Sprengel's deformity and spinal dysraphism: connecting the shoulder and the spine.

BACKGROUND AND PURPOSE: Sprengel's deformity, a rare congenital malformation of the scapula, may be observed in combination with spinal dysraphism. The co-occurrence of these malformations suggests an unknown shared etiology. Therefore, we reviewed the medical records of eight children presenting with both malformations and performed a review of the literature. PATIENTS AND METHODS: Databases from four university medical centers were searched for children presenting between 1992 and 2012 with spinal dysraphism and a Sprengel's deformity. CONCLUSION: The combination of spinal dysraphism and Sprengel's deformity is rare, and is associated with segmentation defects of the spine and ribs. Although the etiology of both spinal dysraphism and Sprengel's deformity remains unclear, all deformities of the spine, ribs, and shoulder might result from a common genetic defect affecting somitogenesis.

The spinal dermal-sinus-like stalk.

OBJECTS: In this study, a disjunction anomaly mimicking the spinal congenital dermal sinus (DS) is described. This anomaly is referred to as the dermal-sinus-like stalk. Dissimilarities between a true dermal sinus and a dermal-sinus-like stalk are discussed. CLINICAL MATERIAL: Three cases in which a spinal congenital dermal sinus was suspected are presented. A similar anatomical configuration, different from that of a dermal sinus, was found. All cases presented with a skin-covered dimple from which a solid tract was seen continuing intramedullary in two cases and intraspinally in one case. None of the patients presented with signs of infection or an associated dermoid-epidermoid tumor. Clinical, radiological, and surgical findings are discussed. A hypothesis is made on the pathological genesis of this malformation. CONCLUSION: A dermal-sinus-like stalk is a malformation similar to a spinal congenital dermal sinus but is not associated with DS-related complications. Despite important clinical, radiological, surgical, and histopathological differences, it is difficult to distinguish this malformation from a true DS based on clinical and radiological examination alone. Therefore, surgical intervention, at the time of diagnosis, is recommended in all cases.

Intraspinal dermoid and epidermoid tumors: report of 18 cases and reappraisal of the literature.

Intraspinal dermoid and epidermoid tumors are two histopathological subtypes of cutaneous inclusion tumors of the spine. This classification is based on obsolete embryological knowledge. In fact, according to current embryology, both tumor types consist of ectodermal derivatives. Therefore, we hypothesized that dermoid and epidermoid tumors do not differ in clinical practice. To explore this hypothesis, we studied the clinical, radiological and intraoperative findings of 18 patients, and related these findings to the histopathological characteristics of the tumor. No differences were found between dermoid and epidermoid tumors regarding clinical presentation, radiological examination and outcome, while intraoperative diagnosis by the surgeon correlated with the histopathological diagnosis in only 8 of 18 cases. Therefore, the histopathological difference between intraspinal dermoid and epidermoid tumors is not important in clinical practice and should be avoided. A new nomenclature is proposed in which both tumor types are referred to as 'spinal cutaneous inclusion tumors'.

Parental perceptions and morbidity: tracheostomy and Pierre Robin sequence.

OBJECTIVE: Evaluate parental perceptions associated with tracheostomy morbidity and quality of life in the management of Pierre Robin Sequence (PRS). STUDY DESIGN: Retrospective review/survey. METHODS: 42 Pierre Robin patients were identified, records were reviewed and airway assessments evaluated relative to airway compromise. Twenty patients had undergone tracheostomy. Perceptions of quality of life/morbidity related to tracheostomy were assessed using parental surveys. RESULTS: 31/41 (76%) patients participated in the survey. 15/31 (48%) of survey participants required tracheostomy and were decannulated after a mean of 28 months. Of the patients who had undergone tracheostomy, 10/15 (67%) had isolated Pierre Robin (iPRS) and the remaining 5/15 (33%) had syndromes associated with Pierre-Robin (sPRS). 9/10 (90%) iPRS and 4/5 (80%) sPRS families' expectations were met regarding expected duration of tracheostomy although 3/5 (60%) sPRS, and 8/10 (80%) iPRS described the overall experience as difficult. Of the 2/15 patient's families who were dissatisfied 1 patient had iPRS and the other sPRS. 9/15 (60%) required multiple > or = 3) hospitalizations. 3/13 (23%) reported airway problems after decannulation and 2/15 (13%) remained tracheostomy dependent at the time of survey. Prolonged tracheostomy duration represented a significant parental concern. CONCLUSIONS: A subset of patients required extended duration of tracheostomy; some continued to have airway problems after decannulation and/or distraction. Although some patients benefit from early mandibular distraction other Pierre Robin patients have multi-level obstruction requiring additional therapies and often tracheostomy. Parental concerns and perceptions relative to tracheostomy have not been adequately studied for Pierre Robin airway obstruction. Of those responding to this survey, the majority of parents' expectations were met regarding tracheostomy. Of those whose expectations were not met, it seems that better pre-intervention counseling regarding length of tracheostomy tube dependence, as well as a discussion about potential complications and hospitalizations frequently associated with prolonged tracheostomy, may lead to improved parental expectations.

GAND classification and volumetric assessment of unilateral cleft lip and palate malformations using cone beam computed tomography.

The aim of this study was to propose a classification for unilateral cleft lip and palate (UCLP) malformations based on cone beam computed tomography (CBCT) images, as well as to estimate the amount of bone necessary for grafting, and to evaluate the relationship of this volume with scores obtained using the classification. CBCT images of 33 subjects with UCLP were evaluated according to gap, arch, nasal, and dental parameters (GAND classification). Additionally, these defects were segmented and the amount of graft needed for alveolar bone grafting was estimated. The reproducibility of GAND classification was analyzed by weighted kappa test. The association of volume assessment with the classification (gap and nasal parameters) was verified using analysis of variance, while the intra-observer agreement was analyzed using the intra-class correlation coefficient. The intra-observer reproducibility of the classification ranged from 0.29 to 0.92 and the inter-observer agreement ranged from 0.29 to 0.91. There were no statistically significant values when evaluating the association of the volume with the classification (P>0.05). The GAND classification is a novel system that allows the quick estimation of the extent and complexity of the cleft. It is not possible to estimate the amount of bone needed for alveolar bone grafting based on the classification; individualized surgical planning should be done for each patient specifically.

Hydrogen sulfide inhalation injury.

Hydrogen sulfide is a colorless, noxious gas with the distinctive smell of rotten eggs. This compound is a powerful reducing agent that is encountered in a number of industrial processes. When hydrogen sulfide is present, it exposes workers to the potentially lethal effects of the rapid hypoxemia that results from exposure to this agent. The "warning sign" is the characteristic smell of rotten eggs; this smell should alert anyone in the area that a potentially serious risk exists. The immediate removal of the victim and administration of high-flow oxygen is essential. Neurologic sequelae may require anticonvulsants and care must be exercised to observe for cardiac, hepatic, and renal insufficiency. Depending on the concentration, hydrogen sulfide can rapidly overcome a potential victim.

Effects of lip revision surgery in cleft lip/palate patients.

The decision for lip revision surgery in patients with repaired cleft lip/palate is based on surgeons' subjective evaluation of lip disability. An objective evaluation would be highly beneficial for the assessment of surgical outcomes. In this study, the effects of lip revision on circumoral movements were objectively quantified. The hypothesis was that lip revision increases scarring and impairment. The study was a non-randomized clinical trial that included patients with cleft lip who had revision, patients with cleft lip who did not, and non-cleft control individuals. Three-dimensional facial movements were measured. Revision patients were measured before and after surgery. Other individuals were measured at similar intervals. Regression models were fit to summary measurements, and changes were modeled. Patients with repaired cleft lip/palate had fewer mean movements than control individuals. Lip revision did not worsen mean movements; however, individual patients' movements varied from 'improvement' to 'no change' to 'worse' relative to those of control individuals.

Anatomy and surgery of the infected dermal sinus of the lower spine.

OBJECT: Cases of infected dermal sinus are scarce and detailed surgical anatomical descriptions are hardly found in literature. The clinical, radiological, and surgical findings in four cases of an infected dermal sinus located at the lower spine are presented to elucidate the pathological anatomical configuration. CLINICAL MATERIAL: The first case showed two dermal sinuses with a parallel course extra- and intradurally, ending in a confluence of cavities connected to the conus. In this case, as well as in the fourth case, the signs and symptoms were those of meningitis. The second case presented with meningitis and a subdural empyema, while the third case presented with an intradermoid-intramedullary abscess at the junction between the DS and the conus. This child probably showed signs and symptoms of conus involvement as early as during pregnancy. CONCLUSION: The anatomy of the nervous elements in this congenital anomaly is heavily disturbed, more particularly in case of infection, due to extensive arachnoidal scarring. The latter renders dissection laborious and recognition of anatomical details difficult, resulting in complete excision of a dermal sinus in less than half of the cases. Despite their variability in presentation, most cases of an infected dermal sinus show similar characteristic features.

The intermediate type split cord malformation: hypothesis and case report.

METHODS: A patient is described in which a complete osteofibrotic dorsally implanted septum was found in combination with a split cord malformation in a single dural tube. This case cannot be explained using the widely used theory as proposed by Pang et al. [Pang D, Dias MS, Ahab-Barmada M (1992) Split cord malformation, part I: A unified theory of embryogenesis for double spinal cord malformations. Neurosurgery 31:451-480] but must be regarded as a combination of type I and II split cord malformation. RESULTS: The authors state that all types of split cord malformation can be reduced to a single derailment during development, with various degrees of severity. CONCLUSIONS: The configuration of the malformation is determined by the way the median parts of the mesoderm come to development. Type I and II split cord malformation are not distinct entities.

Adrenal suppression and growth retardation after injection of periocular capillary hemangioma with corticosteroids.

Adrenal suppression and/or growth retardation were noted in two patients after injection of periocular capillary hemangiomas with corticosteroids. Parents should be warned of these two potential complications of intralesional corticosteroid therapy. Baseline and posttreatment adrenal function should be monitored. If iatrogenic adrenal suppression occurs, supplemental systemic corticosteroids may be necessary in situations involving systemic stress such as infection or surgery.

The white blood cell response to splenectomy and bacteraemia.

The aim of this study was to determine the relationship of the white blood cell (WBC) count to bacteraemia, and the risk of bacteraemia after splenectomy. The case series study was carried out at the Vanderbilt University Level I Trauma Center, and included 11,870 consecutive trauma admissions: 258 required a splenectomy (191) or splenorrhaphy (67). Bacteraemia was defined as the presence of a positive blood culture. Statistical analysis included ANOVA, the non-parametric Kruskal-Wallis test and logistical regression. Forty-two (22 per cent) of the splenectomy patients had positive blood cultures, while only six (9 per cent) of the splenorrhaphy patients had positive cultures. For the group of patients requiring a splenectomy, the mean WBC count was higher and more persistent in patients with bacteraemia. Logistical regression demonstrated that the type of surgery does not significantly correlate with bacteraemia after accounting for severity of injury (TRISS). The study conclusions were as follows (1) for the group of patients with bacteraemia after splenectomy, the peak WBC count was higher and more persistent than that which occurred in the group of patients without bacteraemia after splenectomy; (2) the WBC count cannot be used to predict bacteraemia for an individual patient, but a WBC count greater than 20,000 after 10 days should initiate a vigorous search for infection; (3) the severity of injury (and not splenectomy/splenorrhaphy) accounted for the increased risk of bacteraemia in population studied.

Severely injured geriatric patients return to independent living: a study of factors influencing function and independence.

Our previous work demonstrated that geriatric trauma patients (age greater than 65 years) consume disproportionate amounts of health care resources. In the past we hypothesized that late mortality is high, long-term outcome is poor, and return to independence is low in a severely injured geriatric population. Of 6,480 trauma admissions over 5 years, geriatric patients (n = 495) with blunt trauma injury (n = 421) and an ISS greater than 16 (n = 105) who survived until discharge (n = 61) underwent long-term follow-up (mean = 2.82 years). We surveyed 20 measures of functional ability; 10 measures of independence; availability and use of rehabilitation resources; employment history; alcohol use; support systems; and nursing home requirements. Of the 105 patients, 7 were subsequently lost to follow-up. Among the remaining 98, 44 (44.9%) died in hospital and 54 (55.1%) were discharged and interviewed. The mean age of the contacted patients was 72.6; their mean ISS was 23.3. Forty eight of 54 (88.9%) were alive at the time of interview, while 6/54 (11.1%) had died. Although only 8/48 patients regained their preinjury level of function, 32/48 (67%) returned to independent living. The 32 independent patients, those with "acceptable" outcome, were compared with an "unacceptable" outcome group composed of the 44 in-hospital deaths, the 6 late deaths, and the 16 dependent patients. Factors associated with poor outcome include a GCS score less than or equal to (p = 0.001), age greater than or equal to 75 (p = 0.004), shock upon admission (p = 0.014), presence of head injury (p = 0.03), and sepsis (p = 0.03).(ABSTRACT TRUNCATED AT 250 WORDS)

Mechanism of dacron-activated monocytic cell oxidation of low density lipoprotein.

PURPOSE: Oxidized lipids are believed to contribute to atherogenesis and may play a role in the development of anastomotic intimal hyperplasia in prosthetic vascular grafts. This study examines the hypothesis that clinically relevant graft material activates monocytes to oxidize low density lipoprotein (LDL). METHODS: LDL and Dacron or expanded polytetrafluoroethylene (ePTFE) graft material were incubated in the presence of U937 cells, a monocytic cell line. LDL oxidation was measured by conjugated dienes, lipid peroxides, thiobarbituric acid-reacting substances, and electrophoretic mobility. Cell production of superoxide was measured by ferricytochrome c reduction. Metal ion requirement was assessed with the metal chelators, ethylenediaminetetra-acidic acid, deferoxamine, and bathocuproinedisulfonic acid. To determine whether human monocytes were capable of being activated by Dacron graft material to oxidize LDL, freshly isolated peripheral blood monocytes were also studied. RESULTS: Incubation of LDL with U937 cells and Dacron increased LDL oxidation by 5- to 20-fold. LDL incubated with ePTFE or U937 cells alone resulted in minimal oxidation. Dacron graft increased U937 cell production of superoxide by 4-fold, whereas ePTFE had no effect. Superoxide dismutase inhibited Dacron-activated U937 cell oxidation of LDL by greater than 50%, which indicates a role for superoxide. Ethylenediaminetetra-acidic acid, deferoxamine, and bathocuproinedisulfonic acid each inhibited Dacron-activated U937 cell oxidation of LDL. Human peripheral blood monocytes were activated by Dacron graft material to oxidize LDL; superoxide dismutase inhibited Dacron-activated human monocytic oxidation of LDL, which suggests a role for superoxide. CONCLUSION: These results suggest that Dacron graft material activates monocytes to oxidize LDL by a mechanism that involves superoxide and requires iron and copper ions. Our work suggests a mechanism by which lipids that have been deposited within implanted vascular grafts may become oxidized. Oxidized lipids may contribute to the cellular dysfunction that results in anastomotic intimal hyperplasia and graft failure.

Regional differences in phorbol 12-myristate 13-acetate stimulation of PDGF production in the normal canine aorta.

OBJECTIVE: Platelet-derived growth factor (PDGF) is a potent smooth muscle cell mitogen implicated in the development of intimal hyperplasia and atherosclerosis. A regional variation in canine aortic production of PDGF (greater in the distal than in the proximal aorta) was demonstrated previously in organ culture. The response of aortic segments in organ culture, as well as of aortic endothelial cells and smooth muscle cells, to stimulators of PDGF secretion-phorbol 12-myristate 13-acetate (PMA) and thrombin-was assessed to elucidate whether these regional variations were due to intrinsic differences in the abilities of cells to produce PDGF. METHODS: Proximal and distal aortic segments were removed from 10 dogs and placed in organ culture, then treated with PMA or thrombin for 72 hours. PDGF in the conditioned media was measured by radioreceptor assay. RESULTS: PDGF production in the distal, unstimulated aorta was 2.5-fold higher than that in the proximal aorta (P <.05). Treatment of the proximal aorta with 10 nmol/L and 100 nmol/L PMA increased PDGF production twofold and threefold, respectively, whereas no increase with PMA treatment was seen in the distal aorta. After thrombin treatment, no increase in PDGF production was noted in the proximal aorta and only a minimal increase was noted in the distal aorta. Endothelial cells and smooth muscle cells (n = 6) were cultured from four aortic segments (ascending thoracic, descending thoracic, abdominal, and infrarenal) and treated with PMA. PDGF production by unstimulated endothelial cells from the infrarenal aorta was 2.5-fold higher (P <.01) than that from the ascending thoracic aorta. With PMA treatment, PDGF secretion increased in endothelial cells from all segments, the greatest percentage increase being observed in the proximal segments. Thrombin also increased PDGF release from endothelial cells, but with no regional variation. Unstimulated smooth muscle cells did not exhibit regional variation in PDGF production and did not increase PDGF secretion after treatment with PMA or thrombin. CONCLUSIONS: These findings suggest that endothelial cells in the aorta may have a differential capacity to produce PDGF in response to stimulants, reflecting intrinsic differences in endothelial cells from the proximal aorta versus the distal aorta, and this may account in part for the propensity of the distal aorta to develop atherosclerosis.

Long-term follow-up of unsuccessful violent suicide attempts: risk factors for subsequent attempts.

STUDY POPULATION: Of 9046 consecutive trauma admissions, all suicide attempts (n = 156) were identified: 38 patients (24%) died in hospital; 118 (76%) were discharged and received long-term follow-up (mean = 2.8 years). Factors assessed included suicidal ideation and planning, reason for attempt; number of attempts, methods, dates of prior and subsequent attempts; psychiatric diagnoses, substance abuse history, treatment and medication compliance, hospitalizations, incidence of family depression and suicide; education level, job history, and living conditions. RESULTS: 104 (88%) patients were interviewed and 14 (12%) were lost to follow-up. Seventy-seven of the patients (74%) used guns in their attempt; their mean ISS was 14.2. Seven (6.7%) made repeat suicide attempts (all unsuccessful). Late mortality was 7% (one related to index suicide, five to chronic illness, one to motor vehicle crash). Most patients (96%) had psychiatric diagnoses at discharge, 77 of 93 (83%) had diagnosed depression. Sixty-six percent (69 of 104) had histories of alcohol abuse, 42% (42 of 101) histories of drug abuse. Thirty-five percent (34 of 96) were noncompliant with psychiatric follow-up and 70% (16 of 23) were noncompliant with alcohol abuse treatment. CONCLUSIONS: (1) Repeat attempts were rare (7%) after failed suicide attempts. (2) No late deaths resulted from repeat suicide attempts. (3) Risk factors associated with repeat attempts were younger age (p = 0.002), prior attempts (p = 0.02), family history of suicide (p = 0.03), schizophrenia (p = 0.005), and not living at home (p = 0.04). (4) Identifying patients with these risk factors, ensuring that they receive inpatient alcohol abuse treatment, along with sustained psychiatric treatment and help in maintaining home environments, may prevent repeat suicide attempts.

Platelet-derived growth factor production by cells from Dacron grafts implanted in a canine model.

PURPOSE: Previous studies of grafts implanted in dogs documented a time-dependent increase in platelet-derived growth factor (PDGF) production that correlated with inner-capsule thickness. The purpose of this study was to identify the cells in vascular grafts that produce PDGF. METHODS: Dacron thoracoabdominal grafts were seeded with autologous endothelial cells (ECs), implanted in 11 beagles, and removed after 4 or 20 weeks. ECs and smooth muscle cells (SMCs) were cultured from grafts and adjacent aorta, and PDGF in the conditioned media was measured by radioreceptor assay. The PDGF A-chain mRNA level in freshly harvested cells was assessed using reverse transcriptase, followed by polymerase chain reaction, and expressed as a ratio of glyceraldehyde-3-phosphate dehydrogenase signal. Localization of PDGF A-chain and B-chain protein was also examined with immunohistochemical analysis. RESULTS: Graft and aortic ECs in primary culture did not produce significantly different amounts of PDGF in 72 hours, averaging 368 +/- 160 and 340 +/- 81 pg/microgram DNA, respectively. Graft SMCs in primary culture produced significantly more PDGF than aortic SMCs (584 +/- 343 and 113 +/- 94 pg/microgram DNA, respectively; p < 0.01). Graft SMC PDGF secretion remained greater than aortic SMC PDGF secretion through at least six cell passages. PDGF A-chain mRNA levels were not significantly different for aortic or graft ECs. The PDGF A-chain mRNA level was significantly higher for graft SMCs than aortic SMCs (2.44 +/- 0.67 and 1.45 +/- 0.57 pg/microgram, respectively; p < 0.03). Immunocytochemical analysis detected PDGF A-chain and B-chain protein in the ECs from both native aorta and graft as well as the subendothelial SMCs in the graft, but not in the SMCs of the native aorta. CONCLUSIONS: These results suggest that graft SMCs are functionally altered, producing more PDGF than aortic SMCs. PDGF produced by graft SMCs may contribute to the development of intimal hyperplasia.

The migratory response to platelet-derived growth factor of smooth muscle cells isolated from synthetic vascular grafts in a canine model.

OBJECTIVE: Previous studies on smooth muscle cells (SMCs) harvested from implanted synthetic grafts demonstrate increased production of platelet-derived growth factor (PDGF) but decreased proliferative response compared with aortic SMCs. The purpose of this study was to determine the migratory response of graft versus aortic SMCs. METHODS: Thoracoabdominal grafts were implanted in beagles. The SMCs were harvested from the graft and infrarenal aorta. Migration was determined with the use of a razor-scrape assay and computerized image analysis. RESULTS: The mean distance migrated and the number of cells that migrated were greater in graft SMCs at baseline (185 +/- 18 micrometer and 108 +/- 17 cells) compared with aortic cells (110 +/- 10 micrometer and 42 +/- 5 cells)(P <.05). Baseline differences persisted after treatment with antibodies to PDGF. The addition of PDGF (10 ng/mL) resulted in increased migration in both graft (229 +/- 23 micrometer and 146 +/- 20 cells) and aortic SMCs (130 +/- 9 micrometer and 70 +/- 5 cells) compared with baseline (P <.05). The relative increase in response to PDGF was similar between the two groups (P = not significant). CONCLUSIONS: Graft SMCs differ phenotypically from aortic SMCs; they exhibit increased basal migration that is independent of autocrine stimulation by PDGF. In contrast to their blunted proliferative response, graft SMCs have a similar migratory response to PDGF compared with aortic SMCs.

The proliferative response to platelet-derived growth factor of smooth muscle cells isolated from synthetic vascular grafts in a canine model.

OBJECTIVE: Previous studies on graft healing have shown increased platelet-derived growth factor (PDGF) production in graft segments versus native aortic segments. The purpose of this study was to characterize the proliferative response of graft smooth muscle cells (SMCs) to PDGF. METHODS: Thoracoabdominal grafts were implanted in beagles. SMCs were harvested from the graft and the proximal and distal aortas. Basal proliferation was assessed with growth curves in primary culture. The proliferative response to PDGF then was compared with [3H]thymidine uptake studies and cell counts. Finally, PDGF receptors were characterized with radio-labeled ligand binding assays. RESULTS: The growth curves showed that the graft SMCs entered log-phase growth 2 days earlier than did the aortic SMCs. Stimulation of quiescent early-passage graft SMCs with PDGF (10 ng/mL) resulted in a 1.7 +/- 0.1-fold increase in [3H]thymidine incorporation, which was significantly less than that of the SMCs from both the proximal aorta (11.8 +/- 3.0) and the distal aorta (10. 2 +/- 1.9; P <.5). Similarly, the 1.1 +/- 0.1-fold increase in graft SMC cell number was significantly less than the increases for both proximal (2.8 +/- 0.5) and distal (2.9 +/- 0.8) aortic SMCs (P <.5). Binding studies on quiescent first-passage cells showed fewer PDGF receptors available for binding in the graft SMCs (185 +/- 70 fmol/million cells) as compared with both the proximal (419 +/- 147 fmol/million cells) and the distal (387 +/- 112 fmol/million cells) aortas (P <.5). Binding affinity was similar for the three groups. CONCLUSION: Graft SMCs exist in a chronic proliferative state but exhibit a decreased proliferative response to PDGF and have fewer receptors available for binding PDGF than do aortic SMCs in vitro.