scholarly journals Cleft Palate and Vascular Malformation in a Pediatric Patient With PIK3CA-related Overgrowth Spectrum

2021 ◽  
Vol 3 (1) ◽  
pp. e032
Author(s):  
Rishub K. Das ◽  
Alexandra J. Borst ◽  
James D. Phillips
2021 ◽  
Vol 9 ◽  
Author(s):  
Francesca Grussu ◽  
Luigino Santecchia ◽  
Urbano Urbani ◽  
Giorgio Spuntarelli ◽  
Massimo Rollo ◽  
...  

Introduction: Vascular orbital lesions in pediatric population represent a demanding therapeutic challenge which requires a multidisciplinary team. In severe cases, orbital enucleation can be considered. Surgical management of enucleated orbital region in children, differently from the adults, represents a challenging procedure owing to the intrinsic relation between volume replacement and normal orbital growth. Many reconstructive options have been proposed, and many donor sites have been utilized for this purpose but each one have demonstrated potential disadvantages. Despite its well-known versatility, no report of the vastus lateralis free flap in children requiring orbital reconstruction exists in literature. Herein, we propose this surgical strategy as a valid option for the reconstruction of an extended orbital defect in a pediatric patient suffering from a mixed type of vascular malformation.Material and Methods: A patient was referred from a foreign country with an unclear medical history, presenting exorbitism and exophthalmos, proptosis of the eyeball, visus 4/10, and limited ocular motility. We made clinical-instrumental investigations with a diagnosis of complex vascular malformation. It expanded in intraorbital and retrorbital space with bulb anterior dislocation and optic nerve involvement. We performed an emptying of the orbital content via transconjunctival and via coronal incision with eyelid preservation. A free vastus lateralis muscle flap was used for reconstruction, filling the orbital cavity. We anastomosed the flap on the superficial temporal artery. An ocular conformator was then positioned.Results: We report the result at 12 months, showing a good orbital rehabilitation with an adequate prosthetic cavity, a good recovery of volume and facial symmetry, guaranteeing balanced orbital and periorbital growth. There were no major or minor complications associated with the procedure.Discussion: The reconstruction of the orbit remains a “surgical challenge” both in adults, whose goal is the restoration of volume, adequate symmetry and facial esthetics, and children, in which correcting the asymmetry has the additional objective to balance orbital growth. Many reconstructive techniques have been proposed, including the use of free flaps. The versatility of the free vastus lateralis muscle flap is well-known. It offers adequate amount of tissue with minimal morbidity to the donor site, provides a long pedicle, gives the possibility of simultaneous work in a double team, and has a constant anatomy and a safe and rapid dissection. There are no descriptions of its use for pediatric orbital reconstructions.Conclusions: In our opinion, the free vastus lateralis flap should be included as one of the best option for orbital pediatric reconstruction after enucleation.


2022 ◽  
Vol 13 ◽  
pp. 6
Author(s):  
Michel Gustavo Mondragon-Soto ◽  
Lior Elkaim ◽  
Alexander G. Weil

Background: Ventriculoperitoneal shunt (VPS), the mainstay of the treatment for hydrocephalus, is associated with relatively high revision rates. Transient hydrocephalus due to intermittent VPS obstruction should be recognized as a cause of VPS malfunction. While transient VPS dysfunction is well-recognized complication, there is a relative paucity of well-documented cases in the literature. Case Description: We present the case of a 4-year-old boy with a history of vascular malformation and hydrocephalus secondary to intraventricular hemorrhage. The patient presented with transient, self-resolving hydrocephalus (without intervention), as documented by clinical and radiological findings. Conclusion: Transient hydrocephalus due to intermittent VPS dysfunction in children is a rare entity, but it should be suspected in certain patients with VPS presenting with transient or self-improving symptoms.


1975 ◽  
Vol 6 (3) ◽  
pp. 119-124 ◽  
Author(s):  
Robert T. Wertz ◽  
Michael D. Mead

Typical examples of four different speech disorders—voice, cleft palate, articulation, and stuttering—were ranked for severity by kindergarten, first-grade, second-grade, and third-grade teachers and by public school speech clinicians. Results indicated that classroom teachers, as a group, moderately agreed with speech clinicians regarding the severity of different speech disorders, and classroom teachers displayed significantly more agreement among themselves than did the speech clinicians.


2020 ◽  
Vol 51 (4) ◽  
pp. 914-938
Author(s):  
Anna Cronin ◽  
Sharynne McLeod ◽  
Sarah Verdon

Purpose Children with a cleft palate (± cleft lip; CP±L) can have difficulties communicating and participating in daily life, yet speech-language pathologists typically focus on speech production during routine assessments. The International Classification of Functioning, Disability and Health: Children and Youth Version (ICF-CY; World Health Organization, 2007 ) provides a framework for holistic assessment. This tutorial describes holistic assessment of children with CP±L illustrated by data collected from a nonclinical sample of seven 2- to 3-year-old children, 13 parents, and 12 significant others (e.g., educators and grandparents). Method Data were collected during visits to participants' homes and early childhood education and care centers. Assessment tools applicable to domains of the ICF-CY were used to collect and analyze data. Child participants' Body Functions including speech, language, and cognitive development were assessed using screening and standardized assessments. Participants' Body Structures were assessed via oral motor examination, case history questionnaires, and observation. Participants' Activities and Participation as well as Environmental and Personal Factors were examined through case history questionnaires, interviews with significant others, parent report measures, and observations. Results Valuable insights can be gained from undertaking holistic speech-language pathology assessments with children with CP±L. Using multiple tools allowed for triangulation of data and privileging different viewpoints, to better understand the children and their contexts. Several children demonstrated speech error patterns outside of what are considered cleft speech characteristics, which underscores the importance of a broader assessment. Conclusion Speech-language pathologists can consider incorporating evaluation of all components and contextual factors of the ICF-CY when assessing and working with young children with CP±L to inform intervention and management practices.


1965 ◽  
Vol 30 (2) ◽  
pp. 166-173 ◽  
Author(s):  
Alta R. Brooks ◽  
Ralph L. Shelton ◽  
Karl A. Youngstrom

1966 ◽  
Vol 31 (2) ◽  
pp. 208-208

In the February 1966 issue of this journal, two errors occurred in Joan C. Pitzner’s and Hughlett L. Morris’s article “Articulation Skills and Adequacy of Breath Pressure Ratios of Children with Cleft Palate.” On page 29, the heading “Reality” should be “Reliability,” and on page 30, the heading “Pressure-Ration Group One” should be “Pressure-Ratio Group One.”


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