair is a significant risk factor for subsequent development of preterm premature rupture of membranes and preterm delivery. Fetal management of myelomeningocele repair <23 weeks is associated with higher rates of preterm premature rupture of membranes and chorioamniotic membrane separation. Therefore fetal management of myelomeningocele repair should be deferred until ≥23 weeks to mitigate these complications. Nulliparity also appears to increase the risk for preterm premature rupture of membranes.

Fetal myelomeningocele repair: the post-MOMS experience at the Children's Hospital of Philadelphia.

BACKGROUND: Fetal myelomeningocele (fMMC) repair has become accepted as a standard of care option in selected circumstances. We reviewed our outcomes for fMMC repair from referral and evaluation through surgery, delivery and neonatal discharge. MATERIAL AND METHODS: All patients referred for potential fMMC repair were reviewed from January 1, 2011 through March 7, 2014. Maternal and neonatal data were collected on the 100 patients who underwent surgery. RESULTS: 29% of those evaluated met the criteria and underwent fMMC repair (100 cases). The average gestational age was 21.9 weeks at evaluation and 23.4 weeks at fMMC repair. Complications included membrane separation (22.9%), preterm premature rupture of membranes (32.3%) and preterm labor (37.5%). Average gestational age at delivery was 34.3 weeks and 54.2% delivered at ≥35 weeks. The perinatal loss rate was 6.1% (2 intrauterine fetal demises and 4 neonatal demises); 90.8% of women delivered at the Children's Hospital of Philadelphia and 3.4% received transfusions. With regard to the neonates, 2 received ventriculoperitoneal shunts prior to discharge; 71.1% of neonates had no evidence of hindbrain herniation on MRI. Of the 80 neonates evaluated, 55% were assigned a functional level of one or more better than the prenatal anatomic level. CONCLUSION: In an experienced program, maternal and neonatal outcomes for patients undergoing fMMC repair are comparable to results of the MOMS trial.

Surgical resection of heterotopic bone about the elbow: an institutional experience with traumatic and neurologic etiologies.

PURPOSE: We evaluated the outcomes of patients with elbow heterotopic ossification (HO) who underwent surgical intervention. Our goal was to elucidate differences in outcome of surgical treatment between those patients with traumatic brain injury, direct elbow trauma, or combined etiologies. In addition, we used regression analysis to adjust for confounding factors (such as age, gender, preoperative range of motion [ROM], location of HO, chronicity of HO [ie, time from HO formation to surgery], and whether motor control was spastic or normal) on the relationship between surgical outcome and etiology. METHODS: We reviewed 60 patients (64 elbows) surgically treated for heterotopic ossification. A total of 42 patients had trauma as the primary etiology, 15 had traumatic brain injury, and 7 had combined etiologies. All had pain or functional limitations at presentation. All patients had surgical resection of their HO. Functional and ROM outcomes were recorded. RESULTS: Mean preoperative arc of motion for the entire cohort was 57° (range, 0° to 150°). Mean postoperative arc for the entire cohort was 106° (range, 0° to 145°) at a mean follow-up of 44 months (range, 21-72 mo), demonstrating a significant gain. Average gain, in arc of motion was 49° (range, 10° to 140°). Gains in motion were not significantly different in any individual etiologic group. A total of 6% of cases were complicated by infection, 13% of cases had recurrence of HO, and 11% of cases required repeat surgery for infection or recurrence. Preoperative ROM was an important independent predictor of final range achieved and gain in ROM after surgical intervention. Recurrence rates were higher in patients with neurologic involvement. Postoperative stiffness was related to preoperative stiffness, delay of surgery longer than 12 months, and anterior location of the HO. CONCLUSIONS: Surgical excision of heterotopic bone about the elbow results in significant gains in ROM regardless of etiology. The likelihood of recurrence is higher in patients with central nervous system injuries than in patients with purely localized trauma.

Adolescent disc dysplasia and back pain.

PURPOSE: Adolescent disc dysplasia can be a cause of significant back pain and functional impairment in patients. We present a case series of patients inflicted with adolescent disc dysplasia (ADD). METHODS: A retrospective search was performed identifying patients presenting with ADD. Radiographic studies and advanced imaging were described. We documented presenting symptoms and clinical course. RESULTS: Six patients were identified. All patients presented with mechanical back pain, which worsened with flexion and extension. Magnetic resonance imaging was most accurate imaging modality. CONCLUSION: Among our patient cohort, treatment for adolescent disc dysplasia consisted of a combination of physical therapy and bracing. Neither approach proved to be very effective, with only one patient asymptomatic at follow-up.

The utility of posterior sloping angle in predicting contralateral slipped capital femoral epiphysis.

PURPOSE: Prophylactic pinning of the uninvolved side after unilateral slipped capital femoral epiphysis (SCFE) is controversial. The posterior sloping angle (PSA) has previously been proposed to predict contralateral slip. The purpose of this study was to determine whether the PSA can predict subsequent slip after unilateral SCFE, and if so, whether a sex difference exists. METHODS: A retrospective case-control study was performed comparing 51 patients who initially presented with unilateral SCFE and subsequently developed contralateral slip (Bilateral) with 51 patients who had unilateral SCFE only (Unilateral). Data collected included age, sex, ethnicity, and PSA. RESULTS: The patients in the Bilateral group had significantly higher PSA (14.5±6.1 vs. 10.6±5.3, P=0.001) and were younger (11.3±1.5 vs.12.3±1.2, P<0.001) than the patients in the Unilateral group. A receiver-operating characteristic curve demonstrated that the threshold for pinning a contralateral hip with PSA >12.66 yields an area under the curve of 67%. When the analysis was repeated with respect to sex, girls in the Bilateral group had significantly higher PSA (15.9±6.3 vs. 10.1±6.0, P=0.002) and were younger (10.7±1.1 vs.11.9±1.0, P<0.001) than the girls in the Unilateral group. Among boys, these associations were not significant. A receiver-operating characteristic curve demonstrated that the threshold for pinning a contralateral hip with PSA >13 in girls yields an area under the curve of 76%. CONCLUSIONS: PSA is predictive of contralateral slip in patients presenting with unilateral SCFE. However, it is more predictive in girls, and we recommend considering prophylactic pinning in girls with PSA >13. LEVEL OF EVIDENCE: Level III.