Progressive vascular tumor in infant: A case report and literature review of PIK3CA vascular malformation.

PURPOSE: Vascular anomalies are classified as either vascular tumors or vascular malformations. Vascular malformations can be difficult to diagnose and treat in the pediatric population and can masquerade as malignant processes. Understanding the genetics behind vascular malformations can lead to identification of specific mutations which can be treated with targeted immunotherapy. METHODS: Our case presents a pediatric patient with progressively enlarging vascular malformation despite multiple surgical resections and systemic medical treatments who underwent genetic evaluation and was found to have PIK3CA mutation. RESULTS: After identification of PIK3CA mutation, our patient was successfully treated with the p110ɑ-specific inhibitor, alpelisib, with both shrinkage of malformation on follow-up imaging as well as gains in her developmental milestones. CONCLUSION: Progressive vascular malformations in the pediatric population can be hard to diagnose and treat and are thought to arise from somatic mutations. Our case highlights a patient with progressive malformation despite multiple surgical resections who was successfully treated with targeted immunotherapy after proper identification of genetic mutation.

Contrast Enhancement Patterns in Pediatric Glioblastomas.

BACKGROUND AND PURPOSE: Brain tumors are the most common cause of cancer-related deaths among the pediatric population. Among these, pediatric glioblastomas (GBMs) comprise 2.9% of all central nervous system tumors and have a poor prognosis. The purpose of this study is to determine whether the imaging findings can be a prognostic factor for survival in children with GBMs. MATERIALS AND METHODS: The imaging studies and clinical data from 64 pediatric patients with pathology-proven GBMs were evaluated. Contrast enhancement patterns were classified into focal, ring-like, and diffuse, based on preoperative postcontrast T1-weighted magnetic resonance images. We used the Kaplan-Meier method and Cox proportional hazard regression to evaluate the prognostic value of imaging findings. RESULTS: Patients with ring-enhanced GBMs who underwent gross total resection or subtotal resection were found to have a significantly shorter progression-free survival ( P = 0.03) comparing with other enhancing and nonenhancing glioblastomas. CONCLUSIONS: In this study, we analyzed survival factors in children with pediatric glioblastomas. In the group of patients who underwent gross total resection or subtotal resection, those patients with focal-enhanced GBMs had significantly longer progression-free survival ( P = 0.03) than did those with other types of enhancing GBMs (diffuse and ring-like).

Can Craniosynostosis be Diagnosed on Physical Examination? A Retrospective Review.

Craniosynostosis is a developmental craniofacial defect in which one or more sutures of the skull fuse together prematurely. Uncorrected craniosynostosis may have serious complications including elevated intracranial pressure, developmental delay, and blindness. Proper diagnosis of craniosynostosis requires a physical examination of the head with assessment for symmetry and palpation of sutures for prominence. Often, if craniosynostosis is suspected, computed tomography (CT) imaging will be obtained. Recent literature has posited that this is unnecessary. This study aims to address whether physical examination alone is sufficient for the diagnosis and treatment planning of single suture craniosynostosis. Between 2015 and 2022, the Divisions of Pediatric Neurosurgery and Pediatric Plastic Surgery at UTHealth Houston evaluated 140 children under 36 months of age with suspected craniosynostosis by physical examination and subsequently ordered CT imaging for preoperative planning. Twenty-three patients received a clinical diagnosis of multi-sutural or syndromic craniosynostosis that was confirmed by CT. One hundred seventeen patients were diagnosed with single suture craniosynostosis on clinical examination and follow-up CT confirmed suture fusion in 109 (93.2%) patients and identified intracranial anomalies in 7 (6.0%) patients. These patients underwent surgical correction. Eight (6.8%) patients showed no evidence of craniosynostosis on CT imaging. Treatment for patients without fused sutures included molding helmets and observation alone. This evidence suggests that physical examination alone may be inadequate to accurately diagnose single suture synostosis, and surgery without preoperative CT evaluation could lead to unindicated procedures.

Choroid plexus cauterization to treat inadequate abdominal cerebrospinal fluid absorption: case report.

INTRODUCTION: Inadequate absorption of cerebrospinal fluid (CSF) in the setting of high CSF production is a relatively rare cause of shunt malfunction. CASE REPORT: We present the unique case of a 3-year-old boy who developed sterile ascites and abdominal distension in a delayed fashion after shunt placement. The shunt was externalized, and the patient was noted to have high CSF output. Bilateral choroid plexus cauterization resulted in a significant decrease in CSF production and enabled the shunt to be re-inserted into the abdomen.

Back to the Beginning: An Algorithmic Approach to Neonatal Myelomeningocele Repair.

INTRODUCTION: Myelomeningoceles are formed by prenatal failure of neural tube closure and can cause hydrocephalus, motor abnormalities, and developmental delay. Although small defects are amenable to primary closure, larger defects often require complex reconstruction. Our goal was to identify factors associated with postoperative soft tissue complications and develop a systematic approach for myelomeningocele closure. METHODS: A retrospective review was performed at the Children's Memorial Hermann Hospital from January 2013 to January 2019. Patients were identified using International Classification of Diseases, Ninth Revision/Tenth Revision , codes for myelomeningocele. Cohorts were stratified by reconstruction type and defect location. Primary outcomes were incidence of complications including cerebrospinal fluid leak, superficial and deep infection, and wound dehiscence. In addition, we developed an algorithm to standardize closure approach for patients with myelomeningoceles. RESULTS: A total of 172 patients with myelomeningocele were identified with 73 patients undergoing postnatal repair. Overall, 72% of defects were >5 cm. Defects were in the lumbar (9%), sacral (8%), and junctional (83%) regions. Overall, 30.1% patients underwent lumbar myofascial repair with 39.7% requiring fasciocutaneous flaps. Larger defects (>5 cm) were more likely to be closed with complex fasciocutaneous flaps (82.8% vs 66.0%, P = 0.11). No significant differences were observed in complication rates. CONCLUSIONS: In this series, patients with larger myelomeningoceles appear to benefit from complex flap closure. We propose a 5-layer closure for patients with myelomeningocele including the routine use of a myofascial layer. Cutaneous closure technique should be tailored based on specific defect characteristics as outlined in our algorithm. This approach streamlines myelomeningocele repair while optimizing outcomes and decreasing downstream complications.

Spontaneous resolution of large subdural hematoma with midline shift: a case report.

Subdural hematomas with mass effect and midline shift are typically offered urgent surgical evacuation. The authors report a 6-month-old baby who was referred for macrocephaly and found to have a large subdural hematoma with midline shift. Given her lack of symptoms and normal neurological examination, the patient was observed without neurosurgical intervention. She remained asymptomatic, and serial brain imaging showed progressive decrease of the subdural hemorrhage and complete resolution of midline shift. This case is presented to alert neurosurgeons that observation of a large subdural hematoma may rarely be appropriate in babies with open fontanelles.

Outcomes and prognostic factors of pediatric patients with a Glasgow Coma Score of 3 after blunt head trauma.

PURPOSE: This study aims to assess outcomes of pediatric patients with blunt traumatic brain injury (TBI) with a presenting Glasgow Coma Score (GCS) of 3. METHODS: After local institutional review board approval, we identified patients ages 0 to15 years with blunt TBI and a reported GCS of 3 between 2007 and 2017 from a pediatric level 1 trauma center prospective registry. Exclusion criteria were cardiac death on arrival and penetrating injury. We recorded clinical variables from patients with a non-pharmacologic GCS of 3 and pupillary exam documented by a neurosurgical attending or resident. The original Glasgow Outcome Scale (GOS) was used to compare with other studies. Importance of variables to survival was calculated. RESULTS: A total of 88 patients (mean age 6.9 years) were included with a mortality rate of 68%. Twelve percent had a poor long-term outcome (GOS 2 or 3) while 20% had a good long-term outcome (GOS 4 or 5). Median follow-up was 1.8 years. Initial group comparison revealed patients in group 1 (survivors) had less hypotension on arrival (14% SBP < 90 mmHg vs. 66%, p < 0.0001), higher temperatures on arrival (36.3 °C vs 34.9 °C, p = 0.0002), lower ISS (29.7 vs 39.5, p = 0.003), less serious injury to other major organs (34% vs 61%, p = 0.02), more epidural hematomas (24% vs 7%, p = 0.04), and less evidence of brain ischemia on CT (7% vs 39%, p = 0.002) or brainstem infarct, hemorrhage, or herniation (0% vs 27%, p = 0.002). Differences between the 2 groups in age, sex, race, MOI, AIS score, presence of midline shift > 5 mm, or time from injury to hospital arrival or time to surgery were not statistically significant. Classification tree analysis showed that the most important variable for survival was pupillary exam; mortality was 92% in presence of bilateral, fixed dilated pupils. The relative importance of initial temperature, MOI, and hypotension to survivability was 0.79, 0.75, and 0.47, respectively. CONCLUSION: Twenty percent of our pediatric non-pharmacologic GCS 3 cohort had a good functional outcome. Lack of bilaterally fixed and dilated pupils was the most important factor for survival. Temperature, MOI, and hypotension also correlated with survival. The data support selective aggressive management for these patients.

Posttreatment Maturation of Medulloblastoma into Gangliocytoma: Report of 2 Cases.

INTRODUCTION: We report 2 cases of medulloblastoma maturing into gangliocytoma after receiving multimodal therapy. Here we present 2 cases of diagnosed medulloblastoma which on re-resection were noted to be gangliocytoma without heterogeneity, which is an extremely rare occurrence. CASE PRESENTATION: The first patient, an 11-year-old boy diagnosed with high-risk (non-WNT, non-SHH) medulloblastoma, was treated with near-total surgical resection followed by craniospinal radiation therapy with weekly vincristine. He then received maintenance chemotherapy with vincristine, cyclophosphamide, and cisplatin. On surveillance MR imaging studies residual tumor in the lateral aspect of the tumor bed was noted to be slowly growing, eliciting gross-total resection of the residual tumor. Histopathology showed benign gangliocytoma without residual medulloblastoma. The second patient, a 3-year-old girl, was diagnosed with medulloblastoma, desmoplastic nodular variant. She was initially treated with gross total resection and chemotherapy with etoposide, carboplatin, and high-dose methotrexate. At 4 months off therapy, she was noted to have local recurrence along the resection cavity. Second-line therapy was started with irinotecan and temozolomide, but MRI assessment during treatment showed further disease progression. She then received craniospinal radiation. Eleven months off therapy, further radiographic progression was noted, and the patient underwent second-look surgery, with pathology showing gangliocytoma and treatment-related gliosis. DISCUSSION/CONCLUSION: The maturation of medulloblastoma into a ganglion cell-rich lesion is very rare, with few well-characterized previous reports. Given the rare nature of this entity, it would be of great value to understand the process of posttreatment maturation and the genetic and treatment factors which contribute to this phenomenon.

Is Less Actually More? An Evaluation of Surgical Outcomes Between Endoscopic Suturectomy and Open Cranial Vault Remodeling for Craniosynostosis.

BACKGROUND: Both open cranial vault remodeling (CVR) and endoscopic suturectomy are effective in treating the anatomical deformity of craniosynostosis. While parents are increasingly knowledgeable about these 2 treatment options, information regarding the perioperative outcomes remains qualitative. This makes preoperative counseling regarding surgical choices difficult. The purpose of this study was to evaluate the outcomes in patients with craniosynostosis who underwent traditional CVR versus endoscopic suturectomy. METHODS: Open and endoscopic craniosynostosis surgeries performed at our institution from January 2014 through December 2018 were retrospectively reviewed and perioperative data, including operative time, estimated blood loss, transfusion rate and length of stay, was analyzed. A student t test was used with significance determined at P < 0.05. RESULTS: CVR was performed for 51 children while 33 underwent endoscopic procedures. Endoscopic suturectomy was performed on younger patients (3.8 versus 14.0 months, P < 0.001), had shorter operative time (70 versus 232 minutes, P < 0.001), shorter total anesthesia time (175 versus 352 minutes, P < 0.001), lower estimated blood loss (10 versus 28 ml/kg, P < 0.001), lower percentage transfused (42% versus 98%, P < 0.001), lower transfusion volume (22 versus 48 ml/kg, P < 0.001), and shorter length of stay (1.8 versus 4.1 days, P < 0.001) when compared to open CVR. CONCLUSION: Both open CVR and endoscopic suturectomy are effective in treating deformities due to craniosynostosis. The endoscopic suturectomy had significantly shorter operative and anesthesia time as well as overall and PICU length of stay. CVR was associated with greater intraoperative blood loss and more frequently required higher rates of blood transfusions.

Infusion of 5-Azacytidine (5-AZA) into the fourth ventricle or resection cavity in children with recurrent posterior Fossa Ependymoma: a pilot clinical trial.

BACKGROUND: DNA methylation inhibitors are logical therapeutic candidates for ependymomas originating in the posterior fossa of the brain. Our objective was to test the safety of infusing 5-Azacytidine (5-AZA), a DNA methylation inhibitor, directly into cerebrospinal fluid (CSF) spaces of the fourth ventricle or tumor resection cavity in children with recurrent ependymoma originating in the posterior fossa. MATERIALS AND METHODS: In patients with recurrent ependymoma whose disease originated in the posterior fossa, a maximal safe subtotal tumor resection was performed. At the conclusion of the tumor resection, a catheter was surgically placed into the fourth ventricle or tumor resection cavity and attached to a ventricular access device. CSF flow from the posterior fossa to the sacrum was confirmed by CINE phase contrast magnetic resonance imaging (MRI) postoperatively. 12 consecutive weekly 10 milligram (mg) infusions of 5-Azacytidine (AZA) were planned. Disease response was monitored with MRI scans and CSF cytology. RESULTS: Six patients were enrolled. One patient was withdrawn prior to planned 5-AZA infusions due to surgical complications after tumor resection. The remaining five patients received 8, 12, 12, 12, and 12 infusions, respectively. There were no serious adverse events or new neurological deficits attributed to 5-AZA infusions. All five patients with ependymoma who received 5-AZA infusions had progressive disease. Two of the five patients, however, were noted to have decrease in the size of at least one intraventricular lesion. CONCLUSION: 5-AZA can be infused into the fourth ventricle or posterior fossa tumor resection cavity without causing neurological toxicity. Future studies with higher doses and/or increased dosing frequency are warranted.

Phase-contrast cerebrospinal fluid flow magnetic resonance imaging in qualitative evaluation of patency of CSF flow pathways prior to infusion of chemotherapeutic and other agents into the fourth ventricle.

PURPOSE: Nuclear medicine studies have previously been utilized to assess for blockage of cerebrospinal fluid (CSF) flow prior to intraventricular chemotherapy infusions. To assess CSF flow without nuclear medicine studies, we obtained cine phase-contrast MRI sequences that assess CSF flow from the fourth ventricle down to the sacrum. METHODS: In three clinical trials, 18 patients with recurrent malignant posterior fossa tumors underwent implantation of a ventricular access device (VAD) into the fourth ventricle, either with or without simultaneous tumor resection. Prior to infusing therapeutic agents into the VAD, cine MRI phase-contrast CSF flow sequences of the brain and total spine were performed. Velocity encoding (VENC) of 5 and 10 cm/s was used to confirm CSF flow from the fourth ventricular outlets to the cervical, thoracic, and lumbar spine. Qualitative CSF flow was characterized by neuroradiologists as present or absent. RESULTS: All 18 patients demonstrated CSF flow from the outlets of the fourth ventricle down to the sacrum with no evidence of obstruction. One of these patients, after disease progression, subsequently showed obstruction of CSF flow. No patient required a nuclear medicine study to assess CSF flow prior to initiation of infusions. Fourteen patients have received infusions to date, and none has had neurological toxicity. CONCLUSIONS: CSF flow including the fourth ventricle and the total spine can be assessed noninvasively with phase-contrast MRI sequences. Advantages over nuclear medicine studies include avoiding both an invasive procedure and radiation exposure.

Survey of Techniques Utilized to Access Ventricular Shunts and Reservoirs.

AIMS: This study assessed variations in pediatric neurosurgical technique when accessing shunts and ventricular access devices (VADs). METHODS: A 12-question survey was developed and sent to members of the American Association of Neurological Surgeons (AANS) whose self-identified subspecialty was pediatric neurosurgery. RESULTS: Four hundred and twenty surveys were sent out, and 149 responses were received (35.5% response rate); 95.3% of respondents always use sterile gloves, 55.0% never use a sterile gown, and 69.8% always have a member of the neurosurgery team perform the procedure. The majority of respondents answered "sometimes" for use of a facemask (38.3%), sterile drapes (39.6%), site shaving (45.6%), having an attending present (68.5%), and having an assistant hold the patient's head (78.5%). The majority reported using a 23- or 25-gauge butterfly needle for site entry (96.6%), and betadine or ChloraPrep™ as the preferred antiseptic solution (64.4%). The frequency in which CSF is sent for analysis is not standardized in 31.5% of respondents, and wait time for the antiseptic solution to dry is not standardized in 62.4%. CONCLUSIONS: There is great variation in the technique for accessing shunts and VADs. Future studies are needed to assess whether these discrepancies affect infection rates.

Correlating Prenatal Imaging Findings of Fetal Ventriculomegaly with the Need for Surgical Intervention in the First 3 Months after Birth.

BACKGROUND/AIMS: This study evaluates the predictive value of prenatal imaging measurements regarding the need for cerebrospinal fluid (CSF) diversion for fetal hydrocephalus in the first 3 months after birth. METHODS: We retrospectively reviewed a consecutive case series of patients with fetal hydrocephalus from January 2011 to December 2014 (n = 45). Prenatal measurements included head circumference (HC), biparietal diameter (BPD), and lateral ventricle (LV) width. Patients requiring CSF diversion within 12 weeks of birth were compared to those who did not require CSF diversion using the Wilcoxon rank sum test, and receiver-operating characteristic analysis was used to evaluate threshold values. RESULTS: CSF diversion was required within 12 weeks of birth in 30 of 45 patients. Mean LV width (mm) during the entire pregnancy was greater for the surgery group than the nonsurgery group. Neither BPD nor HC showed differences between the groups. A mean LV size ≥15 mm predicted the need for CSF diversion with a sensitivity of 67% and specificity of 73%. CONCLUSION: LV width is the prenatal imaging measurement that best predicts the need for postnatal CSF diversion.

Ventricular access device placement in the fourth ventricle to treat malignant fourth ventricle brain tumors: technical note.

PURPOSE: Ventricular access devices (VADs) are commonly placed in the lateral ventricle but rarely placed in other ventricular compartments. This manuscript describes technical aspects of VAD placement into the fourth ventricle for the purpose of treating malignant posterior fossa brain tumors. METHODS: As part of a pilot clinical trial to treat recurrent malignant brain tumors in children, seven patients underwent posterior fossa craniotomy and placement of a ventricular catheter under direct vision into the fourth ventricle. The catheter was placed without passing through any brain parenchyma. It was then connected to a VAD placed subcutaneously at the inferior aspect of the incision. Three of the seven patients underwent simultaneous subtotal resection of recurrent tumor located in the fourth ventricle or cerebellum, and one patient underwent simultaneous tumor biopsy. The VAD was used to administer chemotherapy (methotrexate) in five of the seven patients. RESULTS: Six patients had no new neurological deficits after surgery, and one patient had partial left-sided facial weakness that was attributed to resection of tumor close to the floor of the fourth ventricle. No new neurological deficits were caused by VAD placement or by methotrexate infusions into the fourth ventricle. CONCLUSIONS: A VAD for chemotherapy infusion can be placed safely into the fourth ventricle without damaging the brainstem or cerebellum. Attention to anatomical details specific to the fourth ventricle are important when placing a fourth ventricle VAD and when using it to administer chemotherapy.

An Outcomes Comparison Between Autologous and Alloplastic Cranioplasty in the Pediatric Population.

BACKGROUND: The use of alloplastic material in cranial reconstruction has been well described in the adult population, especially when a paucity of autologous tissue exists. In children it is unknown how long-term growth, however, may be affected by the implantation of nonexpansible alloplastic material. Therefore, the authors sought to compare the outcomes of pediatric patients undergoing alloplastic versus autologous cranial reconstruction. METHODS: To assess the safety and long-term outcomes of alloplastic cranioplasty in children, an institutional review board-approved, retrospective, single institution review of pediatric patients undergoing cranioplasty was performed from 2000 to 2014. The age at surgery, cause of the cranial defect, defect size, time since initial surgery to reconstruction, implant type, and complications were assessed. Postreconstruction imaging was reviewed if available. RESULTS: A reconstructive cranioplasty was performed in 41 pediatric patients (ages 1-19 years, average 7.35 years). Thirty patients underwent alloplastic reconstruction (age 4.37 ±â€Š5.57 years), and 11 underwent autologous reconstruction (age 2.00 ±â€Š3.74 years). The size of the cranial defects was 144.01 ±â€Š393.04 cm for autologous and 405.31 ±â€Š572.96 cm for alloplastic reconstructions. Follow-up for all patients was an average of 2.33 ±â€Š2.76 years (0.1-9 years). No patients in either group showed evidence of elevated intracranial pressure after cranioplasty. In long-term follow-up, none of the implants were exposed or lost because of infection. Computed tomography and physical examination demonstrated that there was no skull growth restriction in either group. CONCLUSIONS: Our data show that alloplastic cranioplasty in the pediatric population is a safe alternative, when autologous cranial bone is not available.

Methotrexate administration directly into the fourth ventricle in children with malignant fourth ventricular brain tumors: a pilot clinical trial.

We hypothesize that chemotherapy can be safely administered directly into the fourth ventricle to treat recurrent malignant brain tumors in children. For the first time in humans, methotrexate was infused into the fourth ventricle in children with recurrent, malignant brain tumors. A catheter was surgically placed into the fourth ventricle and attached to a ventricular access device. Cerebrospinal fluid (CSF) flow was confirmed by CINE MRI postoperatively. Each cycle consisted of 4 consecutive daily methotrexate infusions (2 milligrams). Disease response was monitored with serial MRI scans and CSF cytologic analysis. Trough CSF methotrexate levels were sampled. Five patients (3 with medulloblastoma and 2 with ependymoma) received 18, 18, 12, 9, and 3 cycles, respectively. There were no serious adverse events or new neurological deficits attributed to methotrexate. Two additional enrolled patients were withdrawn prior to planned infusions due to rapid disease progression. Median serum methotrexate level 4 h after infusion was 0.04 µmol/L. Range was 0.02-0.13 µmol/L. Median trough CSF methotrexate level 24 h after infusion was 3.18 µmol/L (range 0.53-212.36 µmol/L). All three patients with medulloblastoma had partial response or stable disease until one patient had progressive disease after cycle 18. Both patients with ependymoma had progressive disease after 9 and 3 cycles, respectively. Low-dose methotrexate can be infused into the fourth ventricle without causing neurological toxicity. Some patients with recurrent medulloblastoma experience a beneficial anti-tumor effect both within the fourth ventricle and at distant sites.

Hydrocephalus in patients with closed neural tube defects.

BACKGROUND: Hydrocephalus is common in patients with open neural tube defects (NTDs), but association with closed NTDs has rarely been reported. We describe three patients with untreated closed NTDs who required surgery for hydrocephalus. CASE REPORTS: Case 1: A full-term newborn presented with a skin-covered midline lumbar spine lipoma. Magnetic resonance imaging (MRI) scan demonstrated a lipomyelomeningocele. An ultrasound at birth showed mild ventriculomegaly but cerebrospinal fluid diversion was not offered because his fontanelle was soft. He was lost to follow-up and then presented at age 6 months with signs and symptoms of hydrocephalus. An endoscopic third ventriculostomy and choroid plexus coagulation were performed, and the lipomyelomeningocele was subsequently repaired electively. Case 2: A full-term neonate presented with a skin-covered midline lumbar spine lesion consistent with a meningocele on MRI scan. He was additionally noted to have macrocephaly, a full fontanelle, and ventriculomegaly on MRI scan. At age 2 days, an endoscopic third ventriculostomy and choroid plexus coagulation were performed. A ventriculoperitoneal shunt was required 1 month later. Three months after birth, an elective spinal cord untethering procedure was performed. Case 3: A neonate presented with a midline lumbar skin tag. An MRI scan demonstrated a lipomyelomeningocele, and surgery was planned electively. On preoperative evaluation at age 3 months, the patient had a tense fontanelle. The surgery was postponed, and a ventriculoperitoneal shunt was placed after imaging studies revealed hydrocephalus. CONCLUSION: Patients with closed NTDs should be assessed for clinical signs and symptoms of hydrocephalus because of this rare but possible association.

Brain metastasis of crystal-deficient, CD68-positive alveolar soft part sarcoma: ultrastructural features and differential diagnosis.

We report a case of alveolar soft part sarcoma (ASPS) presenting as an isolated frontal lobe metastasis. The tumor demonstrated little or no immunoreactivity for a broad panel of antibodies yet strong, diffuse immunoreactivity with CD68. On electron microscopy, the characteristic rectangular to rhomboid crystalline inclusions of ASPS were not present. Electron-dense granules resembling peroxisomes were present, sometimes in association with elongated granular structures having a periodic, lattice-like arrangement. Metastatic ASPS was confirmed by demonstration of an ASPSCR1-TFE3 fusion and imaging studies that excluded metastatic Xp11.2 translocation renal cell carcinoma. The primary site was subsequently identified in the lower extremity.

Elevation of Depressed Skull Fracture in Neonates Using a Breast Pump and a Custom-Molded Flange.

BACKGROUND AND IMPORTANCE: "Ping-pong fractures" (PPF) can occur in neonates and result in cosmetic deformity and local mass effect. Standard treatment involves surgical elevation of the depressed bone when the indentation is considerable or cosmetically apparent. Surgical correction of PPF subjects patients to the risks of surgery and general anesthesia. This article and corresponding video demonstrate a novel means of correcting PPF at bedside without surgery or anesthesia. We used a hospital-grade breast pump connected to a custom-fabricated flange to successfully elevate PPF in two neonates. CLINICAL PRESENTATION: Two moderately preterm infants were noted at birth to have large parietal PPF. To avoid surgical intervention, elevation using a suction device was attempted. A hospital-grade breast pump was used to provide suction. A custom device was fabricated out of a breast pump flange and molded Coloplast Brava® protective seal rings. This device was carefully applied to the skull to exactly match the diameter of the PPF and contour of the bone. Brief (15-30 seconds) periods of suction were applied several times until the PPF was successfully elevated as documented on subsequent computed tomography scans. Both infants achieved excellent cosmetic results with no adverse effects over 24- and 9-month follow-ups, respectively. DISCUSSION: This technique eliminates the risks of open surgical correction and corrected the PPF without general anesthesia or adverse effects to the infant. While there may be limitations due to patient age and/or location of the PPF, the use of widely available and inexpensive custom-fitted materials with a hospital grade breast pump achieves maximal efficacy without requiring higher negative pressure suction application. CONCLUSION: Elevation of PPF can be safely achieved in some neonates using readily available equipment: a hospital-grade breast pump, flange, and moldable adherent material. This technique is reasonable to attempt in lieu of surgical elevation.