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Ghazaleh Kheiri, Negin Naderian, Sajedeh Karami, Zohreh Habibi, and Farideh Nejat

OBJECTIVE

Prenatal ventriculomegaly is classified as mild, moderate, or severe based on the atrium diameter. The natural course and intrauterine progression of mild and moderate ventriculomegaly associated with the neurodevelopmental status of these children has been widely reported.

METHODS

One hundred twenty-two pregnancies with mild and moderate ventriculomegaly referred to the pediatric neurosurgery clinic of Children’s Medical Center between 2010 and 2018 were retrospectively studied. The authors collected demographic and first and sequential ultrasonographic information, associated abnormalities, information about pregnancy outcomes, and the latest developmental status of these children according to Centers for Disease Control and Prevention criteria by calling parents at least 1 year after birth.

RESULTS

The mean gestational age at the time of diagnosis was 29.1 weeks, and 53% of fetuses were female. The width of the atrium was registered precisely in 106 cases, in which 61% had mild and 39% had moderate ventriculomegaly. Information on serial ultrasound scans was collected in 84 cases in which ventriculomegaly regressed in 5, remained stable in 67, and progressed in 12 patients. Fourteen cases (29.7%) in the mild ventriculomegaly group and 6 cases (16.2%) in the moderate group had associated abnormalities, with corpus callosum agenesis as the most frequent abnormality. The survival rate was 80% in mild and 89.4% in moderate ventriculomegaly. Considering survival to live birth and progression of the ventriculomegaly, the survival rate was 100% in regressed, 97% in stable, and 41.6% in progressed ventricular width groups (p < 0.001). Neurodevelopmental status was evaluated in 73 cases and found to be normal in 69.8% of the cases; 16.4% of children had mild delay, and 5.4% and 8.2% of cases were diagnosed with moderate and severe delays, respectively.

CONCLUSIONS

In spite of a high rate of missed data in our retrospective study, most patients with mild or moderate ventriculomegaly had a stable or regressed course. Most cases had near-normal developmental status. Prospective studies with a larger sample size and detailed developmental evaluation tests are needed to answer the questions related to the natural course, survival, and prognosis of prenatal ventriculomegaly.

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James T. Goodrich

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Mahdi Arjipour, Farideh Nejat, and Zohreh Habibi

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Majid Dadmehr, Farideh Nejat, Saeed Ansari, and Zohreh Habibi

✓ Occipitocervical teratoma is an extremely rare condition, and only a few cases have been reported. The authors report on a neonate who harbored a ruptured posterior midline occipitocervical lesion that mimicked an upper cervical myelomeningocele, although the pathological findings were diagnostic of teratoma.

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Shima Shahjouei, Sara Hanaei, Zohreh Habibi, Mostafa Hoseini, Saeed Ansari, and Farideh Nejat

OBJECTIVE

No evidence-based guideline has been approved for the postoperative management of pediatric patients with tethered cord syndrome (TCS). The purpose of this randomized clinical trial was to evaluate the effectiveness of prone positioning and acetazolamide administration on complication rates following spinal cord untethering surgeries.

METHODS

From October 2012 to February 2015, patients with a primary diagnosis of TCS who were admitted to the Children's Medical Center Hospital of Iran were randomly allocated to 1 of 4 intervention modality groups postoperatively: 1) Group A, acetazolamide administration for 10 days; 2) Group B, prone positioning for 10 days; 3) Group C, acetazolamide administration and prone positioning for 10 days; and 4) Group D, no intervention. CSF leakage, CSF collection, wound dehiscence, operative site infection, and secondary surgical wound repair were considered failure.

RESULTS

A total of 161 patients were enrolled in this study (Group A, n = 39 [24.2%]; Group B, n = 41 [25.5%]; Group C, n = 39 [24.2%]; and Group D, n = 42 [26.1%]). The overall failure rate was 12.42% (20 patients). Complication rates through pooled analyses were as follows: CSF leakage (n = 9, 5.6%), CSF collection (n = 12, 7.5%), wound dehiscence (n = 2, 1.2%), and infection of operation site (n = 3, 1.9%). Two patients (1.2%) required surgical secondary wound repair due to complications. CSF leakage and collection rates were significantly lower in patients who underwent prone positioning (p = 0.042 and 0.036, respectively). The administration of acetazolamide, either isolated or in combination with prone positioning, not only could not significantly lower the complication rates, but also added the burden of side effects.

CONCLUSIONS

The current study demonstrates the possible role of prone positioning in mitigating the complication rates subsequent to untethering surgeries.

Clinical trial registration no.: NCT01867268 (clinicaltrials.gov)

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Mehdi Zeinalizadeh, Seyed Mousa Sadrehosseini, Zohreh Habibi, Farideh Nejat, Harley Brito da Silva, and Harminder Singh

OBJECTIVE

Congenital transsphenoidal encephaloceles are rare malformations, and their surgical treatment remains challenging. This paper reports 3 cases of transsphenoidal encephalocele in 8- to 24-month-old infants, who presented mainly with airway obstruction, respiratory distress, and failure to thrive.

METHODS

The authors discuss the surgical management of these lesions via a minimally invasive endoscopic endonasal approach, as compared with the traditional transcranial and transpalatal approaches. A unique endonasal management algorithm for these lesions is outlined. The lesions were repaired with no resection of the encephalocele sac, and the cranial base defects were reconstructed with titanium mesh plates and vascular nasoseptal flaps.

RESULTS

Reduction of the encephalocele and reconstruction of the skull base was successfully accomplished in all 3 cases, with favorable results.

CONCLUSIONS

The described endonasal management algorithm for congenital transsphenoidal encephaloceles is a safe, viable alternative to traditional transcranial and transpalatal approaches, and avoids much of the morbidity associated with these open techniques.

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Zohreh Habibi, Mehdi Golpayegani, Bahar Ashjaei, Keyvan Tayebi Meybodi, and Farideh Nejat

OBJECTIVE

Distal catheter malfunction due to pseudocyst formation or intraabdominal adhesion is a significant problem, especially in pediatric patients who have limited sites for distal catheter insertion. In this study, the authors present a series of 12 patients with intractable distal shunt malfunctions due to peritoneal pseudocyst formation who underwent distal catheter replacement in the suprahepatic space to reduce the risk of distal catheter malfunction.

METHODS

Twelve consecutive patients with shunt malfunction due to pseudocyst formation who had undergone ventriculosuprahepatic shunting from 2014 to 2019 were identified. According to medical records, after primary evaluations, shunt removal, and antibiotic therapy, they underwent revision surgeries with placement of a distal catheter into the suprahepatic space.

RESULTS

Nine boys and 3 girls, ranging in age from 5 months to 14 years, with one or more episodes of pseudocyst formation, underwent shunt revision with placement of a distal catheter into the suprahepatic space. After a median follow-up of 31 months, none of the patients experienced further distal malfunction.

CONCLUSIONS

The suprahepatic space appears to be a safe place to secure the distal end of a ventricular catheter following pseudocyst formation, with less risk of re-adhesion. This lower risk might be attributable to the lack of omentum in the suprahepatic space.

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Esmaeil Mohammadi, Sara Hanaei, Sina Azadnajafabad, Keyvan Tayebi Meybodi, Zohreh Habibi, and Farideh Nejat

OBJECTIVE

The role of tunneling an external ventricular drain (EVD) more than the standard 5 cm for controlling device-related infections remains controversial.

METHODS

This is a randomized, double-blind, 3-arm controlled trial done in the Children’s Medical Center in Tehran, Iran. Pediatric patients (< 18 years old) with temporary hydrocephalus requiring an EVD and no evidence of CSF infection or prior EVD insertion were enrolled. Patients were randomly assigned (1:1:1) into the following arms: 5-cm (standard; group A); 10-cm (group B); or 15-cm (group C) EVD tunnel lengths. The investigators, parents, and person performing the analysis were masked. The surgeon was informed of the length of the EVD by the monitoring board just before operation. Patients were followed until the EVD’s fate was established. Infection rate and other complications related to EVDs were assessed.

RESULTS

A total of 105 patients were enrolled in three random groups (group A = 36, group B = 35, and group C = 34). The EVD was removed because there was no further need in most cases (67.6%), followed by conversion to a new EVD or ventriculoperitoneal shunt (15.2%), infection (11.4%), and spontaneous discharge without further CSF diversion requirement (5.7%). No statistical difference was found in infection rate (p = 0.47) or EVD duration (p = 0.81) between the three groups. No group reached the efficacy point sooner than the standard group (group B: hazard ratio 1.21, 95% CI 0.75–1.94, p = 0.429; group C: hazard ratio 1.03, 95% CI 0.64–1.65, p = 0.91).

CONCLUSIONS

EVD tunnel lengths of 5 cm and longer did not show a difference in the infection rate in pediatric patients. Indeed, tunneling lengths of 5 cm and greater seem to be equally effective in preventing EVD infection.

Clinical trial registration no.: IRCT20160430027680N2 (IRCT.ir)

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Zohreh Habibi, Farideh Nejat, Parisa Emami Naeini, and Fatemeh Mahjoub

Object

A myelomeningocele (MMC) can be associated with paraplegia, bladder and bowel incontinence, Chiari malformation Type II, and hydrocephalus. The coincidence of an MMC and a neoplasm is rare, and only limited reports on the concurrence of a teratoma within an MMC have been published.

Methods

A retrospective study was performed using the records of 330 children who underwent operations to correct an MMC at the Children's Hospital Medical Center in Tehran between January 2001 and June 2005. The postoperative histopathological assessments in 15 (4.5%) of these patients revealed evidence of a teratoma inside the MMC. The age of these patients at admission ranged from 2 days to 2 years (median 30 days). Neurological findings were normal in all patients except for three with lower-extremity weakness or paralysis. Hydrocephalus was detected in six patients. Physical appearance of the teratoma in all cases included cystic or solid soft-tissue masses in the dorsal midline area, covered with abnormal rudimentary skin. Pedunculated skin tags, a primitive genitalia-like appendage, dermal sinus, a human tail, and cutaneous stigmata such as color changes, hemangioma, dysplastic skin, and tufts of hair around the mass were occasionally observed.

Conclusions

The special feature of a protruding fingerlike appendage or intraoperative detection of a cystic portion of an MMC without direct connection to the spinal canal can be possible signs of teratoma concurrent with an MMC. Teratoma inside an MMC is a benign neoplasm, without any recurrence after standard surgery for an MMC.