Median Arcuate Ligament Syndrome (MALS)

    Median Arcuate Ligament Syndrome (MALS)

Rare condition characterized by abdominal pain attributed to compression of the celiac artery and the celiac ganglia by the median arcute ligament. The abdominal pain may be related to meals, may be accompanied by weight loss, and may be associated with an abdominal bruit herd by a clinician. 

  MALS  may occur in anyone, even children. other names of MALS are:

- Celiac artery compression

- Celica axis syndrome

- Dunbar syndrome

 Symptoms:

  Often, compression of the celiac artery doesn't cause any symptoms. 

 However those with median arcuate ligament syndrome(MALS) can have long-term(chronic) stomach pain. The symptoms may be due to a lack of blood flow through the celaic artery or compression on nerves in the area. 

Symptoms of MALS include: 

- Pain in the upper middle stomcah area, which may go away when leaning forward 

- Stomch pain after eating , exercising or changing body position. 

- Bloting

- Diarrhea: Diarrhea is a common symptoms, some experience constipation. While some experience vomiting, not everyone dose. Exercise or certain postures can aggravate the symptoms. Occasionlly, physical examination reveals an abdominal bruit in the mid-epigastrium. 

- Fear of eating food due to pain, leading to significant weight loss.

 

- Nausea and vomiting. 

 If your stomach pain is severe and activity or movement makes it worse, call your provider immediately. Seek immediate medical help if your stomach pain occurs with:

- Bloody stools

- Fever

- Nausea and vomiting that doesn't go away

- severe tenderness when you touch your belly area

- Swelling of the belly area

- Yellowing of the skin or whites of the eyes(Jaundice). 

 Sometimes upper stomach pain can be confused with chest pin. Sometimes it can be due to a heart attack. Upper stomach pain with or without any of the following symptoms: 

 - pressure, fullness or tightness in your chest

- Crushing or searing pain that spreads to your jaw, neck, shoulders, and one or both arms. 

- Pain that lasts more than a few minutes or gets worse with activity

- Shortness of breath

- cold sweats

- Dizziness or weakness

- Nausea or vomiting.

Risk factors:

- Because the cause of MALS is poorly understood, the risk factors for the syndrome are unclear. MALS has been seen in children, even twins, which might mean genetics plays a role. Some people have developed MALS after pancreatic surgery and blunt injury to the upper stomach area. 

Prognosis:

 

 There are few studies of the long-term outcomes of patients treated for MALS. according to Duncan, the largest and more relevant late outcomes data come from a study of 51 patients who underwent open surgical treatment for MALS, 44 of whom were available for long-term follow-up at an average of nine yers following therapy. 

- The investigators reported that among patients who underwent celiac artey decompression and revascularization, 75% remained asymptomatic at follow-up. In the study, predictors of favorable outcome included

- Age from 40 to 60 years

- Lack of psychiatric condition or alcohol use

- Abdominal pain that was worse after meals

- Weight loss greater than 20 lb. 

 Complications:

 MALS complications include long-term pain, especially after meals. The pain cn led to a fear of eating and significant weight loss. The pain and related depression or anxiety can greatly impact. 

 The pain and related depression or anxiety can greatly impact quality of life. MALS symptoms may be vague and can mimic other conditions. It may take some time to get an accurate diagnosis. 

 

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Ellis-van Creveld syndrome

                            Ellis-van Creveld syndrome

      Ellis-van Creveld syndrome is an autosomal recessive skeletal dysplasia, which is characterized by short stature, retarded growth, polydactyly, and ectodermal and heart defects. The first case of EVC syndrome was reported by Mclntosh in 1933, but Richard W, B, Ellis of Edinburgh, and Simon van Creveld of Amsterdam in 1940, first described this condition and defined it as EVC syndrome. It is also known as chondroectodermal dysplasia and meso-ectodermal dysplasia. 

  The features of Ellis-van Creveld syndrome overlap with those of another, a milder condition called Weyers acrofacial dysostosis. Like Ellis- van Crevelf syndrome, Weyers acrofacial dysostosis involves tooth and nail abnormalities, although affected individuals have less pronounced short stature and typically do not have heart defects. The two conditions are caused by mutations in the same genes. 

Inheritance:

  This condition is inherited in autosomal recessive patterns which means both copies of the gene in each cell have mutations. The parents of an individual with an autosomal recessive condition each carry one copy o the mutated gene, but they typically do not show signs and symptoms of the disease. 

Causes:

  Ellis- van Creveld is passed down through families. it is caused by defects in 1 of 2 Ellis-van Crevel syndrome genes (EVC and EVC2). These genes are positioned next to each other on the same chromosome. The severity of the disease varies from person to person. The rights rate of the condition is seen among the Old Order Amish population of Lancaster County, Pennsylvania. It is fairly rare in the general population. 

Symptoms: Include

- Cleft lip or palate

- Epispdis or undescended testicle (cryptorchidism)

- Extra fingers (polydactyly)

- Limited range of motion

- Nail problems, including missing or deformed nails

- Short arms and legs, especially forearm and lower leg

- Short height, between 3.5 to 5 feet 

- Spares, absent, or fine-textured hair 

- Tooth abnormalities, such as peg teeth, widely-spaced teeth

- Teeth present at birth (natal teeth)

- Delayed or missing teeth

Disorders with similar symptoms:

 Ellis- van Creveld syndrome is in the category of rare skeletal disorders called short rib- polydactyly syndromes, belonging to the ciliopathies group. 

  These disorders are characterized by growth deficiency resulting in short stature, abnormally short ribs, extra fingers and toes, and variable visceral manifestations. These additional findings may include polycystic kidneys, underdevelopment of the lungs, vertebral and genitourinary abnormalities, central nervous system abnormalities, and cleft lip and cleft palate. They are inherited as autosomal recessive genetic conditions. 

 The short rib- polydactyly group includes 4 antenatal lethal types, saldino-Noonan, Majewski, Verma- Naumoff, and Beemer- Langer syndromes, and 2 types compatible with life, EVC, and Asphyxiating Thoracic Dystrophy or Jeune syndrome. 

 ATD is characterized by variable respiratory insufficiency due to thorax narrowness, kidney, liver, and retinal abnormalities, and inconstant short stature. At least two genes are associated with this condition. The neonatal clinical presentation overlap with EVC features, especially in the absence of heart abnormalities. 

 Weyers acrofacial dysostosis is another genetic disorder associated with polydactyly, dental and nail abnormalities, short stature, and abnormal facial features. This condition has been found to be associated with a single mutation in either the EVC or EVC2 gene and follows autosomal dominant inheritance. 

Diagnosis:

 Ellis- Van Creveld syndrome is diagnosed by the observation of short stature, slow growth, and skeletal abnormalities determined by imaging techniques and sometimes teeth present at birth (natal teeth). Molecular genetic testing for the EVC and EVC2 genes is available on a research basis only. Prenatal diagnosis is possible by ultrasound. 

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Reactive attachment disorder(Attachment Disorder in Adult) part- III

                    Reactive attachment disorder

Reactive attachment disorder (RAD) is a psychiatric condition wherein people have difficulty forming attachments with others. It is usually the result of early childhood trauma. 

About Reactive attachment disorder (RAD):

 RAD is a rare condition in which a child or adolescent experiences difficulties forming attachments with others. According to the diagnostic and statistical manual 5th edition, RAD is the result of a trusted source of experiencing neglect or abuse during early childhood. 

Reactive attachment disorder (RAD) can develop in children who do to form a predictable and nurturing bond with a trusted parent or caregiver. This may happen as a result of:

- Growing up in an overcrowded orphanage

- Frequently changing foster homes

- Having a parent or caregiver who has mental or physical health issues.

- Being taken away from a primary caregiver after establishing a bond with them.

- Experiencing long periods of hospitalization. 

     Being unable to form early emotional bonds can cause a child to stop engaging with others emotionally and develop behavioral problems. 

     Reactive attachment disorder (RAD) tends to develop in early childhood and typically affects children and adolescents. However, the symptoms may also extend into adulthood for some people. 

Reactive attachment disorder (RAD) in children: 

   A child can develop RAD following early life trauma that prevents them from forming may consistent emotional bonds with others. 

 Some children may develop RAD as a result of early neglect, such as being left crying, wet, or hungry for hours and having no one to interact with. Others may develop RAD following emotional or physical abuse. 

  Neglect or abuse can cause feelings of isolation, abandonment, and fear. Children who develop RAD tend to stop trying to form consistent emotional bonds with others. 

  This can extend into adolescence and adulthood. For example, some people may have difficulty trusting others and forming close personal relationships. 

Signs and symptoms: 

 Children with RAD may display some or all of the following signs and symptoms trusted source:

- Rarely seeking comfort when distressed

- Not responding to comfort when distressed 

- Minimal emotional or social responses, such as avoiding eye contact and not smiling 

- Persistent low mood and crying 

- Unexplained episodes of irritability, anxiety, or fearfulness

- A lack of interest in interacting or playing with others.

- A lack of social skills

- Cognitive problems

- Hyperactivity 

- Repetitive movements, such as rocking back and forth or hand flapping 

 These symptoms must occur before the age of 5 years in order for the child to receive a diagnosis of RAD. 

Patterns: 

When children with RAD grow a little older, their symptoms usually fall into one of two general patterns: inhibited RAD symptoms or disinhibited RAD symptoms. 

The following sections will discuss these in more detail. 

Inhibited RAD symptoms: 

  With this pattern, children are usually aware of what happens around them, but they do not respond typically to outside stimuli. 

children showing inhibited RD symptoms are often withdrawn and emotionally unresponsive. They may not show or seek affection from caregivers or others, instead preferring to keep to themselves. 

Disinhibited RAD symptoms: 

 With this pattern, children may be overly friendly toward strangers. Also, they may not prefer their primary caregiver over other people. 

In most cases, these children act younger than others' age and may seek affection from others in a potentially unsafe way. 

Reactive attachment disorder (RAD) in adolescents and adults:

 Without treatment, a child who develops RAD may continue to experience issues throughout adolescence and adulthood. 

 These issues may include:

- Avoidance of physical contact

- Difficulty displaying affection or care for others

- A lack of empathy

- Poor social skills

- Difficulty forming stable 

- Relationships with others

- Cognitive problems, such as poor memory or problem-solving skills

- Anger problems

- Control issues

- Academic or workplace problems

- Engaging in risky behaviors, such as substance abuse

- Mental health problems, such as depression or anxiety

Complications:

RAD can cause complications in children, adolescents, and adults. 

Children with RAD may be at increased risk of developmental delays and delayed growth. 

Older children and adolescents may be at increased risk of:

- Anger management issues

- Difficulties in school

- Depression

- Anxiety 

- Eating disorders

- Drug and alcohol abuse

If a person does not receive treatment for RAD during childhood or adolescence, the above complications may occur.

Diagnosis:

 A doctor or therapist will diagnose according to the DSM-5 guidelines. These guidelines trusted source state that a child must meet the following criteria in order to receive a diagnosis of RAD:

- A consistent pattern of emotionally withdrawn behavior toward a parent or caregiver, as evidenced by the child rarely seeking or responding to comfort when distressed

- A consistent emotional or social disturbance, as characterized by at least two of the following behaviors: 

- Minimal social and emotional responsiveness to other people 

- Limited displays of positive emotion.

- Episodes of unexplained irritability, sadness, or fearfulness even during nonthreatening interactions with adult caregivers 

- A traumatic experience that consists of one or more of the following:

- Social neglect or deprivation

- Repeated changes to the child's primary caregiver

- Rearing in a setting that severely limits the child's ability to form selective attachments. 

- Not meeting the diagnostic criteria for an autism spectrum disorder

- Experiencing signs of RAD before the age of 5 years

- Having a developmental age of at least 9 months. 

     Some of the signs and symptoms of RAD overlap with those of other conditions, such as intellectual disabilities or attention deficit disorders. A doctor will want to rule out these conditions before diagnosing RAD.

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Attachment Disorder in Adults(Avoidant attachment) part- II

                      Avoidant attachment 

    An attachment style is the pattern of behaviors a person exhibits in response to relationships and bonds. Developing an avoidant attachment style as a child can lead to difficulties in forming close relationships as an adult.

  Avoidant attachment is one of three attachment styles trusted sources that Mary Ainsworth and Barbara Wittin developed in 1970. 

The Four attachment styles are:

 Secure: 

   Secure attachment develops in children with a parent or caregiver who is sensitive and responsive to their needs. Securely attached children have confidence that a parent or caregiver will be available to meet their needs and comfort them when they are distressed. 

Avoidant, or insecure-avoidant:

 

   Avoidant attachment develops in children who do not experience sensitive responses from a parent or caregiver to their needs or distress. Children with an avoidant attachment may become very independent, both physically and emotionally. 

Anxious, or insecure-anxious: 

  Children with anxious attachment do not have consistent responses to their needs from a parent or caregiver. Children with an anxious attachments may be clingy around their caregiver while insecure in themselves or in their interactions with others. 

Fearful, or Disorganized:

 Disorganized attachment occurs when a child wants love and care from their parent or caregiver but is also afraid of them. Disorganized attachment can develop if a parent or caregiver responds to a child seeking comfort by ignoring, yelling at, or punishing them in some way. 

Strange situation procedure:

  In the 1970s, Mary Ainsworth did an experiment called the " strange situation procedure". In this experiment, parents or caregivers left the room as their child played with a trained observer nearby. The researchers observed and documented the child's response to their parent or caregiver leaving the room. Children with a secure attachment style would cry when their parent or caregiver left the room but go to them and quickly become soothed on their return. 

 Children with an avoidant attachment style would be calm when their parent or caregiver left the room. Once they returned, the child would avoid or resist the child would avoid or resist having contact with them. 

Causes: 

  Infants and children generally need to form a close bond with their parents or caregiver. The repeated rejection of attempts to form this secure attachment may result in a child learning to suppress their desire for comfort when distressed or upset. 

  Avoidant attachment develops when an infant or young child has a parent or caregiver who is consistently emotionally unavailable or unresponsive to their needs. Infants with an avoidant attachment style may also have faced repeated discouragement from crying or expressing outward emotion. 

 The parent or caregiver of a child who has avoidant attachment may: 

- Lack of knowledge on how to support their child

- Lack empathy

- Feel overwhelmed by parenting 

- Responsibilities

- Not have developed a sense of commitment

- Have an avoidant attachment style themselves

 Children with an avoidant attachment may also disconnect from their own needs and feelings. These children may learn to self-soothe and feel as though they can only rely on themselves. As a result, they have little motivation or trust to seek help or support from others. 

Signs and symptoms: 

   A child with an avoidant attachment style may show no outward display of desire for closeness, affection, or love. However, internally, the child will feel the same stress and anxiety attachment when they are in stressful situations. 

 These children may also want to be near their primary caregiver but not interact with them. They may also reject physical contact with their caregiver. 

 Attachment styles and their associated behaviors can last into adulthood. As an adult, a person with an avoidant attachment style may experience the following: 

- Avoiding emotional closeness in a relationship

- Feeling as though their partners are being clingy when they simply want to get emotionally closer. 

- Withdrawing and coping with difficult situations alone

- Suppressing emotions

- Avoiding complaining, preferring to sulk or hint at what is wrong. 

- Suppressing negative memories

- Withdrawing, or tuning out, from unpleasant conversations or sights 

- Fearing rejection

- Having a strong sense of independence 

- Having feelings of high self-esteem while having a negative view of others 

- Being overly focused on their own needs and comforts. 

 Avoidant attachment can also affect older adults. A study from Hong Kong found that in older married couples, a male partner with an avoidant attachment style experienced more detrimental effects on their well-being than a female partner. 

Prevention:

  A parent or caregiver can prevent their child from developing an avoidant attachment style by being sensitive to their needs and feeling while encouraging them to express their wants and emotions. It is also important for a person to let their child know that they are safe and cared for through both actions and words. 

    Parents or caregivers should also be mindful to avoid making their children feel ashamed if they make a mistake or are afraid, Instead, they should soothe and comfort their children as often as possible when they are distressed or scared. 

   If a parent or caregiver finds that they are struggling with parenting and suspects that they may not consistently be meeting the emotional needs of their child, they should seek help from a mental health professional who specializes in working with people with these issues. 

 Anyone with concerns about how their child is developing, including their attachment style, may also find speaking with a pediatrician or child psychologist helpful.

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Attachment Disorder in Adult- Part 1

                    Attachment Disorder in Adults- Part 1

      An attachment disorder is a type of mood or behavioral disorder affecting a person's ability to form and maintain relationships. 

   These disorders typically develop in childhood. They can result when a child is unable to have a consistent emotional connection with a parent or primary caregiver. 

  There is no formal attachment disorder diagnosis for adults, but they can experience attachment issues. These can stem from untreated or undiagnosed attachment disorders in childhood. 

The Fundamentals: Attachment theory 

Attachment theory deals with how people form emotional bonds. The way that a person learns to form and maintain relationships primarily stems from their initial interactions with a parent or primary caregiver during childhood. 

 

 Psychologists initially study and categorized different types of attachment that can develop during childhood. Researchers later developed the Adult Attachment Interview to distinguish the types in adults. The questions assess the type of easy relationship that an adult had with their primary caregiver. 

 Types of attachment in adults are similar to those observed in children. They include:

Secure: An adult with secure attachments likely had a positive emotional bond with their primary caregiver. They are comfortable in their relationship and have low relationship anxiety. 

 Avoidant or dismissing: Adults with these attachments are uncomfortable with closeness and value independence in their relationship. As a child, their caregiver may not have been attuned to their needs. 

 Anxious or preoccupied: Adults with these attachments crave intimacy and do not feel secure in their relationships. A child may develop this attachment style if their caregiver has intermittent or unpredictable availability. 

 Disorganized: Adults with this attachment style may have intense or chaotic patterns of relationships, marked by seeking closeness and then pushing people away, for example. It may develop in response to childhood trauma or abuse. 

Types of attachment disorder: 

 -Reactive attachment disorder 

 -Disinhibited social engagement disorder 

- Reactive attachment disorder:

 RAD is typically a trusted source of early childhood maltreatment or neglect. 

  American academy of child and adolescent psychiatry notes that children with RAD may:

 - Have low levels of interaction with other people

- Show little or no evidence of emotion during social interactions

- Have difficulty claiming down when stressed

- Seem unhappy, irritable, sad, or scared when engaging in everyday activities with their caregivers. 

 If the child does not receive adequate treatment, the symptoms of RAD may manifest or continue into adulthood. Possible symptoms of the disorder in adults include:

- Difficulty reading emotions

- resistance to affection

- Difficulty showing affection

- Low levels of trust

- Difficulty maintaining relationships

- A negative self-image

- Anger issues

- Impulsivity

- Detachment. 

Disinhibited social engagement disorder:

 DSED may develop in response to social neglect and lack of consistent attachment to a primary caregiver during the first 2 years of life. 

Children in care often demonstrate symptoms of DSED. These may include:

- Hyperactivity

- Minimal social boundaries 

- Extreme sociability

- Readiness to approach and engage with strangers

If a child with DSED does not receive adequate treatment, the issue can manifest continuing into adulthood. An adolescent or adult with DSED may display:

- Hyperactivity

- An extreme trust in people that they do not know well

- A lack of awareness of social boundaries

- A tendency to ask intrusive questions to people that they have just met 

- Other behaviors that show a lack of inhibition 

Signs and symptoms:

- Uncertainty about their true identity

- Discontinuity in their sense of self 

- Related changes in behavior, consciousness, and memory

- A feeling of disconnectedness from 

- Memory loss relating to personal information or everyday events

- Reduced ability to feel physical pain. 

Attachment disorder and adult relationships:

 An attachment disorder that develops in childhood may affect relationships in adulthood, and more research into this area is necessary. 

 A person with an attachment disorder may have difficulty trusting others or feeling safe and secure in a relationship. As a result, they may have difficulty forming and maintaining friendships and romantic partnerships. 

Complications:

 Untreated childhood RAD or DSED can cause the following during adulthood:

 - Low self-esteem

- Emotional impairment

- Difficulty in social situations 

- Anxiety 

- Depression

- Dissociation

- Problems with substance use trusted source 

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