OBJECTIVEMany repair techniques have been proposed to treat large myelomeningocele (MMC), and although effective in many cases, some of these techniques can be complex and time consuming, with complications such as cerebrospinal fluid (CSF) leakage, flap loss, tip necrosis, and wound dehiscence. The purpose of this study was to analyze cases of large skin defects and the methods applied and to report the outcomes of the keystone design perforator island flap (KDPIF) technique for large MMC closure.METHODSThe authors performed a retrospective review of all neonatal patients who had undergone KDPIF for MMC closure in the period from 2013 to 2018. All patients had a diagnosis of lumbosacral MMC based on obstetric ultrasound. The neurosurgeons and plastic surgeons had selected the cases after concluding that primary closure would be unlikely. The design of the flap is based on the randomly located vascular perforators, creating two identical opposing flaps to fashion a double keystone flap. During wound closure, V-Y advancement of each end of the double flap in the longitudinal axis creates redundancy in the central portion of the flap and reduces the horizontal tension. After discharge, both the neurosurgery and plastic surgery teams followed up all patients, tracking the results with photography.RESULTSNo skin flap dehiscence or necrosis, infection, or CSF leakage was detected, proving the reliability of the flap. One of the patients required further surgery for the large skin defects after insufficient intrauterine closure of the MMC and successfully underwent KDPIF treatment. Another patient (14.3%) had severe neonatal sepsis, which ultimately led to death. A ventriculoperitoneal shunt was required after the skin defect repair in 5 (83.3%) of the 6 surviving patients. Exceptional aesthetic results were achieved for all patients during the follow-up.CONCLUSIONSThe KDPIF technique is based on well-known vascular perforators of the intercostal, lumbar, and gluteal regions. Wound tension is widely distributed by the flap and, as a consequence, relevant tissue bulk, reliable vascularity, and important geometrical versatility are provided. In addition, most of the muscles and fascia are preserved, which is another advantage in terms of minimizing secondary morbidity to local tissue rearrangement. The use of KDPIF closure was successfully shown to be a viable alternative for more complex MMCs that present with large skin defects.

A female newborn patient presents with meningitis and hydrocephalus after lumbosacral myelomeningocele repair and skin closure. An external ventricular drain was used during the meningitis treatment. Patient had a late onset multiloculated hydrocephalus and isolated fourth ventricle on CT scan follow-up. We performed combined approach with a craniotomy, dissection of intraparietal sulcus entering in the atrium of left lateral ventricle and used endoscopic assistance to fenestrate intraventricular septations and to put a ventricular catheter through tentorium to drain both supra and infratentorial ventricles. The patient improved clinically and radiologically.

INTRODUCTION: Infratentorial tumors represent 45 to 60% of all brain tumors in children and are associated with hydrocephalus in 70 to 90% of cases. The aim of this study is to describe and analyze the surgical results and correlate them with the incidence of hydrocephalus, as well as identify predictive factors of hydrocephalus in patients underwent to a surgical treatment of posterior fossa tumors. METHODS: This is a retrospective observational study, based on demographic, clinical and radiological information of pediatric patients underwent to surgical resection of posterior fossa tumors by a single group between 2011 and 2019. RESULTS: We included 135 patients with a mean age of 7.56 years, mean follow-up of 35.7 months and overall survival of 18.8 months. Considering the histology, most tumors were pilocytic astrocytomas (34.1%), followed by medulloblastomas (27.4%) Total resection was achieved in 77.8% and tumor recurrence was observed in 16.2%. The rate of hydrocephalus was 36.3%, with most shunt procedures performed preoperatively. There was a high rate of complications associated with shunting (42.9%), mostly represented by infection (71.5%). Patients with hydrocephalus were younger (p = 0.001), showing a faster evolution between the onset of symptoms and diagnosis (p = 0.008). Tumor volume (p = 0.1) and location (p = 0.32) were not statistically significant for hydrocephalus, however, the presence of leptomeningeal disease (p = 0.001) was. The resection rate was also significant, with lesions totally resected showing the best results (p = 0.009). Regarding tumor histology, 33.3% of patients with medulloblastoma and 62.5% of patients with ependymoma were shunted. CONCLUSION: One third of children with posterior fossa tumors will require a shunt procedure. Some factors should guide the neurosurgeon during patient counseling and surgical planning to minimize the use of permanent shunts and avoid related complications.

An 8-month-old boy was referred to our department with a protruding soft mass in the lumbosacral region since birth, with intact overlying skin. The patient had clubfoot, and congenital scoliosis. On neurological examination, he had preserved muscle strength, but evolved with distal weakness and increasing in the lumbar mass volume. MRI confirmed the diagnosis of terminal myelocystocele by the typical finding of a trumpet-like flaring meningocele. Terminal myelocystocele classically presents as a large lumbosacral mass with skin cover, containing fat, cerebrospinal fluid, and neural tissue1,2. The spinal cord herniates through the dysraphic spine and terminates at a neural placode, whereas the central canal opens into a CSF-filled, ependyma-lined cavity3. We present a 2D operative video comprising microsurgical repair of the defect, resection of the nonfunctional caudal cyst wall, reconstruction of the proximal neural placode, and duroplasty.

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