Category Archives: Pediatrics

XXXIII Reunión de la SENEP 2017

Se celebrará en Madrid los próximos días 9, 10 y 11 de Febrero de 2017.

Este año, el tema escogido para la Reunión es “Encuentro con los expertos” (“Meet the experts”).

8:15 h – 8:30 h Apertura de Secretaría y recogida de documentación.
SALA COLUMNAS
8:30 h – 9:10 h TALLER: Brainlab (Grupo 1)
9:15 h – 9:55 h TALLER: Osteoplac (Grupo 1)
10:00 h – 10:40 h TALLER: Brainlab (Grupo 2)
10:45 h – 11:25 h TALLER: Osteoplac (Grupo 2)
SALA A. PALACIOS
8:30 h – 9:10 h TALLER: BBraun (Grupo 2)
9:15 h – 9:55 h TALLER: Acuña y Fombona (Grupo 2)
10:00 h – 10:40 h TALLER: BBraun (Grupo 1)
10:45 h – 11:25 h TALLER: Acuña y Fombona (Grupo 1)
11:30 h – 11:50 h Pausa-Café.
11:50 h – 12:10 h Inauguración de la XXXIII Reunión de la SENEP.
12:15 h – 13:45 h “COLUMNA”
Modera: Dr. Antonio Huete Allut y Dr. Mario García Conde.
12:15 h – 12:45 h.
Malformaciones de charnela occipital. Dr. Dominique Thompson.
Paediatric Neurosurgeon, Great Ormond Street Hospital for Children NHS Trust,
Camden, Londres.
12:45 h – 13:15 h.
Syringomyelia in children. Dr. Michel Zerah. Hôpital Necker-Enfants Malades, París.
13:15 h – 13:45 h.
Embriology of the Neural tube defects with costo-vertebral abnormalities
and other congenital abnormalities. Dr Soner Duru. Profesor Doctor.
Universidad de Duzce, Turquía.
13:45 h – 15:30 h Almuerzo de trabajo.
8:30 h – 11:00 h “ONCOLOGÍA”
Modera: Dr. José Hinojosa Mena-Bernal y Dra. Sonia Tejada Solís.
8:30 h – 9:00 h.
Gliomas de la vía óptica: ¿Cuándo intervenir quirúrgicamente?
Dra. Martina Messing-Junger. Neurocirujana. St. Augustine. Asklepios, Bonn.
9:00 h – 9:30 h.
Intraoperative MRI in LGG. Dr. Connor Mallucci.
Alder Hey Children’s Hospital. Liverpool.
9:30 h – 10:00 h.
Tumores de los hemisferios cerebrales. Dr. Artur Da Cunha.
Presidente de la Sociedad Brasileña de Neurocirugía Pediátrica.
10:00 h – 10:30 h.
Antenatal tumors. Dr. Michel Zerah. Hôpital Necker-Enfants Malades, París.
 10:30 h – 11:00 h.
Abordajes a tumores del III Ventrículo. Dr. Fernando Carceller Benito.
Hospital Universitario La Paz, Madrid.
11:00 h – 11:15 h Pausa – Café.
11:15 h – 11:55 h Taller Baxter.
Taller Hemostasia y Sellado
12:00 h – 14:00 h “ONCOLOGÍA”
Modera: Dr. Javier Orduna Martínez y Dr. Enrique Ferrer Rodríguez.
12:00 h – 12:30 h.
El manejo inicial de los Craneofaringiomas a la edad pediátrica: controversias.
Prof. Maurice Choux. Consultor en el Departamento de Neurocirugía
Pediátrica – Hôpital des Enfants, La Timone.

12:30 h – 13:00 h.
Manejo de los tumores de la región pineal. Dr. Amets Sagarribay.
Centro Hospitalar de Lisboa Central.
13:00 h – 13:30 h.
Astrocitomas y ependimomas de fosa posterior.
Dr. Antonio Guillén Quesada.
Hospital San Juan de Dios, Barcelona.
13:30 h – 14:00 h.
Meduloblastoma: actualización diagnóstica y terapéutica. Dra. Belén Rivero Martín.
Hospital Universitario Infantil Niño Jesús, Madrid.
14:00 h – 15:30 h  Almuerzo de trabajo.
9:00 h – 9:40 h TALLER: Epilepsia. Livanova.
10:00 h – 11:40 h “EPILEPSIA”
Modera: Dr. Francisco Villarejo Ortega y Dr. Antonio López López-Guerrero.
10:00 h – 10:20 h.
Demanda estructural de la nueva cirugía de la Epilepsia.
Dr. Enrique Ferrer Rodríguez. Jefe de Servicio de Neurocirugía
Hospital San Juan de Dios, Barcelona.
10:25 h – 10:45 h.
Tratamiento quirúrgico de las displasias corticales en área elocuente:
indicación, técnica y resultados. Dr. Marcelo Budke. Hospital Universitario
Infantil Niño Jesús, Madrid.
10:50 h – 11:10 h.
Estimulación del nervio vago: técnica e indicaciones.
Dra. Cristina Torres Díaz. Hospital de la Princesa, Madrid.
11:20 h – 11:40 h.
Monitorización invasiva en epilepsia infantil.
Dra. Rebeca Conde Sardon. Hospital La Fe, Valencia.
11:45 h – 12:15 h Pausa – Café.
12:15 h – 13:55 h “HIDROCEFALIA”
Modera: Dra. Eva Cardona Gallego y Dra. Beatriz Pascual Martín.
12:15 h – 12:35 h.
Utilidad y controversias en el tratamiento endoscópico de la hidrocefalia
en lactantes. Dr. Mario García Conde. Hospital Universitario de Canarias, Tenerife.
12:40 h – 13:10 h.
Actualizaciones y controversias en la hidrocefalia pediátrica.
Dra. Mª Antonia Poca Pastor. Hospital Universitario Vall D’Hebron, Barcelona.
13:15 h – 13:35 h.
Hidrocefalia posthemorrágica: clasificación pronóstica.
Dr. Bienvenido Ros López. Hospital Regional Universitario, Málaga.
13:35 h – 13:55 h.
Complicaciones y sobredrenaje en las derivaciones de LCR.
Dr. Pablo Miranda Lloret. Hospital La Fe, Valencia.
14:00 h “CÓCTEL DE DESPEDIDA”
Círculo de Bellas Artes-Sala Valle Inclán

Book: Pediatric Vascular Neurosurgery: Disorders and their Management

Pediatric Vascular Neurosurgery: Disorders and their Management

Pediatric Vascular Neurosurgery: Disorders and their Management

List Price: $179.00

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This book answers frequently asked questions about common pediatric neurosurgical conditions related to vascular malformations of the brain and spinal cord, in an attempt to fill in the gap and answer numerous questions that arises after a diagnosis is made.

Pediatric patients with neurosurgical conditions are almost always referred from either primary care physicians, neurologists internists or a specialist in family medicine. Recently, neurosurgeons treating adult population also refer a pediatric patient to their colleague specialized in this field.
There are over 1500 academic and private hospitals in the US who have dedicated tertiary Neurosurgery services and cater thousands of small children every year, in addition to numerous centers that have level 1 and 2 trauma care. However, there are few tertiary level Pediatric centers which can provide quality care for neurosurgical conditions.
This book is specially written and illustrated for residents, fellows and consultants/attendings in all pediatric related specialties, including but not limited to Neurosurgery, Neurology, Pediatrics, Radiology, Anesthesia.

Product Details

  • Published on: 2017-01-03
  • Original language: English
  • Number of items: 1
  • Dimensions: 9.30″ h x .0″ w x 6.10″ l, .0 pounds
  • Binding: Hardcover
  • 327 pages

Editorial Reviews

From the Back Cover
This book focuses on core concepts of vascular neurosurgery in pediatric population,. It is designed to fill the knowledge gaps and to answer the frequently sought questions on various management strategies for commonly encountered pediatric neurosurgical conditions. The chapters, authored by experts in their respective field, provide a standard of care based on current diagnostic and management guidelines for pediatric neurosurgical diseases.

Pediatric Vascular Neurosurgery – Disorders and their Management is specially written and illustrated for residents, fellows and consultants in all pediatric related specialties, including but not limited to Neurosurgery, Neurology, Pediatrics, Neuroradiology and Neuroanesthesia.

About the Author
Dr Abhishek Agrawal, M.D.: House Staff, Department of Neurosurgery/ Radiology, Brigham and Women’s’ Hospital, Harvard Medical School, Boston.

Dr Gavin Britz, MBCCh, MPH, MBA, FAANS: Chairman, Department of Neurosurgery, Methodist Neurological Institute, Houston, Texas

Update: Caudal duplication syndrome

Spinal and spinal cord duplicity (diastematomyelia) malformations span a wide spectrum of anomalies, ranging from a simple fibrous band splitting the cord into halves to complete duplication of the spine and spinal cord. The more serious forms are rare and only a limited number of cases are on record.

They are usually associated with other systemic malformations, including duplication of vascular structures, of the distal gastrointestinal and urogenital tracts, and possibly limb malformations. The term caudal duplication syndrome has been applied to those instances.

This entity was first described by Dominguez et al.from the Department of Pediatric Radiology, University of Texas Southwestern Medical Center, Dallas. They reported 6 new cases as well as reviewed 8 already reported cases of multiple anomalies and duplication of the distal organs derived from the hindgut, neural tube and adjacent mesoderm 1).


Caudal duplication was considered a rare type of conjoined twins previously 2) in which structures derived from the embryonic cloaca and notochord are duplicated to various extent.

The term encompasses a spectrum and often is quoted as one type of incomplete separation of monovular twins. Bajpai et al., present more evidence giving credence to caudal twining as the mechanism behind the syndrome. 3).

Epidemiology

Caudal duplication syndrome is a rare condition with only about 40 cases reported in the literature 4).

Female patients predominate in a ratio of about 2:1, and no familial or racial predilection has been shown 5).

Etiology

Exact etiology of caudal duplication syndrome is unknown. Various theories have been suggested. Incomplete separation of monozygotic twin has been postulated as the etiologic factor 6) 7).

Pang et al. advanced a unified theory for the spinal cord duplication disorders, suggesting that all result from abnormal adherence between ectoderm and endoderm 8).

In the view of Dominguez et al. these anomalies originate from damage to the mass formed by caudal cells and posterior gut at approximately 25 days of pregnancy 9).

Pathogenesis

Pathogenesis is unclear. Polytopic primary developmental field defect or a disruption sequence or somatic or germ line mutations in certain developmental genes could be involved 10).

Partial or complete duplication of the organizing centre within a single embryonic disc may increase the risk of mesodermal insufficiency and thus account for the failure of complete development of the cloacal membrane and consequent exostrophy or other aberrations 11).

Clinical features

It is a range of disease comprising gastrointestinal, genitourinary, vertebral, spinal, and limb abnormalities. Common gastrointestinal anomalies include duplication of the colon and rectum, which may be associated with a variety of other anomalies such as imperforate anus, rectal fistula, ventral hernia, omphalocele, duplication of terminal ileum, double appendices, Meckel’s diverticulum, intestinal malrotation, and situs inversus.

Urogenital anomalies comprise duplication of the external and internal genitalia, ureters, and bladder and anomalies of the kidney.

Spinal anomalies include hemivertebra, sacral agenesis, myelomeningocele, diplomyelia, duplication of lumbar spine, butterfly vertebra, and spina bifida 12).

Most patients have associate moderate to severe neurodeficit; although some can be neurologically normal 13).

Treatment

Treatment consists of staged correction of duplication anomalies. Either stripping of mucosa or resection of duplicated colon and rectum is undertaken. Division of septum in the UB is done to make it a single chamber. Corrective surgery in the form of fusion is done for the external and internal genitalia. Any spinal anomaly is also corrected suitably. Many authors have reported near-normal cosmetic and functional result for these complicated anomalies.

Shah and Joshi 14) suggested removal of one of the hemi-phalluses for cosmetic reasons in males. No corrective surgery was, however, done in the adult female patient reported by them. Liu et al. reported the case of caudal duplication syndrome in which they did multiple stage correction. The bladder septum was removed, the two hemi-phalluses were fused to form one phallus, and the duplicated colon was excised. The patient also had hydronephrotic left kidney and left megaureter, which were removed. Ultimate outcome is good 15).


These patients are difficult to manage posing numerous surgical as well as medical management challenges. Child’s organ systems are usually working normally. Certain questions arise, should one intervene? When should one intervene? And what should be the best intervention, fusion or excision of accessory organ? Organ duplication syndromes are difficult scenarios to manage. Treatment should always be individualized according to the extent of duplication and functionality of the organ systems involved. The malformations that are potentially life-threatening should be addressed first 16).

Case series

In six children with multiple anomalies and duplications of distal organs derived from the hindgut, neural tube, and adjacent mesoderm, spinal anomalies (myelomeningocele in two patients, sacral duplication in three, diplomyelia in two, and hemivertebrae in one) were present in all the patients. Duplications or anomalies of the external genitalia and/or the lower urinary and reproductive structures were also seen in all our patients. Ventral herniation (in one patient), intestinal obstructions (in one patient), and bowel duplications (in two patients) were the most common gastrointestinal abnormalities.

Dominguez et al., believe that the above constellation of abnormalities resulted from an insult to the caudal cell mass and hindgut at approximately the 23rd through the 25th day of gestation. They propose the term caudal duplication syndrome to describe the association between gastrointestinal, genitourinary, and distal neural tube malformations17).

Case reports

 2016

A full-term male presented with combination of anomalies including anorectal malformation, duplication of the colon and lower urinary tract, split of the lower spine, and lipomyelomeningocele with Tethered cord.

Samuk et al., report this exceptional case of caudal duplication syndrome with special emphasis on surgical strategy and approach combining all disciplines involved. The purpose of this report is to present the pathology, assessment, and management strategy of this complex case 18).


A 28-year-old female, gravida 2 para 2, with congenital caudal malformation who has undergone partial reconstructive surgeries in infancy to connect her 2 colons. She presented with recurrent left lower abdominal pain associated with nausea, vomiting, and subsequent feculent anal discharge. Imaging reveals duplication of the urinary bladder, urethra, and colon with with cloacal malformations and fistulae from the left-sided cloaca, uterus didelphys with separate cervices and vaginal canals, right-sided aortic arch and descending thoracic aorta, and dysraphic midline sacrococcygeal defect. Hydronephrosis of the left kidney with left hydroureter and inflammation of one of the colons were suspected to be the cause of the patient’s acute complaints. She improved symptomatically over the course of her hospitalization stay with conservative treatments 19).

2014

A 3-month-old male infant had presented with the classical form of the disease i.e., duplication of the gastrointestinal, genitourinary system and vertebral column with anterior abdominal wall hernia and a large lipomeningocele 20).

2013

Sur et al., report the case of a baby presenting on the first day of life with complete duplication of caudal structures below the dorsolumbar level 21).


A 3-day-old male neonate presented with features of anorectal malformation and duplication of the external genitalia. He was subsequently diagnosed with complete duplication of the colon, rectum, bladder, and urethra associated with spinal lipoma 22).

2009

A 13-year-old boy was born with duplicated colon-rectum and anus, diphallus, hydronephrosis of left kidney with megaureter, double bladders and urethras, and vertebral abnormalities. Multiple-stage correction was performed to remove the duplicated colon and the mucosa of the duplicated rectum. A new colon was reconstructed. The left kidney and megaureter were excised. The septum in the bladders was removed to convert the double bladders into a single bladder. The double phalluses were fused into a single penis. After these staged procedures, the boy is now living a normal life 23).


A female infant, born by cesarean delivery (dilation dystocia), was referred at age of 24 hours with a history of “imperforate anus”. Physical examination revealed duplicity of the vulvar introit (urine output by the right orifice and feces by the left). She was submitted to the following imaging exams: (1) echodopplercardiogram – interatrial and interventricular communications; (2) ultrasonography – pelvic left kidney; (3) barium enema – one of the perineal orifices had a communication with the rectum; the other communicated with the vaginal dome and the bladder (urogenital sinus); (4) voiding cystourethrogram (VCU) – two urethral orifices communicating with the bladder, bladder diverticulum on the right side and vesico-ureteral reflux (grade II) on the left side.

Investigation of the spine was done with conventional radiographs (XR), computed tomography (CT) and magnetic resonance imaging (MRI), which disclosed complex malformations of the thoracic and lumbosacral spine, with “S” shaped dextroscoliolis. Aortic duplication was also noted. The vertebral bodies of T1, T2, T4 and T10 were widened and split by an anterior median incisure. A T7 hemivertebra was also present. From T11 level there was complete duplication of the vertebral bodies extending down to the S2 level. The remaining sacral and coccigeal vertebrae were absent. Duplicated vertebrae were joined posteriorly by deformed laminae and encompassed an extremely enlarged spinal canal. The spinal cord was duplicated from level T1 downwards. From L1 level, a large lipoma occupied the spinal canal and communicated with the subcutaneous tissues inferiorly 24).

2008

In a 2-year-old female child, a case of abnormal mass in the perineum with undeveloped feet, duplication of colon, external genitalia, and lumbosacral vertebra was reported by D’Costa et al. 25).

2006

Shah and Joshi reported the case of an asymptomatic adult female with duplication of colon, rectum, anus, urinary bladder (UB), urethra, uterus, cervix, vagina, and external genitalia 26).

2004

Bajpai et al., report successful surgical management of a full-term infant with a constellation of anomalies of caudal duplication syndrome 27).


Radiographic, CT, and MR images of a 15-year-old girl who had lower back pain showed asymmetric lumbar spine duplication with spinal cord tethering secondary to a filum lipoma in the sacrum. Despite gross spinal abnormalities, the patient was neurologically intact and has been followed up with conservative treatment 28).

2001

An autopsy case of a full-term infant with incomplete caudal duplication syndrome associated with multiple anomalies.

These anomalies included a duplicated penis; double urinary bladder with an attenuated tunica muscularis; duplication of lower bowel with two ilia, appendices and colons; colonic hypogangliosis and left imperforated anus associated with rectourethral fistula. Other anomalies consisted of sacral meningomyelocele, sacral duplication with hypoplastic left sacrum and pelvic bones, muscle atrophy and hypoplasia of the left lower extremity, abnormal lobation of liver with stomach entrapment, omphalocele, and right atrial isomerism syndrome. The complex pattern of anomalies suggests the possibility that partial caudal duplication might be part of the spectrum of conjoined twinning 29).


1) , 9) , 17) Dominguez R, Rott J, Castillo M, Pittaluga RR, Corriere JN Jr. Caudal duplication syndrome. Am J Dis Child. 1993 Oct;147(10):1048-52. Review. PubMed PMID: 8213674.
2) Kapoor R, Saha MM. Complete duplication of the bladder, urethra and external genitalia in a neonate: a case report. J Urol 1987; 137:1243-4.
3) , 27) Bajpai M, Das K, Gupta AK. Caudal duplication syndrome: more evidence for theory of caudal twinning. J Pediatr Surg. 2004 Feb;39(2):223-5. PubMed PMID: 14966746.
4) , 7) , 24) Taneja AK, Zaffani G, Amato-Filho AC, Queiroz Lde S, Zanardi Vde A, Menezes-Netto JR. Caudal duplication syndrome. Arq Neuropsiquiatr. 2009 Sep;67(3A):695-6. PubMed PMID: 19722052.
5) , 12) , 14) , 26) Shah KR, Joshi A. Complete genitourinary and colonic duplication: a rare presentation in an adult patient. J Ultrasound Med. 2006 Mar;25(3):407-11. PubMed PMID: 16495506.
6) , 25) D’Costa GF, Kirtane J, Najmi S, Shedge R. Caudal duplication. Bombay Hosp J. 2008;50(3):529–531.
8) Pang D, Dias MS, Ahab-Barmada M. Split cord malformation: Part I: A unified theory of embryogenesis for double spinal cord malformations. Neurosurgery. 1992 Sep;31(3):451-80. Review. PubMed PMID: 1407428.
10) Kroes HY. Two cases of caudal duplication anomaly including a discordant monozygotic twin. Am J Med Genet 2002; 112:390-3.
11) Seibert A. Association of cloacal anomalies, caudal duplication and twinning. Ped Dev Pathol 2005; 8:339-54.
13) , 28) Incesu L, Karaismailoglu TN, Selcuk MB. Neurologically normal complete asymmetric lumbar spine duplication. AJNR Am J Neuroradiol. 2004 May;25(5):895-6. PubMed PMID: 15140743.
15) , 23) Liu H, Che X, Wang S, Chen G. Multiple-stage correction of caudal duplication syndrome: a case report. J Pediatr Surg. 2009 Dec;44(12):2410-3. doi: 10.1016/j.jpedsurg.2009.09.018. PubMed PMID: 20006039.
16) , 20) Ramzan M, Ahmed S, Ali S. Caudal duplication syndrome. J Coll Physicians Surg Pak. 2014 Jan;24(1):64-6. doi: 01.2014/JCPSP.6466. PubMed PMID: 24411548.
18) Samuk I, Levitt M, Dlugy E, Kravarusic D, Ben-Meir D, Rajz G, Konen O, Freud E. Caudal Duplication Syndrome: the Vital Role of a Multidisciplinary Approach and Staged Correction. European J Pediatr Surg Rep. 2016 Dec;4(1):1-5. doi: 10.1055/s-0035-1570370. PubMed PMID: 28018799.
19) Hu T, Browning T, Bishop K. Caudal duplication syndrome: imaging evaluation of a rare entity in an adult patient. Radiol Case Rep. 2016 Jan 19;11(1):11-5. doi: 10.1016/j.radcr.2015.12.001. PubMed PMID: 26973727; PubMed Central PMCID: PMC4769617.
21) Sur A, Sardar SK, Paria A. Caudal duplication syndrome. J Clin Neonatol. 2013 Apr;2(2):101-2. doi: 10.4103/2249-4847.116412. PubMed PMID: 24049755; PubMed Central PMCID: PMC3775131.
22) Swaika S, Basu S, Bhadra RC, Sarkar R, Maitra SK. Caudal duplication syndrome-report of a case and review of literature. Indian J Surg. 2013 Jun;75(Suppl 1):484-7. doi: 10.1007/s12262-013-0838-z. PubMed PMID: 24426655; PubMed Central PMCID: PMC3693374.
29) Bannykh SI, Bannykh GI, Mannino FL, Jones KL, Hansen L, Benirschke K, Masliah E. Partial caudal duplication in a newborn associated with meningomyelocele and complex heart anomaly. Teratology. 2001 Feb;63(2):94-9. PubMed PMID: 11241432.

The Global Rise of Endoscopic Third Ventriculostomy with Choroid Plexus Cauterization in Pediatric Hydrocephalus

Endoscopic third ventriculostomy with choroid plexus cauterization (ETV/CPC) offers an alternative to shunt treatment for infantile hydrocephalus.

In the quest to identify the optimal means of cerebrospinal fluid diversion free of shunt dependency, endoscopic third ventriculostomy (ETV) with choroid plexus cauterization (CPC) has been proposed as a promising procedure in select children. Supplementing traditional ETV with obliteration of the choroid plexus has been shown to decrease the likelihood of ultimate shunt dependency by roughly 20%. Originally devised to treat hydrocephalus in infants in sub-Saharan Africa, ETV/CPC has gained eager attention and cautious support in the developed world 1).

Diagnosing treatment failure is dependent on infantile hydrocephalus metrics, including head circumference, fontanel quality, and ventricle size.

Systematic review

Systematic review was performed using four electronic databases and bibliographies of relevant articles, with no language or date restrictions. Cohort studies of participants undergoing ETV/CPC that reported outcome were included using MOOSE guidelines. The outcome was time to repeat CSF diversion or death. Forest plots were created for pooled mean and its 95 % CI of outcome and morbidity.

Of 78 citations, 11 retrospective reviews (with 524 total participants) were eligible. Efficacy was achieved in 63 % participants at follow-up periods between 6 months and 8 years. Adverse events and mortality was reported in 3.7 and 0.4 % of participants, respectively. Publication bias was detected with respect to efficacy and morbidity of the procedure. A large discrepancy in success was identified between ETV/CPC in six studies from sub-Saharan Africa (71 %), compared to three studies from North America (49 %).

The reported success of ETV/CPC for infantile hydrocephalus is higher in sub-Saharan Africa than developed nations. Large long-term prospective multi-center observational studies addressing patient-important outcomes are required to further evaluate the efficacy and safety of this re-emerging procedure 2).

Case series

2016

It is not clear to what degree these metrics should be expected to change after ETV/CPC. Using these clinical metrics, Dewan et al., present and analyze the decision making in cases of ETV/CPC failure.

Infantile hydrocephalus metrics, including bulging fontanel, head circumference z-score, and frontal and occipital horn ratio (FOHR), were compared between ETV/CPC failures and successes. Treatment outcome predictive values of metrics individually and in combination were calculated.

Forty-four patients (57% males, median age 1.2 months) underwent ETV/CPC for hydrocephalus; of these patients, 25 (57%) experienced failure at a median time of 51 days postoperatively. Patients experiencing failure were younger than those experiencing successful treatment (0.8 vs 3.9 months, p = 0.01). During outpatient follow-up, bulging anterior fontanel, progressive macrocephaly, and enlarging ventricles each demonstrated a positive predictive value (PPV) of no less than 71%, but a bulging anterior fontanel remained the most predictive indicator of ETV/CPC failure, with a PPV of 100%, negative predictive value of 73%, and sensitivity of 72%. The highest PPVs and specificities existed when the clinical metrics were present in combination, although sensitivities decreased expectedly. Only 48% of failures were diagnosed on the basis all 3 hydrocephalus metrics, while only 37% of successes were negative for all 3 metrics. In the remaining 57% of patients, a diagnosis of success or failure was made in the presence of discordant data.

Successful ETV/CPC for infantile hydrocephalus was evaluated in relation to fontanel status, head growth, and change in ventricular size. In most patients, a designation of failure or success was made in the setting of discordant data 3).

2014

A study retrospectively reviewed medical records of 27 premature infants with intraventricular hemorrhage (IVH) and hydrocephalus treated with ETV and CPC from 2008 to 2011. All patients were evaluated using MRI before the procedure to verify the anatomical feasibility of ETV/CPC. Endoscopic treatment included third ventriculostomy, septostomy, and bilateral CPC. After ETV/CPC, all patients underwent follow-up for a period of 6-40 months (mean 16.2 months). The procedure was considered a failure if the patient subsequently required a shunt. The following factors were analyzed to determine a relationship to patient outcomes: gestational age at birth, corrected age and weight at surgery, timing of surgery after birth, grade of IVH, the status of the prepontine cistern and cerebral aqueduct on MRI, need for a ventricular access device prior to the endoscopic procedure, and scarring of the prepontine cistern noted at surgery.

Seventeen (63%) of 27 patients required a shunt after ETV/CPC, and 10 patients did not require further CSF diversion. Several factors studied were associated with a higher rate of ETV/CPC failure: Grade IV hemorrhage, weight 3 kg or less and age younger than 3 months at the time of surgery, need for reservoir placement, and presence of a normal cerebral aqueduct. Two factors were found to be statistically significant: the patient’s corrected gestational age of less than 0 weeks at surgery and a narrow prepontine cistern on MRI. The majority (83%) of ETV/CPC failures occurred in the first 3 months after the procedure. None of the patients had a complication directly related to the procedure.

Endoscopic third ventriculostomy/CPC is a safe initial procedure for hydrocephalus in premature infants with IVH and hydrocephalus, obviating the need for a shunt in selected patients. Even though the success rate is low (37%), the lower rate of complications in comparison with shunt treatment may justify this procedure in the initial management of hydrocephalus. As several of the studied factors have shown influence on the outcome, patient selection based on these observations might increase the success rate 4).

2005

A total of 710 children underwent ventriculoscopy as candidates for ETV as the primary treatment for hydrocephalus. The ETV was accomplished in 550 children: 266 underwent a combined ETV-CPC procedure and 284 underwent ETV alone. The mean and median ages were 14 and 5 months, respectively, and 443 patients (81%) were younger than 1 year of age. The hydrocephalus was postinfectious (PIH) in 320 patients (58%), nonpostinfectious (NPIH) in 152 (28%), posthemorrhagic in five (1%), and associated with myelomeningocele in 73 (13%). The mean follow up was 19 months for ETV and 9.2 months for ETV-CPC. Overall, the success rate of ETV-CPC (66%) was superior to that of ETV alone (47%) among infants younger than 1 year of age (p < 0.0001). The ETV-CPC combined procedure was superior in patients with a myelomeningocele (76% compared with 35% success, p = 0.0045) and those with NPIH (70% compared with 38% success, p = 0.0025). Although the difference was not significant for PIH (62% compared with 52% success, p = 0.1607), a benefit was not ruled out (power = 0.3). For patients at least 1 year of age, there was no difference between the two procedures (80% success for each, p = 1.0000). The overall surgical mortality rate was 1.3%, and the infection rate was less than 1%.

The ETV-CPC was more successful than ETV alone in infants younger than 1 year of age. In developing countries in which a dependence on shunts is dangerous, ETV-CPC may be the best option for treating hydrocephalus in infants, particularly for those with NPIH and myelomeningocele 5).


1) Dewan MC, Naftel RP. The Global Rise of Endoscopic Third Ventriculostomy with Choroid Plexus Cauterization in Pediatric Hydrocephalus. Pediatr Neurosurg. 2016 Dec 22. doi: 10.1159/000452809. [Epub ahead of print] PubMed PMID: 28002814.
2) Weil AG, Westwick H, Wang S, Alotaibi NM, Elkaim L, Ibrahim GM, Wang AC, Ariani RT, Crevier L, Myers B, Fallah A. Efficacy and safety of endoscopic third ventriculostomy and choroid plexus cauterization for infantile hydrocephalus: a systematic review and meta-analysis. Childs Nerv Syst. 2016 Nov;32(11):2119-2131. PubMed PMID: 27613635.
3) Dewan MC, Lim J, Morgan CD, Gannon SR, Shannon CN, Wellons JC 3rd, Naftel RP. Endoscopic third ventriculostomy with choroid plexus cauterization outcome: distinguishing success from failure. J Neurosurg Pediatr. 2016 Dec;25(6):655-662. PubMed PMID: 27564786.
4) Chamiraju P, Bhatia S, Sandberg DI, Ragheb J. Endoscopic third ventriculostomy and choroid plexus cauterization in posthemorrhagic hydrocephalus of prematurity. J Neurosurg Pediatr. 2014 Apr;13(4):433-9. doi: 10.3171/2013.12.PEDS13219. PubMed PMID: 24527862.
5) Warf BC. Comparison of endoscopic third ventriculostomy alone and combined with choroid plexus cauterization in infants younger than 1 year of age: a prospective study in 550 African children. J Neurosurg. 2005 Dec;103(6 Suppl):475-81. PubMed PMID: 16383244.

Refractory epilepsy in children with brain tumors. The urgency of neurosurgery

Central nervous system tumors represent the most common solid tumors in children and are a leading cause of cancer-related fatalities in this age group.

Genomics of medulloblastoma, ependymoma, and diffuse intrinsic pontine glioma (diffuse midline glioma, with H3-K27M mutation), have refined, if not redefined, the diagnostic classification and therapeutic stratification of patients with these tumors. They detail the substantial genetic heterogeneity across each disease type and, importantly, link genotypic information to clinical course. The most aggressive, treatment-resistant (and also treatment-sensitive) forms within each disease entity are identified, and their potentially actionable targets.

Molecularly based classification of pediatric brain tumors provides a critical framework for the more precise stratification and treatment of children with brain tumors 1).


High-grade pediatric brain tumors display higher CBF in Arterial Spin Labeling than do low-grade tumors, and they may be accurately graded by using presented values. CBF is correlated with tumor microvascular density 2).

Treatment

Malignant disease of the CNS is the primary etiology for deaths resulting from cancer in the pediatric population. It has been well documented that outcomes of pediatric neurosurgery rely on the extent of tumor resection. Therefore, techniques that improve surgical results have significant clinical implications. Intraoperative ultrasound (IOUS) offers real-time surgical guidance and a more accurate means for detecting residual tumor that is inconspicuous to the naked eye.

Outcome

Thirty day mortality is increasingly a reference metric regarding surgical outcomes.

Data estimate a 30-day mortality rate of 1.4-2.7% after craniotomy for pediatric central nervous system tumor. No detailed analysis of short-term mortality following a diagnostic neurosurgical procedure (e.g., resection or tissue biopsy) for tumor in the US pediatric population has been conducted.

The Surveillance, Epidemiology and End Results (SEER) data sets identified patients ≤ 21 years who underwent a diagnostic neurosurgical procedure for primary intracranial tumor from 2004 to 2011. One- and two-month mortality was estimated. Standard statistical methods estimated associations between independent variables and mortality.

A total of 5533 patients met criteria for inclusion. Death occurred within the calendar month of surgery in 64 patients (1.16%) and by the conclusion of the calendar month following surgery in 95 patients (1.72%). Within the first calendar month, patients < 1 year of age (n = 318) had a risk of death of 5.66%, while those from 1 to 21 years (n = 5215) had a risk of 0.88% (p < 0.0001). By the end of the calendar month following surgery, patients < 1 year (n = 318) had a risk of death of 7.23%, while those from 1 to 21 years (n = 5215) had a risk of 1.38% (p < 0.0001). Children < 1 year at diagnosis were more likely to harbor a high-grade lesion than older children (OR 1.9, 95% CI 1.5-2.4).

In the SEER data sets, the risk of death within 30 days of a diagnostic neurosurgical procedure for a primary pediatric brain tumor is between 1.16% and 1.72%, consistent with contemporary data from European populations. The risk of mortality in infants is considerably higher, between 5.66% and 7.23%, and they harbor more aggressive lesions 3).

Case series

2016

27 patients with drug resistant epilepsy and brain tumor, aged up to 19 years at the time of surgery, were studied between 1996 and 2013 and followed up for at least one year. The mean interval between the onset of seizures and the diagnosis of the tumor was 3.6 years, and from diagnosis to the surgery, 18 months. The location of the tumor was in the temporal lobe in 16, with ganglioglioma and dysembryoplastic neuroepithelial tumors being the most frequent. Among the patients, 92.5% and 90.4% were seizure-free in the first and fifth year after surgery, respectively. Twelve of 16 children were successful in becoming drug-free, with complete withdrawal by 3.2 years. Surgery proved to be potentially curative and safe in these cases, suggesting that the tumor diagnosis and surgery cannot be postponed 4).


1) Vitanza NA, Cho YJ. Advances in the biology and treatment of pediatric central nervous system tumors. Curr Opin Pediatr. 2016 Feb;28(1):34-9. doi: 10.1097/MOP.0000000000000309. PubMed PMID: 26709691.
2) Dangouloff-Ros V, Deroulers C, Foissac F, Badoual M, Shotar E, Grévent D, Calmon R, Pagès M, Grill J, Dufour C, Blauwblomme T, Puget S, Zerah M, Sainte-Rose C, Brunelle F, Varlet P, Boddaert N. Arterial Spin Labeling to Predict Brain Tumor Grading in Children: Correlations between Histopathologic Vascular Density and Perfusion MR Imaging. Radiology. 2016 Nov;281(2):553-566. PubMed PMID: 27257950.
3) Hankinson TC, Dudley RW, Torok MR, Patibandla MR, Dorris K, Poonia S, Wilkinson CC, Bruny JL, Handler MH, Liu AK. Short-term mortality following surgical procedures for the diagnosis of pediatric brain tumors: outcome analysis in 5533 children from SEER, 2004-2011. J Neurosurg Pediatr. 2016 Mar;17(3):289-97. doi: 10.3171/2015.7.PEDS15224. Epub 2015 Nov 20. PubMed PMID: 26588456.
4) Bernardino MR, Funayama C, Hamad AP, Machado H, Sakamoto A, Thome U, Terra VC, Santos AC. Refractory epilepsy in children with brain tumors. The urgency of neurosurgery. Arq Neuropsiquiatr. 2016 Dec;74(12):1008-1013. doi: 10.1590/0004-282×20160157. PubMed PMID: 27992000.

Update: Sylvian fissure arachnoid cyst

A Sylvian fissure intracranial arachnoid cyst (SAC) is a well-recognized location for an intracranial arachnoid cyst in the pediatric population.

Arachnoid cysts situated in the middle cranial fossa constitute the largest group of this type of lesion.

Classification

The Galassi classification of middle cranial fossa arachnoid cysts is used to classify arachnoid cysts in the middle cranial fossa, which account for 50-60% of all arachnoid cysts.

Galassi et al published this classification in 1982, and at the time of writing (November 2016) it remains the most widely used system for these lesions.

It is a simple system, using the size and degree of displacement of adjacent brain to divide cysts three types. The size also correlates with the ease with which the cyst communicates with the subarachnoid space as discerned on CT cisternography or phase contrast MRI.

type I small, spindle-shaped limited to the anterior portion of the middle cranial fossa, below the spenoid ridge free communication of subarachnoid space

type II superior extent along sylvian fissure displacement of the temporal lobe slow communication with subarachnoid space

type III large, fills the whole middle cranial fossa displacement of not only the temporal lobe but also the frontal and parietal lobes often results in midline shift little communication with subarachnoid space

1).

Clinical features

Intracranial sylvian arachnoid cysts are often asymptomatic lesions.

(a) Axial CT scan showing a left sylvian fissure arachnoid cyst. (b) Complete resolution after excision and marsupialization.

Treatment

Sylvian arachnoid cysts pose considerable management dilemmas. Surgical options include cyst fenestration, either endoscopically or microsurgically, and cystoperitoneal shunt.

The option of the mere clinical observation was chosen by the majority of surgeons in case of asymptomatic clinical discovery. On the other hand, a constantly high percentage of participants suggested direct surgical treatment based on clinical manifestations or as a preventive measure justified by the risk of spontaneous or traumatic intracranial bleeding. The only diagnostic investigation result which significantly influenced the surgical indication was a localizing electroencephalography, if the child presented with seizures. The result is that in most cases the surgical indication was based on a specific clinical manifestations and laboratory data. Craniotomy and arachnoid cyst marsupialization represented the preferred surgical option (66.6%), 28.8% of the participants suggesting pure or assisted endoscopic cyst marsupialization as primary surgical procedure. Cyst shunting was suggested by only three centers 2).

For those cysts, which can rupture and be accompanied by a subdural hygroma or subdural hematoma, several treatment modalities have been reported.

A study demonstrated efficacy in a predominantly endoscopically treated patient cohort with Sylvian fissure arachnoid cysts, as indicated by improvement of clinical symptoms and diminished radiological SAC volume after treatment 3)

Complications

it sometimes leads to subdural or intracystic hemorrhage without major trauma. The reason of easy bleeding of the AC is not fully understood.

One of the rare complications after rapid decompression of the arachnoid cysts is haemorrhage in the surrounding brain as well as in remote areas.

Long-standing asymptomatic sylvian arachnoid cysts may suddenly produce severe unilateral visual deficits if the cyst erodes the lateral orbital wall. These deficits may rapidly revert to normal if surgical action is not delayed. If surveillance MRIs of sylvian arachnoid cysts show a narrowing of the conus diameter compared to the contralateral side, a yearly ophthalmological surveillance examination seems to be warranted in else wise asymptomatic patients 4).

Case series

A retrospective review of the database of operative procedures revealed 24 procedures (20 endoscopic and 4 microsurgical procedures) to fenestrate a Sylvian fissure arachnoid cyst (SAC) at university hospitals in Berlin, Germany and Tokyo, Japan.

With the applied technique, a reduction of SAC volume of more than 10% was achieved in 83.3% of all patients. The median volume of SACs (n = 24) was significantly reduced from 83.5 mL (range 21-509 mL) preoperatively to 45.5 mL (range 8.4-261 mL; P < 0.01) after 3.5 months and to 29.0 mL (range 0-266 mL; P < 0.01) after 15 months. In children (n = 8) with a ruptured SAC the combined extraaxial volume of a SAC and accompanying hygroma/hematoma was reduced from 166 mL (range 111-291 mL) before surgery to 127 mL (range 87-329 mL) after 2 months and to 77 mL (range 25-140 mL; P < 0.05) after 11 months. Acute clinical symptoms were generally resolved postoperatively; headaches were resolved or improved in 75%. A significant association of resolution or improvement of headaches and volume reduction was demonstrated.

The study demonstrated efficacy in a predominantly endoscopically treated patient cohort with Sylvian fissure arachnoid cysts, as indicated by improvement of clinical symptoms and diminished radiological SAC volume after treatment 5).

Case reports

2016

A case of multiple remote-site intra-parenchymal haemorrhage as a rare complication after surgical decompression of a sylvian fissure arachnoid cyst 6).

2013

Lohani et al., present the case of an 11-year-old boy who presented after a week of progressive and severe back pain radiating to the back of his thighs. Imaging revealed a spinal subdural blood collection at the L4-S1 level. This finding prompted further cephalad imaging of the spine and the brain, which revealed a sylvian fissure arachnoid cyst with intracystic hemorrhage and frontoparietal subdural hematoma. The child did not have headache at this time, although he had experienced chronic headaches since the age of 4 years. He was treated with a course of oral steroids, which immediately relieved his back and leg pain. Subsequent imaging showed resolution of the cranial and spinal subdural blood collections and diminished size of the arachnoid cyst. No surgical treatment was necessary 7).


Upadhyaya et al. report a case of a sylvian cistern arachnoid cyst presenting with precocious puberty in a 3-year-old girl. The child recovered following a cystoperitoneal shunt. The mass effect of the arachnoid cyst upon the hypothalamus was, at least in part, responsible for the development of precocious puberty. To the best of the knowledge, this is the 1st case of a sylvian cistern arachnoid cyst presenting with precocious puberty. The role of surgical decompression of the cyst is also discussed 8).


Prokopienko et al., report the case of a 36-year-old woman with a Sylvian fissure arachnoid cyst, which diminished after head trauma and minor hemorrhage into the cyst. They discuss the relationship between the cyst volume reduction and the head trauma to determine the main mechanism of this self-healing process 9).

2006

Intraparenchymal hemorrhage after surgical decompression of a Sylvian fissure arachnoid cyst 10).

1995

A case of brain stem hemorrhage after decompression of a sylvian fissure arachnoid cyst has been reported 11).


1) Galassi E, Tognetti F, Gaist G, Fagioli L, Frank F, Frank G. CT scan and metrizamide CT cisternography in arachnoid cysts of the middle cranial fossa: classification and pathophysiological aspects. Surg Neurol. 1982 May;17(5):363-9. PubMed PMID: 7089853.
2) Tamburrini G, Dal Fabbro M, Di Rocco C. Sylvian fissure arachnoid cysts: a survey on their diagnostic workout and practical management. Childs Nerv Syst. 2008 May;24(5):593-604. doi: 10.1007/s00381-008-0585-9. Erratum in: Childs Nerv Syst. 2008 May;24(5):635. Del Fabbro, Mateus [corrected to Dal Fabbro, Mateus]. PubMed PMID: 18305944.
3) Schulz M, Kimura T, Akiyama O, Shimoji K, Spors B, Miyajima M, Thomale UW. Endoscopic and Microsurgical Treatment of Sylvian Fissure Arachnoid Cysts-Clinical and Radiological Outcome. World Neurosurg. 2015 Mar 25. pii: S1878-8750(15)00293-4. doi: 10.1016/j.wneu.2015.03.026. [Epub ahead of print] PubMed PMID: 25818148.
4) Kural C, Kullmann M, Weichselbaum A, Schuhmann MU. Congenital left temporal large arachnoid cyst causing intraorbital optic nerve damage in the second decade of life. Childs Nerv Syst. 2015 Aug 9. [Epub ahead of print] PubMed PMID: 26255149.
5) Schulz M, Kimura T, Akiyama O, Shimoji K, Spors B, Miyajima M, Thomale UW. Endoscopic and Microsurgical Treatment of Sylvian Fissure Arachnoid Cysts-Clinical and Radiological Outcome. World Neurosurg. 2015 Aug;84(2):327-36. doi: 10.1016/j.wneu.2015.03.026. PubMed PMID: 25818148.
6) Ramachandran GM, Nair RP, Kongwad LI, Shanthakumar G. Rapid Brain Shift with Remote-Site Haemorrhage after Arachnoid Cyst Excision: Treatment Dilemmas. Pediatr Neurosurg. 2016 Dec 3. [Epub ahead of print] PubMed PMID: 27915350.
7) Lohani S, Robertson RL, Proctor MR. Ruptured temporal lobe arachnoid cyst presenting with severe back pain. J Neurosurg Pediatr. 2013 Sep;12(3):281-3. doi: 10.3171/2013.6.PEDS13122. PubMed PMID: 23829378.
8) Upadhyaya S, Nair R, Kumar V, Nayal B, Shetty A. Sylvian cistern arachnoid cyst – a rare cause of precocious puberty. Pediatr Neurosurg. 2013;49(6):365-8. doi: 10.1159/000368323. Epub 2014 Nov 21. PubMed PMID: 25428575.
9) Prokopienko M, Kunert P, Marchel A. Unusual volume reduction of Galassi grade III arachnoid cyst following head trauma. J Neurol Surg A Cent Eur Neurosurg. 2013 Dec;74 Suppl 1:e198-202. doi: 10.1055/s-0033-1342931. PubMed PMID: 23696293.
10) Esmaeeli B, Eftekhar B. Intraparenchymal hemorrhage after surgical decompression of a Sylvian fissure arachnoid cyst. Neurol India. 2006 Sep;54(3):320-1. PubMed PMID: 16936408.
11) Borges G, Fernandes YB, Gallani NR. [Brainstem hemorrhage after surgical removal of arachnoid cyst of the Sylvian fissure: A case report]. Arq Neuropsiquiatr 1995;53:825-30.