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PubMed This is a summary of 19 peer-reviewed journal articles Updated
Pediatrics

Classification and Diagnosis: Understanding the Gap

At a Glance

A laryngeal cleft is diagnosed through a functional swallow study (MBS) to check for aspiration, followed by a Microlaryngobronchoscopy (MLB) to visually confirm the gap. Doctors classify the cleft's severity from Type I to Type IV using the Benjamin-Inglis system to guide treatment.

Understanding the biology and the diagnostic process of a laryngeal cleft helps clarify why certain tests are necessary. A laryngeal cleft is a congenital foregut malformation, meaning it is a structural issue that occurs very early in pregnancy [1][2].

The Biology: Why Clefts Form

Between the third and sixth weeks of pregnancy, a baby’s foregut (the early structure that becomes the throat and stomach) begins to divide into two separate tubes: the trachea (windpipe) and the esophagus (food pipe) [3].

This division is guided by a wall of tissue called the tracheoesophageal septum. If these tissues fail to fuse or “zipper” shut correctly, a gap remains [4][5]. This gap is the laryngeal cleft. Because this process happens so early, it is often associated with other “midline” issues or abnormalities in the digestive tract [6].

The Benjamin-Inglis Classification

To help doctors decide on the best treatment, they use the Benjamin-Inglis classification system. This system grades the cleft based on how deep the gap extends [7][8]:

  • Type I: A small gap that extends down to the level of the vocal cords [8][9]. These are often the hardest to diagnose because the gap is subtle.
  • Type II: The cleft extends through the cricoid cartilage (the ring-shaped cartilage at the top of the windpipe) [7][8].
  • Type III: The cleft extends further down into the cervical trachea (the windpipe in the neck) [7][10].
  • Type IV: The most severe form, where the cleft extends deep into the chest, sometimes all the way to where the windpipe splits into the lungs (the carina) [8][11].

The Path to Diagnosis

Diagnosis typically happens in two stages: functional assessment (how the throat works) and anatomical confirmation (how the throat looks).

1. Functional Assessment: Modified Barium Swallow (MBS)

Before surgery is considered, doctors usually order a Modified Barium Swallow (MBS), also known as a Videofluoroscopic Swallow Study (VFSS).

  • How it works: Your child swallows different consistencies of food or liquid mixed with barium (a safe substance that shows up on X-rays) while a video X-ray is taken [12][13].
  • What it tells us: It shows exactly when and how liquid enters the airway. It helps the team determine if thickening liquids or changing feeding positions can safely manage the symptoms without surgery [12][14].
  • What to Expect on Test Day: Before both the MBS and MLB, your child will need to fast (have an empty stomach) for several hours to prevent aspiration during the procedure [12]. Your team will provide specific instructions based on your child’s age.

2. The Gold Standard: Microlaryngobronchoscopy (MLB)

While X-rays are helpful, they cannot confirm a cleft. The “gold standard” for diagnosis is Microlaryngobronchoscopy (MLB) [15].

  • The Procedure: Under general anesthesia, a pediatric otolaryngologist (ENT) uses a specialized telescope and microscope to look directly at the airway [16][17].
  • Palpation: This is the most critical part of the MLB. Because some clefts are “hidden” by redundant tissue, the surgeon will use a small probe to gently pull on the tissue (palpate) to see how deep the gap actually goes [18][9].
  • Synchronous Lesions: The surgeon will also check for other airway issues, such as laryngomalacia (floppy airway tissue), which can occur alongside a cleft [16][19].

Confirming the exact type and depth of the cleft during an MLB is the only way to create an accurate surgical or management plan [9][7].

Common questions in this guide

How is a laryngeal cleft diagnosed?
Doctors use a two-step process. First, a Modified Barium Swallow (MBS) evaluates how your child swallows and if liquid enters the airway. Next, an ear, nose, and throat (ENT) surgeon performs a Microlaryngobronchoscopy (MLB) under anesthesia to directly look at the airway and confirm the cleft.
What are the different types of laryngeal clefts?
The Benjamin-Inglis system grades a laryngeal cleft from Type I to Type IV based on how deep the gap extends between the windpipe and the esophagus. Type I is a small gap near the vocal cords, while Type IV is the most severe, extending deep into the chest.
Why does my child need an MLB if they already had a swallow study?
While a swallow study shows if liquid is entering the airway, it cannot physically confirm a structural cleft. An MLB allows the surgeon to look directly at the airway and gently probe the tissue to uncover hidden gaps that X-rays miss.
Why do laryngeal clefts form?
A laryngeal cleft is a structural malformation that happens early in pregnancy. Between the third and sixth weeks, the tissue wall meant to separate the baby's windpipe from the food pipe fails to fuse completely, leaving a gap.
Can a laryngeal cleft be managed without surgery?
Depending on the results of the swallow study and the cleft's depth, some mild cases can be managed safely without surgery by changing feeding positions or thickening liquids to prevent aspiration. Your care team will guide you on the safest feeding plan.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.What specific type (I, II, III, or IV) did you observe during the microlaryngobronchoscopy?
  2. 2.During the MLB, did you find any other airway issues, like laryngomalacia, that could be adding to my child's symptoms?
  3. 3.In the Modified Barium Swallow, exactly which liquid consistencies (thin, nectar, or honey) caused aspiration?
  4. 4.Does my child have a deep interarytenoid notch that looks like a cleft but might be a normal variation?
  5. 5.How did you use palpation during the procedure to confirm the depth of the cleft?

Questions For You

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References

References (19)
  1. 1

    Gastric tumor mimicking bronchial tissue associated with a laryngotracheoesophageal cleft: a case report.

    Nakatani E, Terui K, Nakata M, et al.

    Surgical case reports 2023; (9(1)):72 doi:10.1186/s40792-023-01650-7.

    PMID: 37156975
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    [Clinical diagnosis and treatments of type Ⅱ-Ⅳ congenital laryngotracheoesophageal cleft in 8 children].

    Zhao J, Wang GX, Wang H, et al.

    Zhonghua er bi yan hou tou jing wai ke za zhi = Chinese journal of otorhinolaryngology head and neck surgery 2021; (56(9)):972-976 doi:10.3760/cma.j.cn115330-20201029-00839.

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    Diagnosis and Management of an H-type Tracheoesophageal Fistula in a Neonate: A Case Report.

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    Cureus 2024; (16(8)):e66618 doi:10.7759/cureus.66618.

    PMID: 39258070
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    Familial and genetic factors in laryngeal cleft: Have we learned anything?

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    International journal of pediatric otorhinolaryngology 2020; (138()):110283 doi:10.1016/j.ijporl.2020.110283.

    PMID: 32771712
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    Primary cilia are critical for tracheoesophageal septation.

    Fitzsimons LA, Tasouri E, Willaredt MA, et al.

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    [Evaluation and treatment of children's laryngeal clefts].

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    Zhonghua er bi yan hou tou jing wai ke za zhi = Chinese journal of otorhinolaryngology head and neck surgery 2018; (53(1)):9-15 doi:10.3760/cma.j.issn.1673-0860.2018.01.003.

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    Laryngeal cleft: A literature review.

    Martha VV, Vontela S, Calder AN, et al.

    American journal of otolaryngology 2021; (42(6)):103072 doi:10.1016/j.amjoto.2021.103072.

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    Current management of type III and IV laryngotracheoesophageal clefts: the case for a revised cleft classification.

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    [Diagnosis and treatment of disorders of laryngeal airway in children and prospect].

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    PMID: 38114307
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    Repair of short type IV laryngotracheoesophageal cleft using long, tapered, engaging graft without need for tracheotomy.

    Propst EJ

    The Laryngoscope 2016; (126(4)):1006-8 doi:10.1002/lary.25472.

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    Swallowing dysfunction among patients with laryngeal cleft: More than just aspiration?

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    International journal of pediatric otorhinolaryngology 2016; (82()):38-42.

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    Spectrum of swallowing abnormalities in children with Type I laryngeal cleft.

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    International journal of pediatric otorhinolaryngology 2022; (163()):111380 doi:10.1016/j.ijporl.2022.111380.

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    Clinical predictors of laryngotracheoesophageal clefts and tracheoesophageal fistulae in children with dysphagia.

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    The changing face of paediatric airway endoscopic surgery: An 8-year single surgeon review.

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This information about laryngeal cleft diagnosis is for educational purposes only. Always consult your child's pediatric otolaryngologist or care team for specific medical advice and diagnostic testing.

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