✓ Between 4% and 8% of cases of spina bifida cystica occur in a cervical or cervicothoracic location. Despite a large body of literature concerning spinal dysraphism, there has been little written specifically about patients afflicted with this disorder in a cervical location. Eight children who presented at birth with posterior cervical or cervicothoracic lumps, all of which represented a dysraphic state, are discussed. Two types of abnormalities were noted. Three patients had hydromyelia with an associated myelocystocele herniating posteriorly into a meningocele sac. In these three patients there was an associated Chiari II malformation and hydrocephalus. The other five children had a meningocele in which a band of tissue extended from the posterior aspect of the spinal cord through a defect in the bone and fascia to the posterior part of the meningocele sac itself. No patient had a lesion that could be described as a meningomyelocele. The investigation and surgical management of these conditions are discussed and the need for intradural exploration to untether the spinal cord in the cervical region is stressed.
Paul Steinbok and D. Douglas Cochrane
Deepak Agrawal, Paul Steinbok and D. Douglas Cochrane
Data from animal studies have shown that in experimentally induced craniosynostosis, removal of the involved calvaria results in the formation of new calvaria with time, and sutures redevelop in their normal anatomical positions. However, the pattern of suture reformation following surgery in humans with craniosynostosis remains ill-defined. The aim of this study was to determine the pattern of postoperative suture reformation in children who have undergone surgery for isolated sagittal synostosis and assess possible factors related to suture reformation.
Records were retrospectively reviewed for 42 consecutive infants who had surgery for isolated sagittal synostosis between 1987 and 2000 and for whom postoperative skull radiographs were available. The radiographs were evaluated for sagittal suture morphology and patency of the coronal and lambdoid sutures. Surgery involved at a minimum 1) a vertex craniectomy, characterized by removal of the sagittal suture and a 1.5- to 2.5-cm piece of adjacent parietal bone with the attached pericranium bilaterally, and 2) parietal osteotomies and/or craniectomies.
The median age at surgery was 3.9 months (range 1.9–7.6 months). The mean duration of follow up was 32.2 months (range 6–144 months). The sagittal suture had reformed in only seven (16.7%) of the children at follow up. In the other 35 (83.3%), the craniectomized bone defects had reossified without any part of the sagittal suture being visible on the radiographs.
There is a very low incidence of suture reformation in children after surgery for isolated sagittal craniosynostosis. Genetic predisposition, inclusion of undiagnosed syndromic patients, and current operative techniques may be some of the factors responsible for the low incidence of suture reformation seen in this series.
D. Douglas Cochrane
✓Tethering of the conus medullaris is assumed to be the primary cause of the deterioration seen in children with transitional lipomyelomeningocele (LMMC). The inevitability of deterioration has led to the use of prophylactic interventions to stabilize or prevent further clinical deterioration. The author reviewed current literature to define the timing and pattern of deterioration prior to and following initial cord untethering in patients with transitional LMMC, as well as the operative burden that these children bear in exchange for optimized function.
Stephen Hentschel, Paul Steinbok, D. Douglas Cochrane and John Kestle
Object. As public concern about the risks of blood transfusions increased in the mid-1990s, avoidance of transfusions became a goal of surgery for sagittal synostosis. This study was performed to confirm a hypothesized reduction in transfusion rates in recent years and to identify factors associated with both the need for transfusion and low postoperative levels of hemoglobin.
Methods. Sagittal synostosis operations performed in children between 1986 and 1999 were reviewed retrospectively. Patients underwent a minimum of vertex strip craniectomy and parietal craniectomies. There were 118 patients whose median age at surgery was 4.2 months. The primary end point for analysis was defined as either the receipt of a blood transfusion or a postoperative level of hemoglobin less than 70 g/L. Forty-two percent of patients (95% confidence interval [CI] 31–52%) treated before 1996 and 11% of patients (95% CI 0–23%) treated from 1996 onward received blood. The reduction in the blood transfusion rate in later years was, in part, related to the acceptance of a lower postoperative hemoglobin level, often below 70 g/L. A univariate analysis showed that the only patient or surgical factors that correlated with reaching the primary end point in a statistically significant manner were the year of surgery and the extent of surgery. A logistic regression of the age and weight of the child, length of surgery time (from skin opening to skin closure), preoperative hemoglobin level, extent of surgery, and surgeon against the primary end point revealed that the best predictor of the need for a blood transfusion or the presence of a postoperative hemoglobin level lower than 70 g/L was the extent of surgery (β = 1.4, standard error of the β statistic = 0.44). Once the extent of surgery was accounted for in the model, no other covariates significantly improved the model.
Techniques implemented to minimize blood loss since 1995 included the following: use of the Colorado needle for scalp incision, selection of the Midas Rex craniotome for cranial cuts, and application of microfibrillar collagen. Postoperative hemoglobin was allowed to decrease to 60 g/L if the child was stable hemodynamically, before blood was administered. There were no cardiovascular, wound healing, or infectious complications, and no surgeries were repeated for cosmetic reasons.
Conclusions. Low blood transfusion rates were achieved using simple intraoperative techniques and by accepting a low level of postoperative hemoglobin.
Monique Marguerie and D. Douglas Cochrane
Albert Tu, Alexander R. Hengel and D. Douglas Cochrane
Although patients with lumbosacral lipomas may be asymptomatic at presentation, most develop neurological symptoms over time. Given the challenges in examining infants, the authors sought to determine whether MRI would be helpful in identifying patients who are more likely to deteriorate early in life and who would potentially benefit from early surgical intervention.
A retrospective review of all patients with lumbosacral lipomas who were seen at the authors' institution between 1997 and 2013 and who were managed without prophylactic surgery was performed. The clinical history and imaging results for each patient were reviewed in detail and then correlated to the pattern of and age at clinical deterioration.
Twenty-four patients were identified. Nine worsened within the first 18 months of life (early deterioration), and 15 patients deteriorated or remained stable after 30 months (late deterioration/stable). No patients worsened between 18 and 30 months of age. Patients who deteriorated early were more likely to have large intradural lipomas that filled the canal, increased during the 1st year of life, and compressed neurological structures. Some had a syrinx extending above the neural-lipoma interface. Syrinxes in patients with early deterioration were large and expanded in infancy. Patients with early deterioration had motor deficits at the time of deterioration, whereas patients with late deterioration developed mixed urological and motor dysfunction.
Patients with large lipomas displacing the cord and an enlarging syrinx have a propensity for early clinical deterioration. Given this, their families may be counseled that 1) the risk of deterioration in infancy may be higher than in infants without these features, and 2) they require more diligent observation. Intervention before deterioration in these infants should also be considered. Patients without these features may be safely observed to a lesser extent.
Christopher M. Bonfield, D. Douglas Cochrane, Ash Singhal and Paul Steinbok
Sagittal craniosynostosis, the most common single suture craniosynostosis, is treated by numerous surgical techniques. Minimally invasive endoscopy-assisted procedures with postoperative helmeting are being used with reports of good cosmetic outcomes with decreased morbidity, shortened hospital stay, and less blood loss and transfusion. This procedure uses small skin incisions, which must be properly placed to provide safe access to the posterior sagittal and lambdoid sutures. However, the lambda is often hard to palpate through the skin due to the abnormal head shape. The authors describe their experience with the use of intraoperative, preincision ultrasound localization of the lambda in patients with scaphocephaly undergoing a minimally invasive procedure. This simple technique can also be applied to other operations where proper identification of the cranial sutures is necessary.