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Duplication of the pituitary gland associated with multiple blastogenesis defects: Duplication of the pituitary gland (DPG)-plus syndrome. Case report and review of literature.

Manjila S, Miller EA, Vadera S, Goel RK, Khan FR, Crowe C, Geertman RT - Surg Neurol Int (2012)

Bottom Line: The complex craniocaudal spectrum of blastogenesis defects associated with DPG is examined with an illustrative case.The authors propose that the developmental deformities associated with duplication of pituitary gland (DPG) occur as part of a developmental continuum, not as chance associations.Considering the fact that DPG is uniquely and certainly present throughout the spectrum of these blastogenesis defects, we suggest the term DPG-plus syndrome.

View Article: PubMed Central - PubMed

Affiliation: Division of Neurosurgery, MetroHealth Medical Center, Cleveland, Ohio.

ABSTRACT

Background: Duplication of the pituitary gland (DPG) is a rare craniofacial developmental anomaly occurring during blastogenesis with postulated etiology such as incomplete twinning, teratogens, median cleft face syndrome or splitting of the notochord. The complex craniocaudal spectrum of blastogenesis defects associated with DPG is examined with an illustrative case.

Case description: We report for the first time in the medical literature some unique associations with DPG, such as a clival encephalocele, third cerebral peduncle, duplicate odontoid process and a double tongue with independent volitional control. This patient also has the previously reported common associations such as duplicated sella, cleft palate, hypertelorism, callosal agenesis, hypothalamic enlargement, nasopharyngeal teratoma, fenestrated basilar artery and supernumerary teeth. This study also reviews 37 cases of DPG identified through MEDLINE literature search from 1880 to 2011. It provides a detailed analysis of the current case through physical examination and imaging.

Conclusion: The authors propose that the developmental deformities associated with duplication of pituitary gland (DPG) occur as part of a developmental continuum, not as chance associations. Considering the fact that DPG is uniquely and certainly present throughout the spectrum of these blastogenesis defects, we suggest the term DPG-plus syndrome.

No MeSH data available.


Related in: MedlinePlus

T1 weighted MRI with contrast (a) axial image of duplicate pituitary gland (b) coronal image of duplicate pituitary gland (c) coronal image demonstrating two widely spaced pituitary stalks
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Figure 2: T1 weighted MRI with contrast (a) axial image of duplicate pituitary gland (b) coronal image of duplicate pituitary gland (c) coronal image demonstrating two widely spaced pituitary stalks

Mentions: Head MRI reveals duplication of multiple midline structures: two sellas and two pituitary glands, a duplicate ventricular system, two basilar arteries and a midline third cerebral peduncle [Figures 2a–c and 3a, b]. Her imaging is also notable for agenesis of the corpus callosum and a clival encephalocele Figure 3 with an adjacent midline nasopharyngeal tumor consistent with residual teratoma [Figure 4]. The craniocervical junction appeared abnormal with a widened C1 arch, C2 body duplication and two odontoid processes [Figure 5].


Duplication of the pituitary gland associated with multiple blastogenesis defects: Duplication of the pituitary gland (DPG)-plus syndrome. Case report and review of literature.

Manjila S, Miller EA, Vadera S, Goel RK, Khan FR, Crowe C, Geertman RT - Surg Neurol Int (2012)

T1 weighted MRI with contrast (a) axial image of duplicate pituitary gland (b) coronal image of duplicate pituitary gland (c) coronal image demonstrating two widely spaced pituitary stalks
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3307243&req=5

Figure 2: T1 weighted MRI with contrast (a) axial image of duplicate pituitary gland (b) coronal image of duplicate pituitary gland (c) coronal image demonstrating two widely spaced pituitary stalks
Mentions: Head MRI reveals duplication of multiple midline structures: two sellas and two pituitary glands, a duplicate ventricular system, two basilar arteries and a midline third cerebral peduncle [Figures 2a–c and 3a, b]. Her imaging is also notable for agenesis of the corpus callosum and a clival encephalocele Figure 3 with an adjacent midline nasopharyngeal tumor consistent with residual teratoma [Figure 4]. The craniocervical junction appeared abnormal with a widened C1 arch, C2 body duplication and two odontoid processes [Figure 5].

Bottom Line: The complex craniocaudal spectrum of blastogenesis defects associated with DPG is examined with an illustrative case.The authors propose that the developmental deformities associated with duplication of pituitary gland (DPG) occur as part of a developmental continuum, not as chance associations.Considering the fact that DPG is uniquely and certainly present throughout the spectrum of these blastogenesis defects, we suggest the term DPG-plus syndrome.

View Article: PubMed Central - PubMed

Affiliation: Division of Neurosurgery, MetroHealth Medical Center, Cleveland, Ohio.

ABSTRACT

Background: Duplication of the pituitary gland (DPG) is a rare craniofacial developmental anomaly occurring during blastogenesis with postulated etiology such as incomplete twinning, teratogens, median cleft face syndrome or splitting of the notochord. The complex craniocaudal spectrum of blastogenesis defects associated with DPG is examined with an illustrative case.

Case description: We report for the first time in the medical literature some unique associations with DPG, such as a clival encephalocele, third cerebral peduncle, duplicate odontoid process and a double tongue with independent volitional control. This patient also has the previously reported common associations such as duplicated sella, cleft palate, hypertelorism, callosal agenesis, hypothalamic enlargement, nasopharyngeal teratoma, fenestrated basilar artery and supernumerary teeth. This study also reviews 37 cases of DPG identified through MEDLINE literature search from 1880 to 2011. It provides a detailed analysis of the current case through physical examination and imaging.

Conclusion: The authors propose that the developmental deformities associated with duplication of pituitary gland (DPG) occur as part of a developmental continuum, not as chance associations. Considering the fact that DPG is uniquely and certainly present throughout the spectrum of these blastogenesis defects, we suggest the term DPG-plus syndrome.

No MeSH data available.


Related in: MedlinePlus