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      Anesthetic Management of Laparoscopic Pyloromyotomy for Pyloric Stenosis in a Neonate with Hereditary Spherocytosis

      case-report
      Author(s): 1 , , 1 , 1 , 2 , 1
      Editor(s): ,
      Publication date (Electronic, pub):
      Publication date (Electronic, collection):
      Journal: Cureus
      Publisher: Cureus
      Keywords: hyperbilirubinemia, hereditary spherocytosis, hemolytic anemia, pyloric stenosis, neonate

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          Abstract

          We describe a case of hereditary spherocytosis in a neonate with pyloric stenosis requiring laparoscopic pyloromyotomy. Hereditary spherocytosis is the most commonly inherited hemolytic anemia causing hyperbilirubinemia and mild anemia. Anesthetic management for laparoscopic pyloromyotomy is challenging. Multiple factors involved, such as anemia, hyperbilirubinemia, and the effect of drugs, play an important role in anesthetic management.

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          Most cited references15

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          Red cell membrane disorders.

          J Narla, N Mohandas (2017)
          Significant advances have been made in our understanding of the structural basis for altered cell function in various inherited red cell membrane disorders with reduced red cell survival and resulting hemolytic anemia. The current review summarizes these advances as they relate to defining the molecular and structural basis for disorders involving altered membrane structural organization (hereditary spherocytosis [HS] and hereditary elliptocytosis [HE]) and altered membrane transport function (hereditary overhydrated stomatocytosis and hereditary xerocytosis). Mutations in genes encoding membrane proteins that account for these distinct red cell phenotypes have been identified. These molecular insights have led to improved understanding of the structural basis for altered membrane function in these disorders. Weakening of vertical linkage between the lipid bilayer and spectrin-based membrane skeleton leads to membrane loss in HS. In contrast, weakening of lateral linkages among different skeletal proteins leads to membrane fragmentation and decreased surface area in HE. The degrees of membrane loss and resultant increases in cell sphericity determine the severity of anemia in these two disorders. Splenectomy leads to amelioration of anemia by increasing the circulatory red cell life span of spherocytic red cells that are normally sequestered by the spleen. Disordered membrane cation permeability and resultant increase or decrease in red cell volume account for altered cellular deformability of hereditary overhydrated stomatocytosis and hereditary xerocytosis, respectively. Importantly, splenectomy is not beneficial in these two membrane transport disorders and in fact contraindicated due to severe postsplenectomy thrombotic complications.
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            Deficient red-cell spectrin in severe, recessively inherited spherocytosis.

            V Bennett, P Agre, E Orringer (1982)
            Article 0 comments Cited 19 times – based on 0 reviews     Review now
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              A comparison of awake versus paralyzed tracheal intubation for infants with pyloric stenosis.

              S Cook-Sather, M S Schreiner, H Tulloch … (1998)
              This prospective, nonrandomized, observational study of 76 infants with pyloric stenosis was conducted at an academic children's hospital and compared awake versus paralyzed tracheal intubation in terms of successful first attempt rate, intubation time, heart rate (HR) and arterial hemoglobin oxygen saturation (SpO2) changes, and complications. Three groups were determined by intubation method: awake (A) with an oxygen-insufflating laryngoscope, after rapid-sequence induction (R), or after modified rapid-sequence induction (M) including ventilation through cricoid pressure. Successful first attempt intubation rate was 64% for Group A versus 87% for paralyzed Groups R and M (P = 0.028). Median intubation time was 63 s in Group A versus 34 s in Groups R and M (P = 0.004). Transient, mild decreases in mean HR and SpO2 and incidences of significant bradycardia and decreased SpO2 did not vary by group. Complications, including bronchial or esophageal intubation, emesis, and oropharyngeal trauma, were few. Senior anesthesiologists intervened in four tracheal intubations. We advocate anesthetized, paralyzed tracheal intubation because struggling with conscious infants takes longer, often requires multiple attempts, and prevents neither bradycardia nor decreased SpO2. After induction, additional mask ventilation with O2 confers no advantage over immediate tracheal intubation in preserving SpO2.
              Article 0 comments Cited 14 times – based on 0 reviews     Review now
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                Author and article information

                Journal
                Journal ID (nlm-ta): Cureus
                Journal ID (iso-abbrev): Cureus
                Journal ID (issn): 2168-8184
                Title: Cureus
                Publisher: Cureus (Palo Alto (CA) )
                ISSN (Electronic): 2168-8184
                Publication date (Electronic, pub): 15 March 2020
                Publication date (Electronic, collection): March 2020
                Volume: 12
                Issue: 3
                Electronic Location Identifier: e7277
                Affiliations
                [1 ] Anesthesiology, NewYork-Presbyterian Brooklyn Methodist Hospital, Brooklyn, USA
                [2 ] Pediatrics/Neonatal Perinatal Medicine, Icahn School of Medicine at Mt. Sinai and New York City Health + Hospitals, Elmhurst, USA
                Author notes
                Article
                DOI: 10.7759/cureus.7277
                PMC ID: 7158607
                SO-VID: 399c3513-f197-465c-8050-97af032d4652
                Copyright © Copyright © 2020, Kamath et al.
                License:

                This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                History
                Date received : 9 December 2019
                Date accepted : 15 March 2020
                Categories
                Subject: Anesthesiology

                Keywords: hyperbilirubinemia,hereditary spherocytosis,hemolytic anemia,pyloric stenosis,neonate
                Data availability:
                Keywords: hyperbilirubinemia, hereditary spherocytosis, hemolytic anemia, pyloric stenosis, neonate

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