Frenotomy and Human Milk Therapy in Patient with Severe Malnutrition and Severe ADEL (Ankyloglossia with Deviation of the Epiglottis and Larynx) 

Written by : dr Ratih Ayu Wulandari, IBCLC

Motherhood os a true gift from God, that woman is able to pregnant, delivering her baby and to breastfeed. This is said by God in various religions Indonesia, including Islam, Protestant, Catholic, Hinduism and Buddhism. The nature given to this woman is indicated by the reproductive organs she has, namely the uterus and all its parts, for the fetus to grow and develop while in the womb and the breasts to be able to breastfeed the child when he is born. This means that all women have the potential to breastfeed their children, as well as the potential for pregnancy and childbirth. (1)

Breastfeeding is natural, but just understanding it, is not enough. It required a deep understanding of breastfeeding, both in terms of benefits and everything related to the technicalities of breastfeeding. Women need to understand the anatomy and physiology of breasts, must also understand the preparation and techniques of breastfeeding, and know the problems that can be faced in breastfeeding and how to overcome them. (1)

Each living thing that is breastfeeding or referred to as a mammal has been provided with a pair or more breasts which will produce milk for the food of the newborn. Milk for each type of mammal is different and is specific for each species, which is adjusted to its needs, growth rate and breastfeeding habits. For example, humans who are resourceful and do school work will certainly need breast milk that is of maximum benefit to their brain. Meanwhile, whales that live in a very cold environment and have a short feeding time, need breast milk with high fat content so that they can stay warm in the cold oceans.

In table 1, we can see the various differences in the composition of breast milk for mammals that are adjusted to their needs. (1)

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Human breast milk has many benefits for human babies, because in it there are various substances that have many benefits for humans, both as nutrition or as a protective factor for babies, including: fat, protein, carbohydrates, mineral salts and vitamins. Human infants who receive breast milk suffer from disease less frequently, because of the protective substances in breast milk, including Lactobacillus bifidus, lactoferrin, lysozymes, complement C3 and C4, antistreptococcal factors, antibodies, cellular immunity, and do not cause allergies. Breastfeeding also provides beneficial psychological effects, leads to good growth, reduces the incidence of dental caries, also reduces the incidence of malocclusion. (1)

In order to latch on well, a baby needs to be well positioned on the mother’s body and needs good mouth attachment to the breast.

The position of breastfeeding can change according to the growth and development of the baby. Babies can be positioned to breastfeed lying on their back, lying on their side, sitting or standing. The baby is held as close as possible and hugs the mother with both hands.

Good attachment to the breast in a newborn is indicated by the baby’s chin touching the breast, part of the areola is inside the baby’s mouth, the baby’s lips are folded out, and the mouth is wide open at least 130-160 degrees.(2)

During sucking, the baby’s mouth acts like a vacuum with negative pressure which sucks the breast, emptying its contents. This negative vacuum in the baby’s mouth is affected by the lips that are folded outward, the tongue which can stick out past the lower gums, the cheeks of the baby which are convex when feeding. If the vacuum is good, the suction is good, the baby will empty the breast properly, and the mother will feel that the breast is soft, not swollen, there is no grip or irritation in the breast. A healthy baby can manage how much he needs to drink according to his body’s needs.(2)

There are things that can interfere with the latching of the baby’s mouth and sucking on the breast. The position of the baby during pregnancy in the mother’s stomach or the difficult and traumatic process of labor can inhibit the baby’s suction reflex. Anatomical abnormalities in the baby’s mouth can also interfere with suction, such as cleft lip or palate, ankyloglossia / tongue-tie or lip tie. To be able to suck properly, anatomical abnormalities in the baby’s mouth need to be corrected. Among them by means of frenotomy (cutting / incision) on the tongue or lip strap or surgery on the cleft lip and cleft palate.(2)

This cutting method is known as Frenotomy using the Pare Method. In 17th century in Italy, almost all babies who were baptized would have a frenotomy because the procedure would make it easier for the baby to latch onto the breast. The Pare method is used to cut the middle part of the tongue frenulum and free the bonds with the fingers to the genioglossus muscle. This action is safe and only takes a few seconds. Immediately after the frenotomy, the baby’s skin turns pinker, the voice becomes clearer, the body temperature increases, and the baby becomes more relaxed, making breastfeeding easier. These changes indicate that the tongue is related to the respiration process or breathing. In infants who have ankyloglossia when seen using a fibroscopy instrument on the laryng, there is a deviation (deviation) of the epiglottis and larynx. So the oximetry device shows a low oxygen saturation (SpO2) and an increased heart rate when feeding to the breast. Then various other signs appear such as crying with difficulty, cold extremities, swollen abdomen, thin hair or bald, shallow sleep, even snoring. The diagnosis of Ankyloglossia with Deviation of the Epiglottis and Larynx (ADEL) is given for this condition and not just Ankyloglossia alone. In Japan, ADEL’s condition was improved with Correction of the glosso-larynx (CGL) and Expansion of the Vestibular Oris (EVO).(3)

Malnutrition is estimated to cause death in one third of child deaths in the world. Lack of access to nutritious food, wrong feeding practices such as inadequate breastfeeding, inadequate complementary foods, cause children not to get the good nutrition they need, leading to malnutrition. Infectious diseases such as persistent diarrhea, pneumonia, measles or malaria can affect the nutritional status of children.(4)

In children, Energy Protein Malnutrition is defined if the measurement is below -2 Standard Deviation. If body weight according to age is below -2 SD defined as underweight, if the height of age is below -2 SD defined as stunting and if weight according to height is below -2 SD defined as wasting. Wasting indicates recent weight loss, while stunting is usually the result of chronic weight loss. Severe malnutrition can take many forms, for example khwarsiorkor and marasmic. Marasmic was caused by severe wasting conditions. Subcutaneous fat and muscle mass is lost due to lack of nutrition. Marasmic kwashiorkor, is a severe wasting condition with edema (accumulation of fluid between the body cell spaces), and kwashiorkor, which is malnutrition with edema, usually accompanied by changes in hair and skin color, anemia, hepaomegaly, lethargy, decreased immunity, and even death. (4)

Here we present a case of Severe Malnutrition with Severe ADEL (Ankyloglossia with Deviation of the Epiglottis and Larynx) Treated with Frenotomy and Human Milk Therapy.

The patient met Praborini Lactation Team for the first time on April 10, 2020. Ms. Y, a teacher who is currently working from home, came with Mr. D’s (husband), an IT Programmer, bringing baby girl By H who is 9 months 1 week old. Mothers complain that babies have difficulty eating, always vomit while eating and often experience allergic reactions.

Babies born with Midwife, initially suckle to the breast, but always cry and were not satisfied. From the age of 1 month old, babies are given powdered goat’s milk apart of breastfeeding. This based on a friend advice, to avoid formula milk that is too sweet. The goat’s milk is given 1 measure and added to 100cc of water, then given with a teat bottle. Babies are not vaccinated, and only control her weight in a local heath center.

From the age of 4 months, a red rash develops all over the baby’s body, the baby also often secretes fluid from the ear. The mother consulted to a pediatrician, allergist consultant and said that the baby was allergic to nuts and eggs. Given anti-allergic drugs and even hospitalized. Breastfeeding is stopped at 6 months of age because the mother’s milk is getting less and the mother is pregnant.
From the age of 6 months and starting solids, babies always vomit and cannot swallow food. During the consultation, the baby can only eat one meal per day of family food with a watery texture. Baby is no longer breastfeeding, she drink goat’s milk 100cc 10 times a day with a bottle teat.

On examination, it was found that the baby looked very sick, she could only moan with limited limb movement. There is edema throughout the body (face, stomach, extremities, even vulva). Cold extremities, thin body skin, thin hair. Delayed development, and cutis marmorata.

Birth weight is 3000gr , body weight during consultation (9 months 1 week old) : 4720 grams (increase 6gr / day <-3SD) there is a Grade 3 Lip Tie and tight submucosal Tongue Tie. On breast examination, mother has protruding nipple with no milk supply. Mother is pregnant with gestation age of 5 months.

The assessment at that time was malnourished infants with khwarsiorkor, severe ADEL and severe allergies to goat’s milk.

Parents were given counseling on the benefits of breast milk, changing goat’s milk with human breast milk, looking for human milk donor, how to pasteurize human milk, parents were given counseling on tongue tie, lip tie and frenotomy and referred to the head of the lactation team.

On April 11, 2020, a lingual frenotomy was performed on the baby. Mothers were taught how to do tongue exercise 5 times a day for 3 weeks. As soon after frenotomy, the baby is able to eat corn cream soup bought in the hospital canteen without gagging and vomiting, the baby can drink from a glass without gagging. Relactation for babies is not suggested because the baby’s condition is difficult to suckle and the mother is pregnant. Parents were taught tongue exercise and how to do tummy time. Babies are encouraged to drink pasteurized human milk donor using a bottle teat and stop giving goat milk.

Review on 18 April 2020
The mother feels that the baby is more active in moving, babbling, and the fluid from the ear has not come out. On examination, the baby was able to hold her own bottle contain human milk donor, the baby’s weight became 5585 (increase 123gr / day) nutritional status <-3SD. Baby’s extremities has been warmer, edema on vulva has gone. A maxillary tie frenotomy was performed, mothers were taught how to do lip exercises.

Review on 25 April 2020
The mother feels the baby can drink more and eat more, the baby’s swelling shrinks and the skin peels off. Babies show a better response after 2 weeks of drinking human milk donor. Upon examination, the edema on the face, stomach, limbs, were decreased and body weight decreased to 4610gr nutritional status <-3SD. Baby were given vaccination of DPT, Hib, Polio, Hepatitis B, INH prophylaxis and iron supplementation.

The photo on the left was taken before the frenotomy, when the baby’s extremities were still cold. The baby’s entire body is still blue. The middle photo was taken one week after the frenotomy, the baby’s body warms up and the scanner shows a green color. The right photo was taken 2 weeks after frenotomy, where the baby’s body is warm and the scanner display is red. Frenotomy in infants improves breathing and oxygen perfusion in the baby’s body.

Review on 30 Mei 2020
Babies was 11 months of age, she already able to sit and roll to the side. Her body weight increased to 6205 (increased by 47gr per day, nutritional status <-2SD). Babies were given MR vaccine, prophylactic INH, and iron supplementation.

Review on 20 Juni 2020
Babies was 12 months, she already able to crawl. Body weight increased to 6895 (increased by 32.9 gr per day, nutritional status <-2SD) Baby was given vaccination of DPT, Hib, Polio, Hepatitis B, prophylactic INH, iron and vitamin D supplementation.

Review on 25 Juli 2020
Babies was 1 year 1 month of age, she was able to babble a lot. Body weight increased to 7650gr and she was already in a good nutrition status. Baby was given vaccination of DPT, Hib, Polio, Hepatitis B, INH prophylaxis, iron and vitamin D supplementation.

Discussion
Breastmilk is species specific and is created with the most appropriate composition for the needs of each species. Humans who get milk from other species such as cow’s milk or goat’s milk cannot be perfect humans, tend to get sick and experience developmental delays.

In this case, holistic therapy is essential. Frenotomy is the initial way to free a baby’s tongue and lips that are bound so that the baby can eat and drink better. Goat milk intake is replaced with human milk donor which has millions of benefits so that baby get the nutrition and balanced composition she needs. WHO 4 star solid food, with a thick texture, was also easier for the baby to swallow after a frenotomy.

ADEL (Ankyloglossia with Deviation of the Epiglottis and Larynx) which occurs in infants causes tissue perfusion to be impaired. So that immediately after the frenotomy, the baby gets enough oxygen for the whole body, thus the metabolism of her body cells gets better.

This baby was also given vaccines and prophylaxis of Tuberculosis to prevent an infection. Because in a malnutrition status, disease will occur more easily, leading to death.

Due to limited costs, parents are exempt from all consultation fees and no laboratory trials are conducted for babies. The diagnosis is purely determined from the physical examination and clinical experience of the doctor who handled this case.

Conclusion
Breastmilk is a golden liquid with millions of benefits and goodness, every baby born alive should get breast milk for up to 2 years without exception. The ability to detect breastfeeding problems and how to solve them needs to be known by mothers and medical personnel, so that breastfeeding problems can be overcome from the start and prevent babies from disease or malnutrition.

References
1. Rulina Suradi et al. Bahan Bacaan Manajemen Laktasi. Jakarta : Perinasia, 2019.
2. Asti Praborini, Ratih Ayu Wulandari. Anti Stres Menyusui . Jakarta : Kawan Pustaka, 2018.
3. Changes in Breastfeeding and of Breasts before and after Babies’ Surgeries for Ankyloglossia with Deviation of the Epiglottis and Larynx (ADEL) – Healthy Breastfeeding. Mukai, Susumu. s.l. : Scientific Research, 2016, Vol. 8.
4. WHO. World Health Organization. Maternal, newborn, child and adolescent health. [Online] World Health Organization. [Cited: August 18, 2020.] https://www.who.int/maternal_child_adolescent/topics/child/malnutrition/en/.
5. Malnutrition and health in developing countries. Olaf Müller, Michael Krawinkel. s.l. : Canadian Medical Association Journal, 2005, Vol. 173.

 

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