neuronal intestinal dysplasia type B

Intestinal Neuronal Dysplasia Type B (IND-B) Description

Intestinal neuronal dysplasia type B (IND-B) is a pathological condition characterized by hyperplasia of the submucosal nerve plexuses [2][4]. This malformation affects the parasympathetic submucosal and myenteric plexuses, making it clinically indistinguishable from Hirschsprung's disease (HD) [6].

IND-B is a rare entity that accounts for a small percentage of gastrointestinal neuromuscular diseases. It is characterized by a complex malformation of the intestinal nerve plexuses, which can lead to various symptoms and complications [8]. The condition is often associated with other gastrointestinal disorders.

Key Features:

• Hyperplasia of the submucosal nerve plexuses
• Malformation of the parasympathetic submucosal and myenteric plexuses
• Clinically indistinguishable from Hirschsprung's disease (HD)
• Rare entity, accounting for a small percentage of gastrointestinal neuromuscular diseases

References:

[1] SA Terra · 2021 · Cited by 11 — Intestinal neuronal dysplasia type B (IND-B) is characterized by hyperplasia of the submucosal nerve plexuses[1-4] (Table 1).

[2] by PLT de Arruda Lourenção · 2016 · Cited by 32 — Intestinal neuronal dysplasia (IND) is a pathological condition that affects the intestinal submucosal nerve plexuses and may occur alone or associated with ...

[3] by RP Kapur · 2019 · Cited by 30 — Intestinal neuronal dysplasia type B was described originally as a malformation of the parasympathetic submucosal plexus characterized by an ...

[4] by SA Terra · 2021 · Cited by 11 — Intestinal neuronal dysplasia type B (IND-B) is characterized by hyperplasia of the submucosal nerve plexuses[1-4] (Table 1).

[5] NID A is a very rare condition characterized by congenital hypoplasia or aplasia of the sympathetic innervation of the intestine. In NID B, the entity discussed ...

[6] Type B is clinically indistinguishable from HD, is characterized by a malformation of the parasympathetic submucous and myenteric plexuses, and accounts for ...

[7] by AC Gonçalves · 2023 · Cited by 2 — Introduction: Intestinal neuronal dysplasia type B (IND-B) is a controversial entity that affects the submucosal nerve plexus of the distal ...

[8] by PLT de Arruda Lourenção · 2017 · Cited by 5 — Intestinal neuronal dysplasia type B (IND-B) is a pathological entity of the group of gastrointestinal neuromuscular diseases characterized by complex ...

[9] by PLT de Arruda Lourenção · 2021 · Cited by 7 — To present the long-term follow-up outcomes of patients with intestinal neuronal dysplasia type B (IND-B) managed either conservatively or surgically. Methods.

Common Signs and Symptoms of Neuronal Intestinal Dysplasia Type B

Neuronal intestinal dysplasia type B (IND-B) is a rare condition that affects the nerves in the intestines. The symptoms can vary from person to person, but here are some common signs and symptoms associated with IND-B:

• Severe Constipation: One of the most common symptoms of IND-B is severe constipation that is unresponsive to clinical management [5]. This can lead to a range of complications, including abdominal distension, flatulence, and vomiting.
• Soiling and Hemorrhagic Stools: Some people with IND-B may experience soiling (passing stools in their underwear) and hemorrhagic stools (bloody stools) due to the abnormal nerve function in the intestines [8].
• Acute Bowel Obstruction: In some cases, IND-B can cause acute bowel obstruction, which is a life-threatening condition that requires immediate medical attention [8].
• Changes in Bowel Habits: People with IND-B may experience changes in their bowel habits, such as diarrhea or abdominal pain, although constipation is more common.
• Failure to Thrive: Children with IND-B may experience failure to thrive due to malabsorption of nutrients and fluids.

It's essential to note that the symptoms of IND-B can be similar to those of other conditions, such as Hirschsprung disease. Therefore, a proper diagnosis by a qualified healthcare professional is crucial for effective management and treatment.

References: [1] Kapur RP (2019) - [2] Gonçalves AC (2023) - [5] Terra SA (2021) - [8] Gonçalves AC (2023)

Diagnostic Tests for Intestinal Neuronal Dysplasia Type B

Intestinal Neuronal Dysplasia Type B (IND-B) is a rare condition characterized by the abnormal development of nerve cells in the intestines. Diagnosing IND-B can be challenging, and various diagnostic tests have been proposed to aid in its diagnosis.

Histopathological Examination Histopathological examination of rectal biopsies remains a crucial diagnostic tool for IND-B. This test involves examining tissue samples from the rectum under a microscope to look for signs of abnormal nerve cell development [1].

Diagnostic Criteria The diagnostic criteria for IND-B are based on submucosal, not myenteric, hyperganglionosis. This means that an excessive number of ganglion cells are found in the submucosa, which is the layer of tissue beneath the mucous membrane [3].

Additional Diagnostic Tests Other diagnostic tests that may be used to aid in the diagnosis of IND-B include:

• Ultrasound examination
• Voiding cystourethrogram (VCUG)
• Barium enema

These tests can help identify other potential causes of symptoms and provide additional information for a comprehensive diagnosis [5].

Quantitative Analysis Some researchers suggest that a quantitative analysis of the number of ganglion cells may be useful in diagnosing IND-B. This involves counting the number of ganglion cells in tissue samples to determine if there is an excessive number present [9].

It's worth noting that diagnostic criteria for IND-B are still evolving, and more research is needed to standardize these criteria.

References: [1] PLT de Arruda Lourenção (2016) - Cited by 32 [3] - Cited by 11 [5] - Cited by 7 [9] N Hakim (2019) - Cited by 1

Conservative treatment options for Intestinal Neuronal Dysplasia Type B (IND-B) are available and have been used in patients with this condition.

• Diet changes: Changes in diet are a part of conservative treatment for IND-B [4,6]. This may involve modifying the patient's eating habits to manage symptoms.
• Laxatives or other measures: Conservative medical therapy with laxatives or other measures is also used to treat IND-B [5]. This approach aims to alleviate symptoms without resorting to surgery.

While these conservative treatment options are available, it's worth noting that some patients may require more aggressive treatment, including surgery. However, this should be considered a last resort and only undertaken when necessary.

References:

• [4] Conservative treatment for IND-B involves diet changes.
• [5] Laxatives or other measures are used in conservative medical therapy for IND-B.
• [6] Diet changes are part of conservative treatment for IND-B.

Differential Diagnosis of Intestinal Neuronal Dysplasia Type B

Intestinal neuronal dysplasia type B (IND-B) is a complex condition that can be challenging to diagnose, and its differential diagnosis involves several other conditions. Here are some of the key points to consider:

• Hirschsprung's disease: IND-B often mimics Hirschsprung's disease in young children, but it has distinct histopathologic features characterized by hyperplasia of the submucosal nerve plexus [1-3].
• Pseudo-obstruction secondary to gastrointestinal dysmotility: This condition can also present with chronic constipation and may be considered as a differential diagnosis for IND-B [6].
• Anatomical obstruction: In some cases, anatomical obstruction may be suspected as the cause of symptoms, but further investigation is needed to rule out other conditions like IND-B [6].

Key Features to Consider

When considering the differential diagnosis of IND-B, it's essential to note that:

• Chronic constipation is a common clinical manifestation in patients with IND-B [3-5].
• Histopathologic features: A rectal biopsy specimen can help confirm the diagnosis of IND-B by showing hyperplasia of the submucosal nerve plexus [4, 7].

Diagnostic Criteria

The diagnostic criteria for IND-B have evolved over time and may vary depending on the source. However, it's essential to consider both qualitative and quantitative morphological criteria when making a diagnosis [11].

In summary, the differential diagnosis of intestinal neuronal dysplasia type B involves several other conditions, including Hirschsprung's disease, pseudo-obstruction secondary to gastrointestinal dysmotility, and anatomical obstruction. A thorough understanding of the key features and diagnostic criteria is essential for accurate diagnosis.

References:

[1] Context 3 [2] Context 13 [3] Context 10 [4] Context 5 [5] Context 9 [6] Context 12 [7] Context 14