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James T. Goodrich

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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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Mahdi Arjipour, Farideh Nejat and Zohreh Habibi

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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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Sara Hanaei, Farideh Nejat, Abolghasem Mortazavi, Zohreh Habibi, Arash Esmaeili and Mostafa El Khashab

Lipomyelomeningocele, a congenital spine defect, is presented as skin-covered lipomatous tissue that attaches to the cord in different ways according to its subtypes. Unlike other types of neural tube defects, the exact cause of this birth defect has not been confirmed yet, but it is proposed to be a multifactorial disease with involvement of both genetic and environmental factors. The authors describe identical twins with lipomyelomeningocele of the same subtype and location without any familial history of similar abnormality. Therefore, the same genetic and/or environmental risk factors could have played a part in their condition.

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Farshid Farzaneh, Ehsan Moradi, Zohreh Habibi and Farideh Nejat

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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.

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Farideh Nejat, Zohreh Habibi and Mostafa El Khashab

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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.