Juvenile nasopharyngeal angiofibromas are vascular tumors that may make resection difficult and potentially dangerous. Preoperative embolization is frequently used to decrease surgical morbidity and blood loss. Embolization has typically been performed via a transarterial route using a variety of embolic materials. The authors present a case in which endoscopic assistance was used for direct transnasal tumor puncture and intratumoral embolization using the liquid embolic agent Onyx. In this case there was excellent infiltration of the parenchymal vasculature with complete angiographic obliteration. There were no complications related to the embolization. The tumor was resected with minimal blood loss. To the authors' knowledge, there have been no previous reports of this novel direct intratumoral embolization technique using endoscopic guidance.
Mohammad Ali Aziz-Sultan, Roham Moftakhar, Stacey Quintero Wolfe, Mohamed Samy Elhammady, Björn Herman and Hamad Farhat
Stacey Quintero Wolfe, Hamad Farhat, Mohamed Samy Elhammady, Roham Moftakhar and Mohammad Ali Aziz-Sultan
A 2-month-old infant presented with an enlarging scalp hemangioma and consumptive coagulopathy. The patient became severely thrombocytopenic despite medical treatment. Transarterial embolization with Onyx was performed with significant reduction in the size of the tumor and complete resolution of the thrombocytopenia within 12 hours. Onyx embolization appears to be an excellent treatment option for hemangiomas presenting with Kasabach-Merritt syndrome that are unresponsive to standard medical therapy.
Lee A. Tan, Manish K. Kasliwal, Roham Moftakhar and Lorenzo F. Munoz
Small-bowel ischemia and necrosis due to knotting of the peritoneal catheter is an extremely rare complication related to a ventriculoperitoneal shunt (VPS). A 3-month-old girl, with a history of Chiari II malformation and myelomeningocele (MM) after undergoing right occipital VPS insertion and MM repair at birth, presented to the emergency department with a high-grade fever. Examination of a CSF sample obtained via shunt tap raised suspicion for the presence of infection. Antibiotic therapy was initiated, and subsequently the VPS was removed and an external ventricular drain was placed. Intraoperatively, as attempts at pulling the distal catheter from the scalp incision were met with resistance, the distal catheter was cut and left in the abdomen while the remainder of the shunt system was successfully removed. While the patient was awaiting definitive shunt revision surgery to replace the VPS, she developed abdominal distension due to small-bowel obstruction. An emergency exploratory laparotomy revealed a knot in the distal catheter looping around and strangulating the distal ileum, causing small-bowel ischemia and necrosis in addition to the obstruction. A small-bowel resection with ileostomy was performed, with subsequent placement of ventriculoatrial shunt for treatment of hydrocephalus. The authors report this exceedingly rare clinical scenario to highlight the fact that any retained distal catheter must be carefully managed with immediate abdominal exploration to remove the distal catheter to avoid bowel necrosis as pulling of a knotted peritoneal catheter may strangulate the bowel and cause ischemia, with significant clinical morbidity and possible mortality.
Christina A. Markunas, R. Shane Tubbs, Roham Moftakhar, Allison E. Ashley-Koch, Simon G. Gregory, W. Jerry Oakes, Marcy C. Speer and Bermans J. Iskandar
Although Chiari Type I (CM-I) and Type 0 (CM-0) malformations have been previously characterized clinically and radiologically, there have been no studies focusing on the possible genetic link between these disorders. The goal of this study was to identify families in whom CM-0 and CM-I co-occurred and to further assess the similarities between these disorders.
Families were ascertained through a proband with CM-I. Detailed family histories were obtained to identify first-degree relatives diagnosed with CM-0. Several criteria were used to exclude individuals with acquired forms of CM-I and/or syringomyelia. Individuals were excluded with syndromic, traumatic, infectious, or tumor-related syringomyelia, as well as CM-I due to a supratentorial mass, hydrocephalus, history of cervical or cranial surgery unrelated to CM-I, or development of symptoms following placement of a lumbar shunt. Medical records and MR images were used to characterize CM-I and CM-0 individuals clinically and radiologically.
Five families were identified in which the CM-I proband had a first-degree relative with CM-0. Further assessment of affected individuals showed similar clinical and radiological features between CM-0 and CM-I individuals, although CM-I patients in general had more severe symptoms and skull base abnormalities than their CM-0 relatives. Overall, both groups showed improvement in symptoms and/or syrinx size following craniocervical decompression surgery.
There is accumulating evidence suggesting that CM-0 and CM-I may be caused by a common underlying developmental mechanism. The data in this study are consistent with this hypothesis, showing similar clinical and radiological features between CM-0 and CM-I individuals, as well as the occurrence of both disorders within families. Familial clustering of CM-0 and CM-I suggests that these disorders may share an underlying genetic basis, although additional epigenetic and/or environmental factors are likely to play an important role in the development of CM-0 versus CM-I.