Persisting embryonal infundibular recess (PEIR) and transsphenoidal-transsellar encephaloceles: distinct entities or constituents of one continuum?

Azab, Waleed A.; Cavallo, Luigi Maria; Yousef, Waleed; Khan, Tufail; Solari, Domenico; Cappabianca, Paolo

doi:10.1007/s00381-022-05467-x

Cited by 5 publications

(5 citation statements)

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“…In addition, most of the patients presented with at least one congenital anomaly like cleft lip/palate, hypertelorism, eye problems, genital abnormalities or lingual midline mass. It is postulated that the development of congenital cleft palate/lip, hypertelorism, and eye problems may originate from the same embryonic tissues involved in the formation of the pituitary gland and other structures associated with TSTSE [ 5 ]. On the other hand, the presence of genital abnormalities is likely attributed to hypogonadism, a consequence of the encephalocele.…”

Section: Discussionmentioning

confidence: 99%

“…By assessing the endocrine function, comprehensive hormonal profiles can be obtained, enabling the identification of any hormonal deficiencies or imbalances associated with the encephalocele. This evaluation helps guide appropriate perioperative interventions and facilitates the implementation of long-term hormone replacement therapy when necessary, ensuring optimal outcomes and addressing the patient’s overall well-being [ 5 ].…”

Section: Discussionmentioning

confidence: 99%

“…Basal encephaloceles have conventionally been categorized into five distinct anatomical types: spheno-ethmoidal, trans-sphenoidal, trans-sphenoidal trans-sellar, spheno-orbital, trans-ethmoidal, and spheno-maxillary [ 4 ]. With the trans-sphenoidal trans-sellar being the rarest among all [ 3 , 5 ].…”

Section: Introductionmentioning

confidence: 99%

“…However, it is widely hypothesized to arise from an embryological defect in the skull base. Among the proposed mechanisms, the persistence of the craniopharyngeal canal (CPC) is currently the most widely accepted theory explaining the pathogenesis of TSE [ 5 – 7 ]. It is postulated that an early intrauterine insult occurring during the critical period of pituitary development may give rise to cascading effects on underdeveloped tissues such as the optic chiasm and osseous sphenoid elements.…”

Section: Introductionmentioning

confidence: 99%

“…Clinical presentations include regional symptoms like respiratory difficulties, feeding difficulties, pulsatile nasopharyngeal mass, recurrent episodes of meningitis, and cerebrospinal fluid (CSF) rhinorrhea; associated craniofacial anomalies like cleft lip and palate, ocular anomaly, craniosynostosis, hypertelorism; endocrinal presentations like hypothyroidism, hypopituitary-related growth retardation, diabetes insipidus, and so on [ 5 , 8 , 9 ].…”

Section: Introductionmentioning

confidence: 99%

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Congenital trans-sellar trans-sphenoidal encephalocele: a systematic review of diagnosis, treatment, and prognosis

Jiang,

Yang,

Ainiwaer

et al. 2024

Eur Arch Otorhinolaryngol

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Purpose Clinical presentations encompass respiratory, feeding issues, nasopharyngeal mass, meningitis, CSF leakage, craniofacial anomalies, and endocrine problems. Surgery is the primary treatment, transitioning from frontal craniotomy to endoscopic methods, offering improved outcomes. Yet, more studies are needed. A comprehensive review on trans-sellar trans-sphenoidal encephalocele (TSTSE) is missing. Our study aims to fill this gap, offering a comprehensive perspective for physicians. Methods This review adhered to the PRISMA guideline. Eligible studies focused on human subjects, specifically trans-sellar encephaloceles, and provided comprehensive treatment details. English language articles published up to April 11th, 2023, were considered. Two trained researchers conducted article screening using consistent criteria. Data extraction covered various aspects, including clinical presentation, surgical methods, and outcomes, with results presented descriptively in two tables. Due to the rarity of this congenital anomaly, meta-analysis and publication bias assessment were not feasible. Data extraction was independently conducted by two reviewers, with subsequent cross-verification. Results A total of 36 patients were identified from 14 studies, the most frequently observed clinical presentation was dyspnea (41.67%) and the most frequently observed accompanying anomaly was cleft lip/palate (55.56%). CT and MRI were adopted in nearly all the cases, and trans-nasal approach was the most often used surgical approach (57.14%) with the ‘soft material combination’ the most commonly used method for cranial base repairment (35.71%). A total of two deaths occurred and diabetes insipidus was the most common perioperative complication which occurred in six surgery patients (21.43%). Conclusion TSTSE predominantly affects males and presents with dyspnea, visual deficits, pituitary insufficiency, and cranial base-related symptoms. Early diagnosis is critical, with advanced imaging playing a key role. Endocrine assessment is vital for hormone management. Surgery offers symptom relief but entails risks, including reported fatalities and complications. The choice between surgery and conservative management requires careful deliberation. The trans-nasal approach is favored for its reduced trauma, yet further research is necessary to validate this preference.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%