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Cranial fetus in fetu

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Gregory G. Heuer, Erin S. Schwartz and Phillip B. Storm

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Jennifer Hong, Jared M. Pisapia, Zarina S. Ali, Austin J. Heuer, Erin Alexander, Gregory G. Heuer and Eric L. Zager

OBJECTIVE

Neurogenic thoracic outlet syndrome (nTOS) is an uncommon compression syndrome of the brachial plexus that presents with pain, sensory changes, and motor weakness in the affected limb. The authors reviewed the clinical presentations and outcomes in their series of pediatric patients with surgically treated nTOS over a 6-year period.

METHODS

Cases of nTOS in patients age 18 years or younger were extracted for analysis from a prospective database of peripheral nerve operations. Baseline patient characteristics, imaging and neurophysiological data, operative findings, and outcomes and complications were assessed.

RESULTS

Twelve patients with 14 cases of nTOS surgically treated between April 2010 and December 2016 were identified. One-third of the patients were male, and 2 male patients underwent staged, bilateral procedures. Disabling pain (both local and radiating) was the most common presenting symptom (100%), followed by numbness (35.7%), then tingling (28.6%). The mean duration of symptoms prior to surgery was 15.8 ± 6.6 months (mean ± SD). Sports-related onset of symptoms was seen in 78.6% of cases. Imaging revealed cervical ribs in 4 cases, prominent C-7 transverse processes in 4 cases, abnormal first thoracic ribs in 2 cases, and absence of bony anomalies in 4 cases. Neurophysiological testing results were normal in 85.7% of cases. Conservative management failed in all patients, with 5 patients reporting minimal improvement in symptoms with physical therapy. With a mean follow-up after surgery of 22 ± 18.3 months (mean ± SD), pain relief was excellent (> 90%) in 8 cases (57.1%), and good (improved > 50%) in 6 cases (42.9%). On univariate analysis, patients who reported excellent pain resolution following surgery at long-term follow-up were found to be significantly younger, and to have suffered a shorter duration of preoperative symptoms than patients who had worse outcomes. Lack of significant trauma or previous surgery to the affected arm was also associated with excellent outcomes. There were 4 minor complications in 3 patients within 30 days of surgery: 1 patient developed a small pneumothorax that resolved spontaneously; 1 patient suffered a transient increase in pain requiring consultation, followed by hiccups for a period of 3 hours that resolved spontaneously; and 1 patient fell at home, with transient increased pain in the surgically treated extremity. There were no new neurological deficits, wound infections, deep vein thromboses, or readmissions.

CONCLUSION

Pediatric nTOS commonly presents with disabling pain and is more frequently associated with bony anomalies compared with adult nTOS. In carefully selected patients, surgical decompression of the brachial plexus results in excellent pain relief, which is more likely to be seen in younger patients who present for early surgical evaluation.

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Sophie Foss, Tracy M. Flanders, Gregory G. Heuer and Jane E. Schreiber

This paper describes some of the more common patterns in neurobehavioral deficits and their underlying neuroanatomical basis in myelomeningocele (MMC). Patients with MMC can face a lifetime of specific organ system dysfunction, chief among them spinal cord malformations, orthopedic issues, hydrocephalus, and urological disabilities. In addition, patients can experience specific patterns of neurobehavioral difficulties due to the changes in neuroanatomy associated with the open spinal defect. Although there is variability in these patterns, some trends have been described among MMC patients. It is thought that early recognition of these potential neurobehavioral deficits by treating neurosurgeons and other members of the treatment team could lead to earlier intervention and positively impact the overall outcome for patients. Neurodevelopmental and neurobehavioral follow-up assessments are recommended to help guide planning for relevant treatments or accommodations.

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Gregory G. Heuer, Michael F. Stiefel, Robert L. Bailey and James M. Schuster

✓Spinal ependymomas are a common type of primary spinal cord neoplasm that frequently occurs in the lumbar spine. The authors report on two patients who presented with acute neurological decline after hemorrhage into ependymomas of the filum terminale. Both were transferred to the authors' institution because of diagnostic uncertainty and a concern about possible intradural vascular abnormalities. Both patients underwent lumbar laminectomies for tumor resection. The pathological finding in each case was myxopapillary ependymoma. Both patients made a significant recovery and were ambulatory and continent at follow-up review. These cases illustrate the rare but clinically significant incidence of acute neurological decline caused by hemorrhagic cauda equina ependymomas, including the potential for delayed diagnosis and treatment.

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Gregory G. Heuer, Michelle J. Smith, J. Paul Elliott, H. Richard Winn and Peter D. Leroux

Object. Increased intracranial pressure (ICP) is well known to affect adversely patients with head injury. In contrast, the variables associated with ICP following aneurysmal subarachnoid hemorrhage (SAH) and their impact on outcome have been less intensely studied.

Methods. In this retrospective study the authors reviewed a prospective observational database cataloging the treatment details in 433 patients with SAH who had undergone surgical occlusion of an aneurysm as well as ICP monitoring. All 433 patients underwent postoperative ICP monitoring, whereas only 146 (33.7%) underwent both pre- and postoperative ICP monitoring.

The mean maximal ICP was 24.9 ± 17.3 mm Hg (mean ± standard deviation). During their hospital stay, 234 patients (54%) had elevated ICP (> 20 mm Hg), including 136 of those (48.7%) with a good clinical grade (Hunt and Hess Grades I–III) and 98 (63.6%) of the 154 patients with a poor grade (Hunt and Hess Grades IV and V) on admission. An increased mean maximal ICP was associated with several admission variables: worse Hunt and Hess clinical grade (p < 0.0001), a lower Glasgow Coma Scale (GSC) motor score (p < 0.0001); worse SAH grade based on results of computerized tomography studies (p < 0.0001); intracerebral hemorrhage (p = 0.024); severity of intraventricular hemorrhage (p < 0.0001); and rebleeding (p = 0.0048). Both intraoperative cerebral swelling (p = 0.0017) and postoperative GCS score (p < 0.0001) were significantly associated with a raised ICP. Variables such as patient age, aneurysm size, symptomatic vasospasm, intraoperative aneurysm rupture, and secondary cerebral insults such as hypoxia were not associated with raised ICP. Increased ICP adversely affected outcome: 71.9% of patients with normal ICP demonstrated favorable 6-month outcomes postoperatively, whereas 63.5% of patients with ICP between 20 and 50 mm Hg and 33.3% with ICP greater than 50 mm Hg demonstrated favorable outcomes. Among 21 patients whose raised ICP did not respond to mannitol therapy, all experienced a poor outcome and 95.2% died. Among 145 patients whose elevated ICP responded to mannitol, 66.9% had a favorable outcome and only 20.7% were dead 6 months after surgery (p < 0.0001). According to results of multivariate analysis, however, ICP was not an independent outcome predictor (odds ratio 1.26, 95% confidence interval 0.28–5.68).

Conclusions. Increased ICP is common after SAH, even in patients with a good clinical grade. Elevated ICP post-SAH is associated with a worse patient outcome, particularly if ICP does not respond to treatment. This association, however, may depend more on the overall severity of the SAH than on ICP alone.

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Joel A. Bauman, Douglas A. Hardesty, Gregory G. Heuer and Phillip B. Storm

An alternative method of bone grafting for pediatric posterior cervical and occipitocervical fixation is presented in detail. Full-thickness autografts from small craniectomies of the occipital bone are used to augment posterior segmental fusion in pediatric patients. Twelve patients have been treated successfully without bone graft donor site complications. The technical differences from previously reported uses of calvarial autograft in spine fusion are reviewed.

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Zarina S. Ali, Eric L. Zager, Gregory G. Heuer and Sherman C. Stein

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Eamon J. McLaughlin, Gregory G. Heuer, Robert G. Whitmore, John K. Birknes, Jean Belasco, Daniel Sterman, David W. Low and Phillip B. Storm

The authors report the case of a 14-year-old girl with a residual malignant peripheral nerve sheath tumor after thoracotomy, chemotherapy, and radiation therapy. The residual tumor, which involved the intercostal muscles, aorta, and neural foramina of T4–10, was completely resected through a costotransversectomy and multiple hemilaminotomies with the patient in the prone position and was stabilized using a T1–12 pedicle screw fusion. Postoperatively, the patient developed several infections requiring multiple washouts and prolonged antibiotics. Thirty months after surgery, she developed a bronchocutaneous fistula. The hardware was removed, and a vascularized latissimus dorsi free flap was placed over the lung. She continued to have an air leak and presented 3 weeks later with a 40° left thoracic curve. She returned to the operating room for a T2–L2 fusion with a vascularized fibular graft. On postoperative Day 1, she underwent a bronchoscopy and had her left lower lobe airways occluded with multiple novel one-way endobronchial valves. She is now 5 years out from her tumor resection and 3 years out from her definitive fusion. She has no evidence of residual tumor, infection, or pseudarthrosis and continues to remain asymptomatic.

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Dana A. Weiss, Albert S. Lee, Tracy M. Flanders, Christopher J. Long, Jason P. van Batavia, Stephen A. Zderic and Gregory G. Heuer

Myelomeningocele (MMC) affects approximately 2000 infants in the US yearly and causes long-term damage to the genitourinary system. There is a wide spectrum of effects on the bladder and urethral sphincter that do not correlate with the level of the spinal cord defect. The bladder changes can provide a safe storage pressure, often at the expense of continence, or can create a hostile environment leading to increased pressure on the kidneys and eventually to chronic kidney disease. The ultimate goals in management are to maintain a safe system that prevents renal deterioration, while providing social continence and independence in the long term. This review intends to provide an overview of the effects of MMC on the genitourinary system, regardless of intervention, in order to understand the pathophysiology of the disease.

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H. Isaac Chen, Gregory G. Heuer, Kareem Zaghloul, Scott L. Simon, John B. Weigele and M. Sean Grady

✓Vertebral hemangiomas are common entities that rarely present with neurological deficits. The authors report the unusual case of a large L-3 vertebral hemangioma with epidural extension in a 27-year-old woman who presented with hip flexor and quadriceps weakness, foot drop, and leg pain. The characteristics of the mass on magnetic resonance imaging suggested an aggressive, hypervascular lesion. The patient underwent embolization of the lesion followed by direct intralesional injection of ethanol. Significant resolution of clinical symptoms was observed immediately after the procedure and at her follow-up visits. Follow-up imaging studies obtained 9 months after the procedure also documented a considerable reduction in the size of the hemangioma with minimal loss of vertebral height and a mild kyphosis at the affected level. On repeated imaging studies obtained 21 months postoperatively, the size of the hemangioma and the degree of vertebral body compression were stable. As demonstrated in this case, patients with vertebral hemangiomas can present with acute nerve root compression and signs and symptoms similar to those of disc herniation. Vertebral hemangiomas can be treated effectively with interventional techniques such as embolization and ethanol injection.